Gastrointestinal Pathology

 

Description:

Lewin Gastrointestinal Pathology and Its Clinical Implications, Second Edition This comprehensive, two-volume resource highlights the practical aspects of the pathology of biopsies and gross specimens, the clinical/pathological correlation, and differential diagnoses, and the ways in which these affect the management of patients with gastrointestinal disorders. The authors provide valuable insights on many important areas of gastrointestinal pathology, and openly address controversies within the specialty. This all-inclusive work stands alone in its illustrative quality and in its emphasis on the clinical implications of patient management as related to pathologic findings. The Second Edition has been completely revised to reflect two decades of advances in the field. The book's focus on clinical/pathological correlations and differential diagnoses emphasizes their affect on patient management. Major revisions of the chapters on colitis and gastritis feature new approaches to treatment. Over 2100 full-color illustrations highlight pathologic features to sharpen diagnostic skills and guide treatment choices.  NEW to the Second Edition… • Completely revised content reflects two decades of advances in the field. • Focus on clinical/pathological correlations and differential diagnoses emphasizes their affect on patient management • Major revisions of the chapters on colitis and gastritis feature new approaches to treatment. • Over 2100 full-color illustrations highlight pathologic features to sharpen diagnostic skills and guide treatment choices. Now with the print edition, enjoy the bundled interactive digital edition, offering tablet, smartphone, or online access to: • Complete content with enhanced navigation • A powerful search that pulls results from content in the book, your notes, and even the web • Cross-linked pages, references, and more for easy navigation • Highlighting tool for easier reference of key content throughout the text • Ability to take and share notes with friends and colleagues • Quick reference tabbing to save your favorite content for future use Pick up your copy today!

 

Table of Contents:

 

Volume I

Chapter 1: Dialogue, Biopsies–Taking and Handling; Resected Specimens; Protocols

Chapter 1 Introduction

Mucosal Biopsy

Table 1-1: Recipe to Improve Biopsy Quality and Interpretation Dramatically

Usual Endoscopic Pinch Biopsies

Hot Biopsy Forceps

Cold Biopsies

Figure 1-1

Electrocautery Snare Biopsy

Snare Polypectomy

Snare Polypectomy after Submucosal Injection (“Lift-and-Cut” Technique)

Shave Biopsy

Endoscopic Mucosal Resection and Endoscopic Submucosal Dissection

Endoscopic mucosal resection

Endoscopic submucosal dissection

Submucosal lesions

Figure 1-2

Figure 1-4

Figure 1-3

Ancillary Techniques Used at Endoscopy

Diagnosis of Infections—Smears, Brushings, Aspiration, and Culture

Cytology

Direct-vision brush cytology

Balloon mesh cytology

Fine-needle aspirates

Chromoendoscopy

Barrett’s esophagus

Inflammatory bowel disease

Screening and surveillance colonoscopy for adenomas in otherwise healthy individuals

Virtual Histology

Biopsy specimen handling and processing

Figure 1-5

Figure 1-6

Figure 1-7

Figure 1-8

Figure 1-9

Figure 1-10

Table 1-2: Guide to Sites for Taking Biopsies

Handling of the Biopsy Specimen Prior to Immersion in Fixatives

Handling Polyps

Routine Fixation

Tissue Processing, Embedding, and Cutting

Figure 1-11

Figure 1-12

Figure 1-13

Figure 1-14

Figure 1-15

Description of Endoscopic Findings

Table 1-3: Suggested Descriptive Terms for Benign-Appearing Lesions at Endoscopy

Biopsy Specimen Location

Table 1-4: Recommended Approach to Describe the Locations of Endoscopic Lesions and Biopsy Sites

Number and Size of Biopsy Specimens

The History and the Question for the Pathologist

Approach to the Microscopic Examination

A Systematic Approach to Biopsy Specimen Interpretation

Table 1-5: Example of a Systematic Approach to the Examination of Gastrointestinal Biopsy Specimens

Table 1-6: Some Infections That May Be Found in Exudate or as Attachments to the Surface Epithelium

Technical Problems in Interpretation

Mucosal Hemorrhage and Edema

Pseudoerosions

Other Artifacts

Figure 1-16

Figure 1-17

Figure 1-18

Figure 1-19

Figure 1-20

Figure 1-21

The Pathologist’s Interpretation

Mild Nonspecific Chronic Inflammation

Special Fixatives, Stains, or Storage Conditions

Immunohistochemical Applications in Gastrointestinal Disorders

Interpretation of Immunohistochemical Stains

Infections

Tuberculosis and Mycobacterium avium-intracellulare

Table 1-7: Disorders That May Require Special Fixatives, Stains, or Storage Conditions

Surgically Resected Specimens

Examination of the Specimen

Frozen Sections

Photography

Opening the Specimen

Fixation

Insufflation with Fixative

Injection studies for vascular diseases

Examination and dissection of the fixed specimen

Reexamination of the Fixed Specimen

Dissection

Dissections of Tumors

Lymph Node Dissections

Depth of Tumor Penetration

Venous Invasion by Tumor

Sections of Resected Margins

Incidental Findings

Chapter 2: Vascular Disorders and Related Diseases

Chapter 2 Introduction

Vascularization of the Digestive Tract—Overview

Vascularization of the Specific Segment of the Digestive Tract

Esophagus

Stomach, Small Intestine, and Large Intestine

Intramural circulation

Extramural (Splanchnic) circulation

Venous drainage

Collateral blood supply

Figure 2-1

Figure 2-2

Figure 2-3

Ischemia of the Gastrointestinal Tract

Pathophysiology

Etiology and Clinical Manifestations

Esophageal ischemia

Gastric ischemia

Acute mesenteric ischemia

Chronic mesenteric ischemia

Pathology

Gross features

Microscopy

Ischemic colitis

Ischemic proctitis

Table 2-1: Causes of Acute and Chronic Mesenteric Ischemia

Table 2-2: Cellular Effects of Ischemia

Figure 2-4

Figure 2-5

Table 2-3: Major Causes of Acute Mesenteric Ischemia

Figure 2-6

Figure 2-7

Figure 2-8

Figure 2-9

Figure 2-10

Figure 2-11

Figure 2-12

Table 2-4: Etiology of Ischemic Colitis in Young Adults (n = 42)

Figure 2-13

Figure 2-14

Figure 2-15

Figure 2-18

Figure 2-16

Figure 2-17

Figure 2-19

Figure 2-21

Figure 2-22

Figure 2-20

Inflammatory Vascular Disorders of the Gastrointestinal Tract (Vasculitides)

Introduction

Classification

Clinical Presentation

Different Types of Vasculitides

Large vessel vasculitides

Infectious vasculitides

Medium vessel vasculitides

Medium and small vessel vasculitis (ANCA-associated vasculitides)

Small vessel vasculitis

Miscellaneous conditions

Malignant-atrophic papulosis (Kohlmeier–Degos Syndrome)

Biopsy Diagnosis of Ischemic Colitis and Differential Diagnosis

Stercoral ulcers

Table 2-5: Classification of Vasculitis According to Histologic Pattern

Table 2-6: Classification of Vasculitides by Caliber of the Vessel

Table 2-7: Frequency of Intestinal Involvement in Different Vasculitides

Table 2-8: Laboratory Parameters Important for Patients Clinically Suspected of Vasculitis

Figure 2-23

Figure 2-24

Figure 2-25

Figure 2-26

Figure 2-27

Figure 2-28

Figure 2-29

Figure 2-30

Mechanical Obstruction and Ischemia of the Digestive Tract

Pathogenesis and Clinical Features

Adhesions

Hernias

Hernia of the anterior abdominal wall

Inguinal and femoral hernia

Umbilical hernia

Internal hernias

Volvulus

Intussusception

Figure 2-31

Iatrogenic Disorders of the Vascular System

Iatrogenic Intestinal Ischemia

Arterial obstruction or constriction

Drug-induced vascular lesions

Neutropenic colitis

Iatrogenic Gastrointestinal Bleeding

Radiation Injury

Pathophysiology

Acute radiation injury

Chronic (late) radiation injury

Figure 2-32

Figure 2-33

Figure 2-34

Figure 2-35

Table 2-9: Vascular Changes in Radiation-Induced Injury

Figure 2-36

Figure 2-37

Figure 2-38

Figure 2-39

Vascular Abnormalities of the Gastrointestinal Tract

Vascular Ectasia (Angiodysplasia)

Gastric Antral Vascular Ectasia

Dieulafoy Malformation (Caliber-Persistent Arteriole)

Telangiectasias

Hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber Syndrome)

Arteriovenous Malformation

Phlebectasia

Diseases Affecting Blood Vessels

Disorders of Connective Tissue Affecting Blood Vessels

Pseudoxanthoma elasticum

Ehlers–Danlos syndrome

Table 2-10: Classification of Vascular Anomalies of the GI Tract

Figure 2-40

Figure 2-41

Figure 2-42

Figure 2-43

Figure 2-44

Handling of Specimens

Endoscopic Biopsies

Surgical Specimens

Ischemic disease

Vascular malformations

Figure 2-45

Chapter 3: Immunodeficiency Disorders

Chapter 3 Introduction

Intestinal Host Defences

Functional Anatomy of the GI Immune System

Normal Distribution of Gut-associated Lymphoid Tissue

Humoral immune system of the gut

Cellular immune system of the gut

Figure 3-1

Figure 3-2

Figure 3-3

Figure 3-4

Figure 3-5

Figure 3-6

Immunodeficiency Disorders of the Intestinal Tract

Clinical features

Histology

Table 3-1: Immunologic Tests for the Categorization of Primary Immunodeficiency Disease

Table 3-2: Pathology of the GI Tract in Primary Immunodeficiency Disorders

Figure 3-7

Figure 3-8

Table 3-3: Incidence of Neoplasia in Immunodeficiency Disorders

Primary Immunodeficiency Disorders

Predominant Antibody Defects

Common variable hypogammaglobulinemia (Late-onset acquired hypogammaglobulinemia, common variable immunodeficiency [CVID], Bruton’s X-linked agammaglobulinemia [XLAG])90,91,92

Selective IgA deficiency

Secretory component deficiency

Infantile X-linked agammaglobulinemia (Bruton’s agammaglobulinemia, congenital agammaglobulinemia)

Miscellaneous B-cell disorders

Predominant Cell-mediated Immunodeficiency

Severe combined immunodeficiency disease (Swiss-type agammaglobulinemia, hereditary thymic dysplasia)

IPEX syndrome

Chronic mucocutaneous candidiasis

Table 3-4: GI Manifestations of Primary Immunodeficiency Syndromes

Figure 3-9

Figure 3-10

Figure 3-11

Immunodeficiency Associated with Other Defects

DiGeorge’s Syndrome—Third and Fourth Pouch/Arch Syndrome (Thymic Hypoplasia, Cellular Immunodeficiency with Hypoparathyroidism)

Phagocytic and Other Cell Dysfunction

Chronic Granulomatous Disease

Systemic Mastocytosis

Secondary (Acquired) Immunodeficiency Disorders

Bone Marrow Transplantation

Transplantation regimen

Infection

Graft versus host disease (GVHD)

Chronic GVHD

Intestinal Transplantation

The Acquired Immunodeficiency Syndrome (AIDS)

Pathogenesis and clinical features

GI AIDS infections

GI neoplasms in AIDS

Table 3-5: Common GI Infections and Parasitic Infestations in Bone Marrow Transplantation

Figure 3-12

Table 3-6: Grading Scheme for Acute Cellular Rejection in Small Bowel Allografts

Figure 3-13

Table 3-7: Common Opportunistic GI Infections in AIDS

Figure 3-14

Figure 3-15

Figure 3-16

Workup of the Immunodeficient Patient

Chapter 4: Lymphoproliferative Disorders of the Gastrointestinal Tract

Chapter 4 Introduction

Overview

Introduction

Definition

Incidence

Pathogenesis of GI Lymphoma

Classification of GI Lymphomas

Clinical Presentation and Other Practical Diagnostic Issues

Role of Molecular Diagnosis in GI Tract Lymphomas

Workup of Lymphoproliferative Disorders of the GI Tract

Table 4-1: Tumor Site of Gastrointestinal Lymphomas

Table 4-2: Classification of GI Lymphomas

Table 4-3: Modified Ann-Arbor Staging System for Primary Gastrointestinal Lymphomas

Lymphoid Hyperplasia of the Gastrointestinal Tract

Localized Lymphoid Hyperplasia of the Stomach—Gastric Lymphoid Hyperplasia (Pseudolymphoma)

Pathology

Angiofollicular Hyperplasia (Lymphoid Hyperplasia with “Castleman-like” Features)

Immunohistochemical features and molecular genetics

Localized Lymphoid Hyperplasia of the Small Intestine

Localized (lymphoid) hyperplasia of the terminal ileum and appendix

Localized lymphoid hyperplasia of the duodenum and small intestine excluding the terminal ileum

Localized Lymphoid Hyperplasia of the Rectum

Diffuse Nodular Lymphoid Hyperplasia of the Intestine

Diffuse nodular lymphoid hyperplasia with hypogammaglobulinemia

Diffuse nodular lymphoid hyperplasia without hypogammaglobulinemia

Figure 4-1

Figure 4-2

Figure 4-3

Figure 4-4

Figure 4-5

Figure 4-6

Figure 4-7

Figure 4-8

Figure 4-9

Figure 4-10

Figure 4-11

Figure 4-13

Figure 4-12

Lymphoproliferative Disorders of the Esophagus

Lymphoproliferative Disorders of the Stomach

MALT Lymphoma (Extra Nodal Marginal Zone Lymphoma)

Pathogenesis

Clinical features

Pathology

Immunophenotype and molecular genetics

Treatment and prognosis

Reporting MALT lymphomas

Follow-up of patients with treated MALT lymphoma

Role of the pathologist

Differential diagnosis and practical approach to the difficult diagnosis

Diffuse Large B-cell Lymphoma of the Stomach

Primary Gastric T-cell Lymphoma

Pathology

Other Miscellaneous Lymphoproliferative Disorders

Figure 4-14

Figure 4-15

Figure 4-16

Figure 4-18

Figure 4-17

Figure 4-19

Figure 4-20

Lymphoproliferative Disorders of the Small Intestine

MALT Lymphoma (Extranodal Marginal Zone Lymphoma) and Diffuse Large B-cell Lymphoma of the Small Intestine (Western-type Lymphoma)

Immunoproliferative Small Intestinal Disease (Mediterranean Lymphoma, α-Chain Disease)

Etiopathogenesis

Clinical features

Pathology

Immunophenotype and molecular genetics

Diagnosis and differential diagnosis

Treatment and prognosis

Burkitt’s and Burkitt’s-like Lymphoma (Malignant Lymphoma of the Small, Noncleaved Type)

Clinical presentation

Pathology

Immunophenotype and molecular genetics

Prognosis and treatment

Enteropathy-type T-cell Lymphoma

Pathogenesis

Clinical presentation

Pathology

Immunophenotype and molecular genetics

Treatment and prognosis

CD4 Positive Small Intestinal T-cell Lymphoma

Other Miscellaneous Lymphoproliferative Disorders

Figure 4-21

Figure 4-22

Table 4-4: Galian Staging System for IPSID

Figure 4-23

Figure 4-24

Figure 4-25

Lymphoproliferative Disorders of the Appendix, Colon, and Anal Canal

MALT Lymphomas of the Colon

Mantle Cell Lymphoma

Multiple Lymphomatous Polyposis

Figure 4-26

Miscellaneous Lymphoproliferative Disorders of the GI Tract

Follicular Lymphomas (Follicular and Diffuse Types)

Clinical features

Pathology

Immunophenotype and molecular genetics

Differential diagnosis

Treatment and prognosis

Lymphoplasmacytic Lymphoma/Waldenström’s Macroglobulinemia

Immunodeficiency-Associated Lymphoproliferative Disorders

Primary immunodeficiency-associated lymphoproliferative disorders

Acquired immunodeficiency-associated lymphoproliferative disorders

Solitary Plasmacytomas of the Gastrointestinal Tract

Lymphomatoid Granulomatosis (Angiocentric Lymphoproliferative Lesion)

Mycosis Fungoides Involving the Gastrointestinal Tract

Anaplastic Large-cell Lymphoma (Ki-1 Lymphoma)

Extranasal NK Cell or NK-like T-cell Lymphoma

NK cell enteropathy

Hodgkin’s Lymphoma of the Gastrointestinal Tract

True Histiocytic Lymphomas (Histiocytic Sarcoma) of the GI Tract

Langerhans Cell Histiocytosis (LCH, Histiocytosis X) of the GI Tract

Other Miscellaneous Lymphomas of the GI Tract

Angioimmunoblastic Lymphadenopathy Involving GI Tract

The Gastrointestinal Tract in Leukemia and Granulocytic Sarcoma

Figure 4-27

Table 4-5: Immunohistochemical Differentiation of Small Lymphocytic Gastrointestinal Lymphomas

Figure 4-28

Table 4-6: Lymphomas Associated with HIV Infection

Figure 4-29

Figure 4-30

Figure 4-31

Figure 4-32

Figure 4-33

Chapter 5: Disorders of Endocrine Cells

Chapter 5 Introduction

Introduction and Historical Perspective

From the APUD System (Amine Precursor Uptake and Decarboxylation) and on

Figure 5-1

Figure 5-2

The Normal Endocrine Cells at Specific Gastrointestinal Sites: Where They Are and What They Do

The Diseases: Perspectives Based on Clinical Implications

Table 5-1: Classification of Endocrine Tumors in the Gastrointestinal Tract

Table 5-2: Endocrine Tumors in the Gastrointestinal Tract and Their Distribution

Genetics of Endocrine Tumors and Endocrine Syndromes Involving the Gut

Carcinoid Tumors (Well-Differentiated Endocrine Tumors)

General Information

Gross Examination

Gross appearance

Gross dissection recommendations

Staging and Grading

Figure 5-3

Figure 5-4

Figure 5-5

Carcinoid Tumors (NETs) in Specific Sites

Carcinoid Tumors (NETs) of the Esophagus

Carcinoid Tumors (NETs) of the Stomach

Type I carcinoid tumors

Type II carcinoid tumors

Type III carcinoid tumors

Potential type IV carcinoid tumors

Carcinoid Tumors of the Duodenum

Gastrinomas

Somatostatinomas

Gangliocytic paraganglioma

Carcinoid Tumors of the Jejunum and Ileum (Midgut Carcinoids, NETs)

Incidental finding of carcinoid in biopsies of the cecum or terminal ileum

Carcinoid Tumors of the Appendix

Carcinoid Tumors of the Abdominal Colon

Carcinoid Tumors of the Rectum

Table 5-3: Gastric Enterochromaffin-like Cell (ECL-Cell) Carcinoid Tumors Subtypes

Figure 5-6

Figure 5-7

Figure 5-8

Figure 5-9

Figure 5-10

Figure 5-11

Figure 5-12

Figure 5-13

Figure 5-14

Figure 5-15

Figure 5-16

Figure 5-17

Figure 5-18

Figure 5-19

Figure 5-20

Poorly Differentiated Endocrine Neoplasms (Neuroendocrine or Endocrine Carcinomas)

General Information

Esophageal Tumors

Colonic and Rectal Tumors

Figure 5-21

Figure 5-22

Figure 5-23

Figure 5-24

Figure 5-25

Adenomas and Adenocarcinomas with Both Epithelial and Endocrine Differentiation

Tumors in Which Endocrine and Columnar Cells are Mixed

Tumors with Separate Components (Composite Tumors)

Figure 5-26

Figure 5-27

Endocrine Tumors Associated with Ulcerative Colitis and Crohn’s Disease

Hyperplasias

Figure 5-28

Figure 5-29

Figure 5-30

Enteroendocrine Cell Dysgenesis

Metastatic Endocrine Tumors with an Unknown Primary

Figure 5-31

Figure 5-32

Practical Approaches to Gastrointestinal Endocrine Abnormalities

General Information

Tumors

Chapter 6: Motility Disorders

Chapter 6 Introduction

Diverticular Disease of the Small and Large Intestines

Definition

Terminology

Large Bowel Diverticular Disease (Diverticulosis of the Colon)

Definitions

Pathogenesis

Clinical Features

Endoscopy

Imaging

Gross Pathology

Prediverticular disease

Handling of specimens

Histology

Defining diverticulitis

Figure 6-1

Figure 6-2

Figure 6-3

Figure 6-4

Figure 6-5

Figure 6-6

Figure 6-7

Figure 6-8

Figure 6-9

Figure 6-10

Figure 6-11

Figure 6-12

Figure 6-13

Figure 6-14

Polyps and Neoplasms Associated with and within Diverticula

Diverticular Polyps

Inverted diverticulum

Other Polyps

Carcinoma in diverticular disease

Figure 6-15

Diverticular Disease and Endometriosis

Diverticular Disease and IBD

Differential Diagnosis and Clinical Implications

Figure 6-16

Diverticulosis of the Cecum and Proximal Colon (Right-Sided Diverticulosis)

Pathogenesis

Clinical Features

Pathology

Figure 6-17

Diverticulosis of the Duodenum and Small Intestine

Pathogenesis

Duodenal Diverticula (Single or Isolated)

Extraluminal diverticula

Intraluminal duodenal diverticulum (prolapsed diaphragm)

Jejunoileal Diverticula

Pathogenesis and clinical features

Gross appearance

Histology

Meckel’s Diverticulum

Figure 6-18

Gastrointestinal Motility Disorders

The Normal Motility Apparatus of the Gut

Musculature of the Gut

Demonstration of Smooth Muscle in Motility Disorders

Figure 6-19

Enteric Nervous System

Interstitial Cells of Cajal

Figure 6-20

Figure 6-21

Figure 6-22

Figure 6-23

Figure 6-24

Figure 6-25

Motility Disorders

Table 6-1: Classification of Neuromuscular Disorders with GI Manifestations

Table 6-2: London Classification of GI Neuromuscular Pathology

Esophageal Disorders

Achalasia (Cardiospasm)

Gross appearance

Histology

Secondary Achalasia

Figure 6-26

Other Motor Disorders of the Esophagus

Figure 6-27

Figure 6-28

Motor Disorders of the Stomach

Pyloric Stenosis

Infantile pyloric stenosis (congenital hypertrophic pyloric stenosis)

Adult pyloric stenosis

Gastroparesis

Intestinal Pseudoobstruction

Definition

Acute intestinal pseudoobstruction

Ogilvie’s syndrome

Pathogenesis and Clinical Presentation of Chronic Pseudoobstruction

Chronic intestinal pseudoobstruction

Table 6-3: Chronic Idiopathic Intestinal Pseudoobstruction

Chronic Idiopathic (Primary) Intestinal Pseudoobstruction

Examining Resections for CIIP

Familial or Sporadic Visceral Myopathy

Pathology

Gross appearance

Histology

α-Smooth Muscle Actin Deficiency

Familial Visceral Myopathy with α-SMA–Positive Inclusion Bodies

Differential diagnosis

Desmin Myopathy

African Visceral Myopathy

Issues Reporting Motility Disorders

Table 6-4: Major Secondary Causes of CIIP

Figure 6-29

Figure 6-30

Figure 6-32

Figure 6-34

Figure 6-36

Figure 6-37

Figure 6-31

Figure 6-33

Figure 6-35

Figure 6-38

Figure 6-39

Figure 6-40

Figure 6-41

Figure 6-42

Figure 6-43

Primary Visceral Neuropathies

Familial Visceral Neuropathy

Paraneoplastic Syndromes

Sporadic Visceral Neuropathy

Differential diagnosis

Abnormalities of Interstitial Cells of Cajal

Hyperplasia of ICCs

Prognosis and therapy of CIIP

Diffuse Lymphoid Infiltration

Diffuse Eosinophilic Infiltrate

Slow Transit Constipation: Severe Idiopathic Constipation

Figure 6-44

Figure 6-45

Figure 6-46

Figure 6-47

Figure 6-48

Figure 6-50

Figure 6-49

Hirschsprung’s Disease

Clinical Presentation and Features

Etiology and Pathogenesis

Gross Appearance

Histology

Diagnosis of Hirschsprung’s Disease

Types of biopsy

Intraoperative frozen section

Variants of Hirschsprung’s Disease

Figure 6-51

Figure 6-52

Figure 6-53

Figure 6-54

Figure 6-55

Figure 6-56

Figure 6-57

Table 6-5: Variants of Hirschsprung’s Disease and Approximate Frequency

Intestinal Neuronal Dysplasia

Internal Sphincter Achalasia (Ultrashort Hirschsprung’s Disease)

Megacystis Microcolon Intestinal Hypoperistalsis

Diagnosis

Immature Ganglia

Mural Eosinophils in Hirschsprung’s Disease

Idiopathic Megacolon

Severe Idiopathic Constipation

Acquired Visceral Neuropathies

Toxic or Drug-Induced Visceral Neuropathy

Inflammatory Visceral Neuropathy (Acquired Aganglionosis)

Chagas’ Disease (American Trypanosomiasis)

Gross appearance

Histology

Differential diagnosis

Paraneoplastic Neuropathy

Miscellaneous Visceral Neuropathies

GI Manifestations Secondary to Neurologic Disorders of the Brain and Spinal Cord

Chronic Intestinal Pseudoobstruction Associated with Generalized Disease and the Muscular Dystrophies

Myotonic Muscular Dystrophy

Histology

Progressive Muscular Dystrophy

Acquired Jejunal Diverticulosis

Solitary Rectal Ulcer Syndrome of the Rectum and Inflammatory Cloacogenic Polyp (Mucosal Prolapse Syndromes)

Gross and Endoscopic Appearances

Histology

Differential Diagnosis

Proctitis (localized colitis) cystica profunda (hamartomatous inverted polyp of the rectum)

Gross and endoscopic appearances

Pathogenesis and histology

Nuclear atypicality, dysplasia, and carcinoma

Inflammatory cloacogenic polyp

Irritable Bowel Syndrome

Pathology of irritable bowel syndrome

Mastocytic enterocolitis

Role of the pathologist

Figure 6-58

Figure 6-59

Figure 6-60

Chapter 7: Mesenchymal Tumors

Chapter 7 Introduction

Introduction

Table 7-1: Classification of Gastrointestinal Mesenchymal Tumors

Gastrointestinal Stromal Tumors

Histogenesis

Demography and Clinical Aspects

Diagnostic Procedures

Targeted Therapy with Imatinib

Surgery for GIST

Frozen Section Diagnosis

Gross Examination and Appearances

Microscopic Appearances

Unusual morphologic variants

Posttreatment changes

Immunohistochemistry

False-positive immunoreactivity

CD117-negative GISTs

CD117- and DOG1-Positive Tumors Other Than GISTs

Molecular Features and Mutational Analysis

Succinyl dehydrogenase subunit B (SDHB) expression, NF1 mutations, and BRAF mutations

SDHB-deficient GISTs

Chromosomal alterations

Predicting Behavior

GIST Syndromes

Familial GIST syndrome

Neurofibromatosis type 1 (von Recklinghausen’s disease)

Carney’s triad

GIST-paraganglioma syndrome (Carney–Stratakis syndrome)

Sporadic multiple GIST

Pediatric GIST

Small GIST

Differential Diagnosis

Reporting GIST

Figure 7-1

Figure 7-2

Figure 7-3

Figure 7-4

Figure 7-5

Figure 7-6

Figure 7-7

Figure 7-8

Table 7-2: Potential GIST Mimics that May Express CD117 and/or DOG1

Figure 7-9

Table 7-3: The NIH 2001 Consensus Classification Scheme for Risk Stratification in GIST

Table 7-4: Risk Stratification Scheme Based on Long-term Follow-up Data from >1,600 Patients from the Armed Forces Institute of Pathology Prior to the Era of Imatinib Therapy

Table 7-5: Molecular Classification of Gastrointestinal Stromal Tumors

Table 7-6: Differential Diagnosis Based on Morphologic Subgroup

Table 7-7: Key Anatomic, Morphologic, and Immunohistochemical Features of Lesions to Be Considered in the Differential Diagnosis of Spindled, Cytologically Bland GIST

Table 7-8: Example of GIST Synoptic Report

Smooth Muscle Tumors

Leiomyomas

Epstein–Barr Virus–associated Smooth Muscle Tumors

Leiomyomatosis

Smooth Muscle Hamartoma

Leiomyomatosis Peritonealis Disseminata

Glomus Tumor

Leiomyosarcoma

Figure 7-10

Figure 7-11

Figure 7-12

Neurogenic Tumors

Schwannoma

Neurofibroma

Ganglioneuroma

Polypoid ganglioneuromas

Ganglioneuromatous polyposis

Diffuse ganglioneuromatosis

Mucosal Neuroma/Schwann Cell “Hamartoma”

Perineurioma and Fibroblastic Polyp

Perineurioma

Fibroblastic polyps

Biopsy Diagnosis of Polypoid Neural Lesions

Paraganglioma

Granular Cell Tumor

Malignant Peripheral Nerve Sheath Tumor

Mixed Neuronal Glial Tumor

Figure 7-13

Figure 7-14

Figure 7-15

Figure 7-16

Figure 7-17

Figure 7-18

Figure 7-19

Figure 7-20

Figure 7-21

Fibroblastic/Myofibroblastic Tumors

Desmoid Tumor (Intraabdominal Fibromatosis)

Inflammatory Fibroid Polyp

Plexiform Fibromyxoma of the Gastric Antrum

Inflammatory Myofibroblastic Tumor

Solitary Fibrous Tumor/Hemangiopericytoma

Calcifying Fibrous Tumor

Elastofibroma

Figure 7-22

Figure 7-23

Figure 7-24

Figure 7-25

Figure 7-26

Figure 7-27

Figure 7-28

Adipocytic Lesions

Lipohyperplasia of the Ileocecal Valve

Submucosal Lipoma

Atypical Lipoma

Angiolipoma

Lipomatous Polyposis and Epiploic Lipomatosis

Liposarcoma

Differential Diagnosis of Fatty Tumors

Figure 7-29

Figure 7-30

Figure 7-31

Endothelial and Vascular Tumors

Hemangioma

Angiosarcoma

Kaposi’s Sarcoma

Lymphangioma

Intestinal Vascular Lesions Associated with Clinical Syndromes

Figure 7-32

Figure 7-33

Figure 7-34

Figure 7-35

Figure 7-36

Striated Muscle Tumors

Rhabdomyoma

Rhabdomyosarcoma

Figure 7-37

Miscellaneous Sarcomas

Clear Cell Sarcoma

Malignant Gastrointestinal Neuroectodermal Tumor (GINECT)

Endometrial Stromal Sarcoma

Undifferentiated High Grade Pleomorphic Sarcoma/Malignant Fibrous Histiocytoma

Undifferentiated Sarcoma

Figure 7-38

Figure 7-39

Figure 7-40

Perivascular Epithelioid Cell Tumors

PEComa

Angiomyolipoma

Figure 7-41

Biphasic Epithelial–Mesenchymal Lesions

Synovial Sarcoma

Spindle Cell Carcinoma/Carcinosarcoma

Gastroblastoma (Epithelial Biphasic Tumor of Young Adults)

Figure 7-42

Figure 7-43

Figure 7-44

Figure 7-45

Nonmesenchymal Tumors That May Mimic Mesenchymal Neoplasms

Melanoma

Follicular Dendritic Cell Sarcoma

Lymphoma

Sarcomatoid Adult Granulosa Cell Tumor

Figure 7-46

Figure 7-47

Fibrosing Lesions of the Mesentery, Peritoneum, and Retroperitoneum

Sclerosing Mesenteritis

Sclerosing Peritonitis

Idiopathic Retroperitoneal Fibrosis

Weber–Christian Disease

Figure 7-48

Figure 7-49

Figure 7-50

Nonneoplastic Lesions That May Mimic Mesenchymal Neoplasms

Reactive Nodular Fibrous Pseudotumor

Pseudosarcomatous Granulation Tissue

Heterotopic Mesenteric Ossification

Xanthogranulomatous Pseudotumor

Mycobacterial Spindle Cell Pseudotumor

Figure 7-51

Figure 7-52

Figure 7-53

Chapter 8: Gastrointestinal Manifestations of Extraintestinal Disorders and Systemic Disease

Chapter 8 Introduction

Introduction

Connective Tissue Disorders (Collagen Vascular Diseases)

Scleroderma (Progressive Systemic Sclerosis)

Pathogenesis and Clinical Features

Gross Pathology and Histology

Clinical Implications

Dermatomyositis and Polymyositis

Systemic Lupus Erythematosus

Mixed Connective Tissue Diseases and the Overlap Syndrome

Rheumatoid Arthritis

Miscellaneous Disorders

Figure 8-1

Figure 8-2

Figure 8-3

Figure 8-4

Figure 8-5

Figure 8-6

Figure 8-7

Gastrointestinal Manifestations in Endocrine Disorders

Thyroid Gland

Hyperthyroidism

Hypothyroidism

Autoimmune Thyroid Disease

Thyroid Neoplasms

Parathyroid Gland

Hyperparathyroidism

Hypoparathyroidism

Endocrine Pancreas

Diabetes

Hyperfunction of Islets of Langerhans

Gastrinoma

VIPoma Syndrome (Verner–Morrisons Syndrome)

Somatostatinoma

Other Islet Cell Tumors

Adrenal Gland

Gonads

Pregnancy

Hypothalamus and Pituitary

Hypopituitarism

Pituitary Adenoma

Autoimmune Polyendocrinopathy Syndrome Type 1

The IPEX syndrome

Figure 8-8

Table 8-1: Gastrointestinal Manifestations of Multiple Endocrine Neoplasia (MEN) Syndromes

Gastrointestinal Manifestations in Renal Disease

Acute Renal Failure

Chronic Renal Failure

Endoscopic and Histologic Appearances

Other Findings

Renal Transplantation

Role of the Pathologist and Clinical Implications

Table 8-2: Interrelationship of Gastrointestinal and Renal Diseases

Gastrointestinal Manifestations of Hepatic Disorders

Portal Hypertension

Primary Sclerosing Cholangitis and Autoimmune Hepatitis

Liver Transplantation

Gastrointestinal Manifestations of Skin Disorders

Bullous Disorders

Epidermolysis bullosa

Epidermolysis Bullosa Acquisita

Pemphigus Vulgaris

Cicatricial Pemphigoid (Benign Mucous Membrane Pemphigoid)

Stevens–Johnson Syndrome

Herpes Simplex Virus Infection

Hyperkeratotic Disorders

Lichen planus

Tylosis

Miscellaneous Disorders

Acrodermatitis enteropathica

Darier’s Disease

Dermatogenic Enteropathy

Malignant Disease of the Gastrointestinal Tract and Skin Disease

Acanthosis Nigricans

Cowden’s Disease

Dermatomyositis

Miscellaneous

Table 8-3: Primary Skin Disorders Associated with Gastrointestinal Disease

Gastrointestinal Manifestations of Cardiac Disease

Congestive Cardiac Failure

Infective Endocarditis

Open Heart Surgery, Extracorporeal Circulation, and Cardiac Transplantation

Hematologic Disorders

Dysproteinemias

Hemolytic Uremic Syndrome

Coagulation Disorders

Hemophilia

Other Coagulation Disorders

Mastocytosis

Rosai–Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy)

Miscellaneous Disorders

Figure 8-9

Figure 8-10

Gastrointestinal Amyloid Deposition

General Properties and Classification

Clinical Features

Histologic Features

Diagnosis and Clinical Implications

Figure 8-11

Figure 8-12

Figure 8-13

Figure 8-14

Figure 8-15

Disorders of Lipid Metabolism

Fabry’s Disease

Tangier Disease

Wolman’s Disease

Abetalipoproteinemia

Granulomatous Disorders

Sarcoidosis

Chronic Granulomatous Disease

Miscellaneous Disorders

Endometriosis

Pellagra

Familial Mediterranean Fever (Familial Paroxysmal Polyserositis)

Figure 8-16

Figure 8-17

Neoplastic Disease

Figure 8-18

Figure 8-19

Chapter 9: Esophagus: Normal Structures, Developmental Abnormalities, and Miscellaneous Disorders

Chapter 9 Introduction

Structure of the Esophagus

Anatomy

Histology

Mucosa

Submucosa and muscularis propria

Figure 9-1

Figure 9-2

Figure 9-3

Figure 9-4

Figure 9-5

Esophageal Function

Age-Dependent Changes

Embryology and Development of the Esophagus

Figure 9-6

Figure 9-7

Developmental and Congenital Anomalies

Esophageal Atresia and Tracheoesophageal Fistulas

Bronchoesophageal Fistula

Developmental and Congenital Cysts

Duplication and congenital cysts

Neurenteric cysts/remnants

Bronchogenic cysts

Other cysts

Heterotopias

Gastric heterotopia in the esophagus

Other heterotopias

Other Developmental Anomalies

Congenital esophageal stenosis or stricture

Short esophagus

Pulmonary sequestrations

Table 9-1: Concomitant Anomalies in Individuals with Esophageal Atresia

Figure 9-8

Table 9-2: The Different Types of Esophageal Atresia

Figure 9-9

Figure 9-10

Figure 9-11

Figure 9-12

Table 9-3: Esophageal Stenosis

Esophageal Perforation

Spontaneous Rupture (Boerhaave’s Syndrome)

Pathogenesis and clinical features

Pathology

Nonspontaneous Rupture and Penetration

Pathogenesis and clinical features

Pathology

Table 9-4: Causes of Esophageal Perforation

Figure 9-13

Esophageal Hemorrhage

Esophageal Tears (Mallory–Weiss Syndrome)

Pathogenesis and clinical features

Pathology

Esophageal Varices

Pathogenesis and clinical features

Pathology

Figure 9-14

Figure 9-15

Esophageal Fistula

Acquired Esophageal Stenosis or Stricture

Esophageal webs and rings

Figure 9-16

Figure 9-17

Figure 9-18

Figure 9-19

Figure 9-20

Diverticula and Pseudodiverticula

Upper Esophageal Diverticula (Zenker’s)

Pathogenesis and clinical features

Pathology

Mid- and Lower Esophageal Diverticula

Pathogenesis and clinical features

Pathology

Atypical Esophageal Diverticula in Scleroderma

Esophageal Intramural Pseudodiverticulosis/Retention Cysts

Pathogenesis and clinical features

Pathology

Figure 9-21

Figure 9-22

Figure 9-23

Figure 9-24

Other Miscellaneous Conditions

Mucosal Bridge

Glycogenic Acanthosis

Esophageal Xanthelasma

Figure 9-25

Chapter 10: Inflammatory Disorders of the Esophagus: Reflux and Nonreflux Types

Chapter 10 Introduction

Gastroesophageal Reflux Disease (Reflux Esophagitis–GERD/GORD)

Definition of GERD

Symptoms of GERD

Correlation between GERD, symptoms, and endoscopy

Therapy

Long-term therapy for GERD and prognosis

The Gastroesophageal Junction and Z-Line

Etiology and Pathogenesis of GERD

Endoscopic Grading of Reflux Disease in Squamous Mucosa

Los Angeles Grading System for Reflux Disease in Squamous Mucosa

Nonerosive Reflux Disease and Its Pathology

Gross/Endoscopic Appearances of GERD

Where to Biopsy for GERD and Criteria Used

Erosions

Traditional biopsy sites

Cardia biopsies

Histologic Diagnosis and Criteria for GERD in Biopsies

Evaluating reactive changes

Grading reactive epithelial changes

Table 10-1: Classification of Esophagitis by Etiology

Figure 10-1

Figure 10-2

Figure 10-3

Figure 10-4

Figure 10-5

Table 10-2: Reflux-independent Esophageal Lesions

Figure 10-6

Figure 10-7

Figure 10-8

Figure 10-9

Figure 10-10

Inflammatory Cells

Intraepithelial Mononuclear Cells

Minor changes

Inflammatory Polyps

Reactive changes versus neoplasia following erosions and ulcers

Atypical Cells in Squamous Mucosa

Carditis as a Manifestation of GERD

Progression of GERD

Complications of GERD

GERD-related strictures

Cameron’s Ulcer

Figure 10-11

Figure 10-12

Figure 10-13

Figure 10-14

Figure 10-15

Figure 10-16

Figure 10-17

Barrett’s Esophagus (BE)

Definition

Practical Aspects of Making the Diagnosis of BE

Irregular Z-line versus “ultrashort” BE

Definitions of BE: short- versus long-segment BE

When BE Does Not Have Goblet Cells, Does It Matter?

When are goblet cells not present?

Risk of carcinoma in patients with non–goblet cell BE

Conclusions regarding the definition and cancer risk in Barrett’s (columnar-lined) esophagus

Epidemiology and Pathophysiology of Barrett’s Esophagus

Morphologic Development of Barrett’s Esophagus

Endoscopic Grading of Barrett’s Mucosa

Histology

Double muscularis mucosae

Palisaded vessels

Handling of Patients and Biopsies Postablation or Postendoscopic Resection

Differential Diagnosis of BE and Other Mucosal Types Encountered

Barrett-associated Neoplasia

Diagnosing and Grading Dysplasia

Indefinite for Dysplasia

Low-grade Dysplasia (Low-grade Intraepithelial Neoplasia—LGNIN)

High-grade Dysplasia (High-grade Intraepithelial Neoplasia—HGIEN)

Implication

Intramucosal Carcinoma

Invasive Carcinoma (Submucosal invasion)

Interobserver Variability and Need for a Second Opinion for Dysplasia

Ancillary Techniques

Surveillance biopsies

Figure 10-18

Figure 10-19

Figure 10-20

Figure 10-21

Figure 10-22

Figure 10-23

Figure 10-24

Figure 10-25

Figure 10-26

Figure 10-27

Figure 10-28

Figure 10-29

Figure 10-30

Figure 10-31

Figure 10-32

Nonreflux Esophagitis

Infections

Viral Infections

Herpes virus infection

Cytomegalovirus esophagitis

Human papillomavirus

Acute HIV infection

Other viral infections

Fungal Infections

Clinic symptoms and prognosis

Other fungal infections

Bacterial Esophagitis

Luetic infection

Other bacteria

Figure 10-33

Figure 10-34

Figure 10-35

Figure 10-36

Figure 10-37

Figure 10-38

Descriptive Esophagitides

Lymphocytic Esophagitis

Exfoliative (Sloughing) Esophagitis (Esophagitis Dissecans Superficialis)

Acute Necrotizing Esophagitis (Black Esophagus)

Eosinophilic Esophagitis

Pathogenesis

Symptoms

Clinical

Biopsy sites

Pathology

Differential diagnosis

Corrosive Esophagitis

Drug-induced Esophagitis (Pill Esophagitis)

Figure 10-39

Figure 10-40

Figure 10-41

Figure 10-42

Figure 10-43

Systemic Diseases Involving the Esophagus

Skin Diseases and Esophageal Inflammation

Glycogenic acanthosis

Skin Diseases and Esophageal Inflammation

Pseudoepitheliomatous Hyperplasia

Leukoplakia

Graft versus Host Disease

Behçet’s Disease

Crohn’s Disease

Lymphocytic esophagitis in Crohn’s disease

Granulomatous Esophagitis and Sarcoid

Ulcerative Colitis

Brown Bowel Syndrome (Ceroid Lipofuscinosis)

Diabetes Mellitus

Parkinson’s Disease

Figure 10-44

Figure 10-45

Figure 10-46

Figure 10-47

Figure 10-48

Chapter 11: Polyps and Tumors of the Esophagus

Chapter 11 Introduction

Table 11-1: Classification of Esophageal Tumors

Role of the Pathologist

Squamous Cell Carcinoma

Pathogenesis and Clinical Features

Risk Factors

Geographic distribution: association with smoking and alcohol

Diet

Genetic factors

Human papillomavirus infection

Other risk or protective factors

Predisposing Conditions

Celiac sprue

Tylosis palmaris et plantaris

Other carcinomas

Prior irradiation

Premalignant Lesions: Dysplasia and Intraepithelial Neoplasia

Gross and Endoscopic Appearances

Microscopic Appearances

Spread of Tumor, Staging, and Prognosis

TNM classification

Local spread and prognostic factors

Effect of chemotherapy and irradiation

DNA ploidy

Molecular factors as prognosticator

Failure and causes of death

Figure 11-1

Figure 11-2

Figure 11-3

Figure 11-4

Figure 11-5

Unusual Variants of Squamous Cell Carcinoma

Superficial Esophageal Carcinoma

Endoscopic mucosal resection

Superficial spreading carcinoma

Verrucous Carcinoma

Spindle Cell Carcinoma

Gross appearances

Microscopic appearances

Histogenesis

Biopsy diagnosis

Small Cell Carcinoma

Basaloid Squamous Cell Carcinoma

Figure 11-6

Figure 11-7

Figure 11-8

Figure 11-9

Figure 11-10

Figure 11-11

Adenocarcinoma

Adenocarcinoma in the Columnar-lined Esophagus (Barrett’s Esophagus)

Risk and pathogenesis

Clinical Features

Gross and Endoscopic Appearances

Microscopic appearances

Endoscopic Ablation Techniques

Endoscopic mucosal resection

Prognosis

Figure 11-12

Figure 11-13

Figure 11-14

Figure 11-15

Unusual Variants of Adenocarcinoma

Adenoid Cystic Carcinoma

Mucoepidermoid Carcinoma

Adenocarcinoma in Heterotopic Gastric Mucosa

Adenocarcinoma in Submucosal Glands

Adenosquamous Carcinoma and Adenoacanthoma

Choriocarcinoma and Hepatoid Adenocarcinoma

Figure 11-16

Biopsy Diagnosis of Squamous and Adenocarcinoma and Associated Problems

Regenerative changes

Granulation tissue

Barrett’s esophagus

Other Polyps and Tumors

Squamous papilloma and papillomatosis

Inflammatory Polyps

Pseudosarcomatous changes in inflammatory polyps

Mucosal Tags

Fibrovascular Polyps

Hamartomas, Choristomas, Thyroid Rests, Parathyroid Rests

Malignant Melanoma

Secondary Tumors

Mesenchymal Tumors

Figure 11-17

Figure 11-18

Figure 11-19

Figure 11-20

Figure 11-21

Figure 11-22

Figure 11-23

Figure 11-24

Figure 11-25

Figure 11-26

Figure 11-27

Figure 11-28

Figure 11-29

Figure 11-30

Figure 11-31

Chapter 12: Stomach: Normal Structures and Developmental Abnormalities

Chapter 12 Introduction

Embryology

Stomach

Cardia

Duodenum

Figure 12-1

Normal Structure of the Stomach

Anatomy

Gastric surfaces (relations of the stomach)

Anatomic regions

Blood vessels and lymphatics

Nerve supply

Histology

Mucosa

Submucosa

Muscularis propria, ICCs, and serosa

Figure 12-2

Figure 12-3

Figure 12-4

Figure 12-5a

Table 12-1: Endocrine Cells of the Stomach

Figure 12-6

Figure 12-7

Figure 12-8

Figure 12-9

Figure 12-10

Figure 12-11

Figure 12-12

Figure 12-13

Figure 12-15

Figure 12-14

Figure 12-16

Figure 12-17

Figure 12-18

Table 12-2: Developmental Abnormalities of the Stomach

Figure 12-19

Developmental Abnormalities of the Stomach

Pathogenesis and Clinical Features

Figure 12-20

Chapter 13: Stomach and Proximal Duodenum: Inflammatory and Miscellaneous Disorders

Chapter 13 Introduction

Classification of Gastritis and Gastropathy

Current Classification of Gastritis

Table 13-1: ABC Classification of Gastritis

Table 13-2: Gastritis Classification “Historical Prospective”

Figure 13-1

Figure 13-2

Table 13-3: Gastritis Classification “Sydney System”

Gastritis

Distinctive (Specific) Types of Gastropathies

Reactive (Predominant Epithelial) Changes

Reactive gastropathy

Toxic gastropathy

Reactive Changes with Erosions in Helicobacter—One or Two Diseases?

Distinction of Reactive Changes from Dysplasia

Reactive changes in intestinal metaplasia

Alcoholic gastropathy

Caustic-induced injury

Graft versus host disease

Chemotherapy and radiation

Ischemia

Predominantly Vascular Changes

Gastric antral vascular ectasia

Portal hypertension (congestive gastropathy)

Hemorrhagic gastropathy (“gastritis”) and “Curling’s ulcer”

Distinctive (Specific) Types of Gastritis

Infections

A H. pylori infection

Histology of H. pylori–associated gastritis

H. pylori diagnosis

Noninvasive methods

Invasive methods

Atrophic gastritis and gastric atrophy

Staging gastric atrophy

Disorders associated with H. pylori gastritis

Gastroduodenal erosions and ulcers (“peptic ulcers”)

Pathogenetic factors

Epidemiology

Atypical clinical presentations

Endoscopic appearance of peptic erosions and ulcers

Table 13-4: Classification by Predominant Histologic Change

Figure 13-3

Figure 13-4

Figure 13-5

Figure 13-6

Figure 13-7

Figure 13-8

Figure 13-9

Figure 13-10

Figure 13-11

Figure 13-12

Figure 13-13

Figure 13-14

Figure 13-15

Figure 13-16

Figure 13-17

Table 13-5: A Helicobacter-associated Diseases

Figure 13-18

Figure 13-19

Figure 13-20

Figure 13-22

Figure 13-21

Figure 13-23

Figure 13-24

Table 13-6: Stains for Detection of Helicobacter pylori

Figure 13-25

Figure 13-26

Figure 13-27

Figure 13-28

Figure 13-29

Figure 13-30

Figure 13-31

Figure 13-32

Figure 13-33

Figure 13-34

Table 13-7: OLGA

Figure 13-35

Figure 13-36

Table 13-8: Factors That Damage or Protect the Gastric Mucosa

Figure 13-37

Table 13-9: Evidence Linking H. pylori to the Pathogenesis of Duodenal Ulcer836–838

Figure 13-38

Figure 13-39

Figure 13-40

Figure 13-41

Figure 13-42

Figure 13-43

Figure 13-44

Figure 13-45

Figure 13-46

Figure 13-47

“Peptic Diseases” of the Duodenal Bulb and the Proximal Duodenum

Pathogenesis and Clinical Features of Duodenitis

Duodenal ulcer

Clinical features

Pathology of Duodenitis and Duodenal Erosions and Ulcer

Gross pathology

Histology

Differential diagnosis of duodenitis

Healing and healed ulcers

Complications of gastroduodenal ulcers

Treatment

The role of the pathologist and clinical implications

Autoimmune Gastritis

Pathogenesis

Subtypes of autoimmune gastritis (AIG) and their etiology

Clinical features

Pathology

Table 13-10: Associations of Erosive and Nonerosive Duodenitis

Figure 13-48

Figure 13-49

Figure 13-50

Figure 13-51

Figure 13-52

Figure 13-53

Figure 13-54

Figure 13-55

Figure 13-56

Figure 13-57

Figure 13-58

Figure 13-59

Figure 13-60

Table 13-11: Oxyntic (Corpus and Fundus) Predominant Gastritis

Figure 13-61

Lymphocytic Gastritis

Morphologic Separation of Etiologies

Table 13-12: Lymphocytic Gastritis Associations

Figure 13-62

Figure 13-63

Granulomatous Gastritis

Figure 13-64

Table 13-13: Conditions Associated with Granulomatous Gastritis

Carditis

Figure 13-65

Non–H. pylori Bacterial Infections

Non–H. pylori Helicobacter Species (NHPH)/“Helicobacter heilmannii”

Gastric disease associated with non–H. pylori Helicobacter species/“Helicobacter heilmannii”

Diagnosis

Tuberculosis

Syphilis

Enterococcal Gastritis

Phlegmonous and Emphysematous Gastritis

Figure 13-66

Table 13-14: Non–Helicobacter pylori Detected in the Stomach of Humans

Figure 13-67

Viral Infections

Cytomegalovirus Infection (HHV-5)

Herpes Viruses (HHV-1,2)

Epstein–Barr Virus (EBV—HHV-4)

Other HHV Viruses

Other Viruses

Figure 13-68

Fungal Infections

Candida albicans

Histoplasmosis

Mucormycosis (Zygomycosis)

Aspergillosis

Figure 13-69

Parasites and Nematodes

Cryptosporidium

Anisakiasis

Other Parasites and Nematodes

Figure 13-70

Other Gastritides

Clinical features

Endoscopic features

Histologic features

Eosinophilic Gastritis

Eosinophilic gastritis as part of gastric involvement in eosinophilic gastroenteritis

Differential diagnosis

Collagenous Gastritis

Diffuse collagenous gastroenterocolitis

Gastric Malakoplakia

Drug- and Chemotherapy-induced Gastritis

Figure 13-71

Figure 13-72

Figure 13-73

Figure 13-74

Table 13-15: Differential Diagnosis of Eosinophilic Gastroenteritis

Table 13-16: Diagnostic Workup for Eosinophilic Gastroenteritis

Figure 13-75

Figure 13-76

Figure 13-77

Figure 13-78

Hypertrophic Gastropathies and Ménétrier’s Disease

Primary/Idiopathic Ménétrier’s Disease

Clinical Presentation

Pathology of Primary Ménétrier’s Disease

Carcinoma Complicating Ménétrier’s Disease

Secondary Ménétrier’s Disease

Table 13-17: Hypertrophic Gastropathies

Figure 13-79

Hypertrophic Gastropathy-associated with Protein Loss

Cytomegalovirus-associated Hypertrophic Gastropathy

Hypertrophic Lymphocytic Gastritis

Helicobacter pylori–associated Hypertrophic Gastritis

HIV-associated Hypertrophic Gastritis

Large Gastric Folds Associated with Other Conditions

Other Types of Large Gastric Folds

Figure 13-80

Figure 13-81

Distinctive Endoscopic Entities

Miscellaneous Disorders of the Stomach

Gastric Calcinosis

Gastric Glandular Siderosis

Approach to the Interpretation of Gastric Biopsies

Surface epithelium

Figure 13-82

Table 13-18: The Differential Diagnosis in Gastric Pathology

Histology

Chapter 14: Gastric Epithelial Polyps and Tumors

Chapter 14 Introduction

Adenoma/Dysplasia/Intraepithelial Neoplasia

Definitions and Terminology

Special Stains for Characterizing Gastrointestinal Epithelial cells

Classification of Gastric Adenomas

Intestinal-type adenomas

Gastric-type adenomas

Pyloric gland adenoma

Foveolar adenoma

Fundic gland adenoma

Grading of Adenoma/Dysplasia/Intraepithelial Neoplasia

Low-grade adenoma/dysplasia/intraepithelial neoplasia

High-grade adenoma/dysplasia/intraepithelial neoplasia

Differential Diagnosis

Distinction of regenerative changes from adenoma (dysplasia/intraepithelial neoplasia)

Distinction of high-grade dysplasia/adenoma/intraepithelial neoplasia) from invasive carcinoma

Figure 14-1

Figure 14-2

Figure 14-3

Figure 14-4

Figure 14-5

Figure 14-5

Figure 14-5

Figure 14-6

Figure 14-7

Figure 14-8

Figure 14-9

Figure 14-10

Gastric Carcinoma

Introduction

Gastric Cardia Carcinoma

Carcinoma of the Gastric Antrum and Body

Pathogenesis

Risk Factors

Helicobacter pylori infection

Epstein–Barr virus, CIMP-H, and K-ras

JC virus

Dietary factors

Smoking

Genetic factors

Hereditary gastric cancer predisposition syndromes

Other genetic abnormalities predisposing to gastric cancer

Polymorphism in genes

Predisposing Conditions

H. pylori–related chronic atrophic gastritis

Postgastrectomy

Immunodeficiency disorders

Menetriér’s disease

Premalignant Lesions of the Stomach

Adenoma/dysplasia/intraepithelial neoplasm

Gastric polyps

Gross Features of Gastric Carcinoma

Gross Features of Superficial Cancers and Their Subdivision

Classification and Natural History of Early and Late (Advanced) Gastric Carcinoma

Early gastric carcinoma/cancer

Advanced gastric cancer

Histologic Classification of Gastric Carcinomas

Lauren classification

Nakamura classification

Ming classification

WHO histologic classification and grading of gastric cancer

Figure 14-11

Figure 14-12

Figure 14-13

Figure 14-14

Figure 14-15

Figure 14-16

Figure 14-17

Figure 14-18

Figure 14-19

Figure 14-20

Histological Subtypes of Carcinoma

Tubular Adenocarcinoma

Well-differentiated Adenocarcinoma

Moderately Well-differentiated Adenocarcinoma

Poorly Differentiated Adenocarcinoma

Papillary Adenocarcinoma

Mucinous Adenocarcinoma (syn. Mucoid, Mucus, Colloid, and Muconodular Adenocarcinoma)

Signet-ring Cell Carcinoma

Endocrine Cell Tumors

Rare Variants of Gastric Carcinoma

Gastric carcinoma with lymphoid stroma (syn. medullary carcinoma, gastric lymphoepithelioma-like carcinoma)

α-Fetoprotein-producing gastric carcinoma and hepatoid adenocarcinoma

Adenosquamous and squamous cell carcinoma

Choriocarcinoma (syn. chorioepithelioma)

Yolk sack tumor (syn. endodermal sinus tumor)

Paneth cell carcinoma

Micropapillary carcinoma

Gastric adenocarcinoma of fundic gland type (fundic gland carcinoma, combined parietal, and chief cell carcinoma) including parietal cell carcinoma and chief cell carcinoma

Fundic gland adenoma

Extremely well-differentiated adenocarcinoma

Pyloric gland carcinoma

Composite adenocarcinoma and endocrine tumor with pancreatic differentiation and pancreatic type tumors

Undifferentiated carcinoma

Serrated dysplasia and carcinoma

Gastric teratoma

Carcinosarcoma

Gastroblastoma

Growth Pattern of Gastric Adenocarcinoma

Superficial (spreading) carcinoma

Organoid differentiation

Stromal reaction

Differential Diagnosis and Clinical Implications

Early gastric cancer

Distinction from histiocytic lesions

Lymphoma

Metastatic carcinoma

Neuroendocrine (NET—carcinoid) tumors

Granular cell tumors

Bizarre undifferentiated cells (pseudosarcomatous changes)

Radiation therapy and chemotherapy

Spread of Gastric Carcinoma and Prognostic Factors

Prognosis of Gastric Carcinoma

Early gastric cancer

Risk of nodal metastases in early gastric carcinoma

Gastric remnant carcinoma

Advanced gastric cancer

Neoadjuvant therapy

Her2 status

K-ras status

Biopsy Diagnosis of Gastric Carcinoma

Biopsy before surgical resection

Misreading of biopsies for carcinoma

Value of Cytology in the Diagnosis of Gastric Carcinoma

Handling of Gastric Specimens and Intraoperative Evaluation

Polypectomy specimens

Endoscopic mucosal or submucosal specimens

Resection specimens

Figure 14-21

Figure 14-21

Figure 14-21

Figure 14-22

Figure 14-23

Figure 14-24

Figure 14-25

Figure 14-26

Figure 14-27

Figure 14-28

Figure 14-29

Figure 14-30

Figure 14-31

Figure 14-32

Figure 14-33

Figure 14-34

Table 14-1: Lymph Node Metastasis in Patients with D2 Gastrectomy Surgery for Early Gastric Cancer

Table 14-2: TNM Classification and Staging of Gastric Carcinoma (Seventh Edition)

Table 14-3: Grading of the Effects of Neoadjuvant Therapy

Table 14-4: Causes of Errors in the Biopsy Diagnosis of Gastric Carcinoma

Table 14-5: One-Piece and Piecemeal EMR in Early Gastric Cancer

Figure 14-35

Table 14-6: Examination of Gastric Resection Specimens

Figure 14-36

Nonneoplastic Polyps

Classification

Biopsy and Excision of Gastric Polyps: Role of the Pathologist

Approach to the biopsy diagnosis of gastric polyps

Fundic Gland Polyps

Pathology

Clinical implications

Hyperplastic and Inflammatory Polyps

Pathology

Dysplasia/carcinoma in hyperplastic polyps

Clinical implications for inflammatory/hyperplastic polyps

Differential Diagnosis

Single polyps

Multiple gastric polyps

Juvenile Polyps

Pathology

Peutz–Jeghers Polyps

Pathology

PTEN Hamartoma Tumor Syndrome

Familiar Gastric Hyperplastic Polyposis

Cronkhite–Canada Syndrome–Associated Polyps

Pathology

Polypoid Gastritis

Gastric Xanthomas (Xanthelasmas, Lipid Islands)

Pathology

Mucosal Bumps and Nodules

Solitary Polypoid Hamartoma (Gastric Hamartoma with Myxoid Stroma, Hamartomatous Inverted Polyp, Heterotopic Inverted Polyp)

Pathology

Gastritis Cystica Polyposa (Gastric Stomal Polypoid Hyperplasia, Gastric Stomal Polypoid Hypertrophic Gastritis)

Pathology

Mucosal and Submucosal Cysts

Gastritis Cystica Profunda

Pathology

Differential Diagnosis

Heterotopic Pancreas (Ectopic Pancreas, Pancreatic Rests, Adenomyoma, Adenomyomatous Hamartoma, Myoglandular Hamartoma, Myoepithelial Hamartoma)

Pathology

Differential Diagnosis

Brunner Gland Heterotopia/Nodule

Heterotopic Antral Mucosa in Oxyntic Mucosa

Table 14-7: Gastric Polyps

Figure 14-37

Figure 14-38

Figure 14-39

Figure 14-40

Figure 14-41

Figure 14-42

Figure 14-43

Figure 14-44

Figure 14-45

Figure 14-46

Figure 14-47

Figure 14-48

Figure 14-49

Figure 14-50

Figure 14-51

Figure 14-52

Volume Ii

Chapter 15: Appendix

Chapter 15 Introduction

Introduction

Function of the Appendix

The Normal Appendix

Normal Anatomy, Development, and Gross Appearance

Histology

Figure 15-1

Figure 15-2

Figure 15-3

Figure 15-4

Figure 15-5

Figure 15-6

Figure 15-8

Figure 15-7

Routine Pathologic Examination of the Appendix

Gross Handling of Dilated Appendices

Congenital Structural Abnormalities

Positional Abnormalities

Agenesis

Duplication

Appendix helicus and horseshoe appendix

Congenital fistula

Heterotopic tissue

Table 15-1: Modified Wallbridge Classification of Appendiceal Duplication

Figure 15-9

Acquired Structural Abnormalities

Diverticula

Torsion and Volvulus

Intussusception

Figure 15-10

Iatrogenic Use of the Appendix

Acute Appendicitis

Clinical Features

Pathogenesis

Gross Appearance

Histology

Resolution of Acute Appendicitis

Crypt architectural distortion

Localized epithelial hyperplasia (reactive hyperplasia)

Submucosal fibrosis

Luminal obliteration (obliterative fibrosis) and neuroma of the appendix

Terminology in Acute Appendicitis

Unusual variants of appendicitis

Issues with Appendicitis and a Practical Approach

Complications of Acute Appendicitis

Perforation

Appendiceal epithelium growing along the external surface of the appendix

Periappendiceal abscesses and productive periappendiceal fibrosis

Formation of multiple lumina (lumens)

Escaped fecalith

Fistula

Suppuration and suppurative pyelophlebitis and hepatic abscess formation

The Appendiceal Stump

Recurrent stump appendicitis

Endoscopic excision of the inverted appendix stump

Abscess formation

Xanthogranulomatous appendicitis and malacoplakia

Hemorrhage

Intussusception

Polyps

Figure 15-11

Figure 15-12

Figure 15-13

Figure 15-14

Figure 15-15

Figure 15-16

Figure 15-17

Figure 15-18

Figure 15-19

Figure 15-20

Specific Infections of the Appendix

Viral Infections

Adenovirus

Cytomegalovirus

Measles

Infectious mononucleosis

Bacterial Infections

Acute enteritis

Spirochetosis

Yersinia

Tuberculosis and mycobacterial infection

Actinomycosis

Whipple’s disease

Parasites

Figure 15-21

Figure 15-22

Figure 15-23

Figure 15-24

Figure 15-25

Figure 15-26

Miscellaneous Inflammatory Conditions Affecting the Appendix

Granulomatous Appendicitis and Crohn’s Disease Isolated to the Appendix

Isolated appendiceal Crohn’s disease

Ulcerative colitis involving the appendix

Histiocytic diseases

Arteritis (localized polyarteritis nodosa)

Inherited disorders

Figure 15-27

Figure 15-28

Figure 15-29

Figure 15-30

Figure 15-31

Figure 15-32

Tumors of the Appendix

Appendiceal Tumors are Not the Same as Large Bowel Tumors

Polyps and Other Benign Lesions of the Appendix

Adenomas

Tubular and tubulovillous adenomas

Villous adenomas

Serrated hyperplasia and polyps

Localised and diffuse mucosal hyperplasia (Serrated hyperplasia)

Hyperplastic polyps

Sessile serrated polyps (“sessile serrated adenomas”)

Sessile serrated adenomas/polyps with dysplasia

Molecular pathology of appendiceal serrated lesions

Table 15-2: Classification of Epithelial Polyps and Tumors of the Appendix

Figure 15-33

Figure 15-34

Table 15-3: Classifications of Serrated Appendiceal Lesions

Figure 15-35

Figure 15-36

Figure 15-37

Issues with Polyps of the Appendix and Their Sequela—A Practical Approach

Terminology and Differential Diagnosis

Terminology Issues

Mucocele

Cystadenomas and cystadenocarcinomas

Hybrid lesions

Mucinous Neoplasms of the Appendix and Pseudomyxoma Peritonei

Mucinous Neoplasms of Appendix

Terminology

Incidence

Clinical features

Gross appearances

Histology

Molecular Pathology and Immunohistochemistry of Appendiceal Neoplasia

Issues with Mucinous Neoplasms—Practical Approach

Grading of dysplasia and distinction of LAMN from mucinous adenocarcinoma

Problems when perforation is present

Rupture and mucin extravasation

Unusual variations of appendiceal mucinous neoplasia

Follow-up and therapy

Pseudomyxoma Peritonei

Gross appearance

Microscopic appearance

Management of pseudomyxoma peritonei and pathology of cytoreduction

Adenocarcinomas of the Appendix

Large bowel-type carcinoma

Grading appendiceal carcinomas

TNM staging of appendiceal tumors and prognosis of appendiceal neoplasms

Goblet Cell Carcinoma (Goblet Cell Carcinoid, Microglandular Goblet Cell Carcinoma, Adenocarcinoid, Microglandular Mucinous Carcinoma, Crypt Cell Carcinoma, Mucinous Carcinoid)

Terminology and histogenesis

Clinical features

Gross pathology

Histology

Immunohistochemistry

High-Grade Carcinomas Ex Goblet Cell Carcinoma/Carcinoid

Prognosis and treatment of GCC and carcinomas ex GCC

Combined Tumors of the Appendix

Comparative Survival of Appendiceal Adenocarcinomas and Carcinoids

Other Rare Tumors and Lesions of the Appendix

Metastatic Disease Involving the Appendix

Table 15-4: Terminology for Mucinous Appendiceal Tumors and PMP

Table 15-5: Comparing the WHO 2010 and Our Preferred Terminology

Table 15-6: Classifications of Low-Grade Mucinous Neoplasia

Figure 15-38

Figure 15-39

Figure 15-40

Figure 15-41

Figure 15-42

Figure 15-43

Figure 15-44

Table 15-7: Comparison of classifications of pseudomyxoma peritonei

Figure 15-45

Figure 15-45

Figure 15-45

Figure 15-46

Figure 15-47

Figure 15-48

Figure 15-49

Figure 15-50

Figure 15-51

Figure 15-52

Figure 15-53

Figure 15-54

Figure 15-55

Figure 15-56

Chapter 16: Small and Large Bowel Structure: Developmental and Mechanical Disorders

Chapter 16 Introduction

Structure of the Small and Large Intestines

External Appearance

Small intestine

Large intestine

Internal Appearance

Small intestine

Large intestine

Histology

Small intestinal mucosa

Ileocecal valve

Large intestinal mucosa

Specialized cells in the small and larger bowel epithelium

Stem cells and kinetics

Pericrypt fibroblast sheath

Lamina propria and immune system

Muscularis mucosae

Submucosa

Muscularis propria

Nerve supply, myenteric plexi, and interstitial cells of Cajal

Serosa

Blood vessels

Lymphatics

Figure 16-1

Figure 16-2

Figure 16-3

Figure 16-4

Figure 16-5

Figure 16-6

Figure 16-7

Figure 16-8

Figure 16-9

Figure 16-10

Figure 16-11

Figure 16-13

Figure 16-12

Figure 16-14

Figure 16-16

Figure 16-15

Figure 16-17

Figure 16-18

Figure 16-19

Figure 16-20

Figure 16-21

Figure 16-22

Figure 16-23

Figure 16-24

Figure 16-25

Figure 16-26

Figure 16-27

Figure 16-28

Figure 16-29

Embryology and Development of Small and Large Intestine

Congenital Anomalies

Heterotopia and metaplasia

Malrotation, lack of fixation, and volvulus

Congenital hernias

Abnormalities of intestinal musculature

Atresia, Webs, Diaphragms, and Stenoses

Atresia

Webs, diaphragms, and stenoses

Enteric duplications

Simple duplications

Complications

Hemorrhage and infarction

Malignancy

Abdominothoracic dorsal enteric diverticula

Meckel’s diverticulum and anomalies of the vitelline duct

Tumors in Meckel’s diverticulum

Miscellaneous anomalies

Mechanical Disorders and Disorders of Propulsion

Obstruction

Neonatal necrotizing enterocolitis and related disorders

Figure 16-30

Figure 16-31

Figure 16-32

Figure 16-33

Figure 16-34

Figure 16-35

Figure 16-36

Figure 16-37

Figure 16-38

Table 16-1: Classification of Intestinal Atresias

Figure 16-39

Figure 16-40

Figure 16-41

Figure 16-42

Figure 16-43

Figure 16-44

Figure 16-45

Figure 16-46

Figure 16-47

Figure 16-48

Chapter 17: Small Bowel Mucosal Disease

Chapter 17 Introduction

Small Bowel Mucosal Disease

Normal Appearances and Artifacts

Normal Villous Architecture

Regional variations in mucosal biopsy appearances

Geographic variation

Tangential artifact

Traumatic artifact

Evaluation of a Small Bowel Biopsy Specimen

The Abnormal Small Bowel Biopsy Specimen

The mild lesion (Marsh type 1)

The moderate lesion (Marsh type 3a and 3b)

The severe flat lesion (Marsh type 3c)

Alternative terminology

Figure 17-2

Figure 17-1

Table 17-1: Overview Classification of Small Bowel Biopsy

Figure 17-3

Figure 17-4

Figure 17-5

Figure 17-6

Figure 17-7

Figure 17-8

Disorders Associated with Abnormal Small Bowel Biopsy Specimens

Table 17-2: Severe (Flat) Lesions: Nonspecific Histologya

Table 17-3: Severe Flat Lesions: Specific/Distinctive Histology

Table 17-4: Variably Severe Lesions: Nonspecific Histologya

Table 17-5: Variably Severe Lesions—Specific/Distinctive Histologya

Table 17-6: Normal Villous Architecture: Specific/Distinctive Histology

Disorders Associated with a Severe Flat Mucosa: Nonspecific and Specific/Distinctive Histology

Celiac Sprue (Syn. Celiac Disease, Gluten-sensitive Enteropathy, Nontropical Sprue)

Pathogenesis

Clinical features

Associations of celiac sprue

Endoscopic appearance

Pathology

Complications of celiac sprue

Dermatitis herpetiformis and celiac sprue

Relatives of patients with celiac sprue

Latent celiac sprue and preclinical disease

Celiac crisis

Rebiopsy in patients with celiac sprue

Refractory celiac sprue

Unclassified sprue

Collagenous sprue

Autoimmune Enteropathy

Pathogenesis

Clinical features

Pathology

Treatment and prognosis

Food Hypersensitivity

Definitions of gastrointestinal reactions to food

Morphologic reactions in food hypersensitivity

Tropical Sprue

Childhood Kwashiorkor (Protein-Calorie Malnutrition)

Microvillus Inclusion Disease/Familial Enteropathy (Hypoplastic Villous Syndrome, Congenital Microvillus Atrophy)

Congenital Tufting Enteropathy

Figure 17-9

Figure 17-10

Figure 17-11

Figure 17-12

Figure 17-13

Figure 17-14

Figure 17-15

Diseases Associated with Variably Severe Lesions with an Intraepithelial Lymphocytosis

Immunodeficiency Syndromes

Common variable immunodeficiency/hypogammaglobulinemia (late-onset immunodeficiency)

Selective IgA deficiency

Acquired immunodeficiency syndrome (AIDS)

Infectious Gastroenteritis

Bacterial Overgrowth Syndrome (Stasis Syndromes, Contaminated Bowel Syndrome)

Pathogenesis and clinical features

Pathology

Zollinger–Ellison Syndrome (Gastrinoma)/Acid Dumping

Acrodermatitis Enteropathica

Figure 17-16

Figure 17-17

Figure 17-18

Figure 17-19

Diseases Associated with Variably Severe Lesions: Diagnostic/Distinctive Histology

Whipple’s Disease

Pathogenesis and clinical features

Endoscopic findings

Pathology

Differential diagnosis

Eosinophilic Gastroenteritis

Pathogenesis and clinical features

Clinical diagnosis and differential diagnosis

Endoscopic findings and gross pathology

Pathology

Differential diagnosis

Crohn’s Disease

Nonspecific Ulcers

Primary Intestinal Lymphoma

Infections

Intestinal Lymphangiectasia

Waldenstrom’s Macroglobulinemia

Impaired Epithelial Cell Replacement

Severe vitamin B12 deficiency, folic acid deficiency, acute radiation reaction, and chemotherapy

Figure 17-20

Figure 17-21

Table 17-7: Comparison of Whipple’s Disease with Mycobacterium avium-intracellare (MAI)

Figure 17-22

Figure 17-23

Figure 17-24

Figure 17-25

Figure 17-26

Normal Villous Architecture: Specific/Distinctive Histology

Abetalipoproteinemia and Hypobetalipoproteinemia

Pathogenesis

Pathology

Amyloidosis

Lipid Storage Diseases

Chronic Granulomatous Disease

Figure 17-27

Figure 17-28

Miscellaneous Disorders

Enzyme Deficiency States

Disaccharidase deficiencies

Ornithine transcarbamylase deficiency

Brown Bowel Syndrome

Protein-losing Enteropathy

Figure 17-29

Case Examples of Biopsy Interpretation and Clinical Implications

Case 1: Refractory Sprue

Part A

Part B

Part C

Part D

Case 2: The Mild Mucosal Lesion

Part A

Part B

Part C

Part D

Chapter 18: Inflammatory Bowel Diseases

Chapter 18 Introduction

Table 18-1: Normal Colonoscopy and Abnormal Histology

Microscopic Colitis

Introduction

Epidemiology

Etiology and Pathogenesis

Clinical Features and Endoscopic Findings

Microscopic Pathology

Collagenous colitis

Lymphocytic colitis

Microscopic colitis and involvement of small intestine

Differential Diagnosis of Lymphocytic Colitis

Treatment and Prognosis

Microscopic Colitis Variants

Brainerd Diarrhea

Microscopic Colitis in Children

Microscopic Colitis and Idiopathic Inflammatory Bowel Diseases

Table 18-2: Medications Potentially Related to the Development of Lymphocytic Colitis

Table 18-3: Autoimmune Disorders Associated with Microscopic Colitis

Table 18-4: Comparison of Collagenous and Lymphocytic Colitis

Figure 18-1

Figure 18-2

Figure 18-3

Figure 18-4

Figure 18-5

Figure 18-6

Chronic Idiopathic Inflammatory Bowel Diseases

Introduction

Epidemiology

Etiology and Pathogenesis

Genetic factors

Environmental factors

Smoking and IBD

Appendectomy and IBD

The immune system

Ulcerative Colitis

Clinical features

Endoscopic features and gross pathology

Microscopic pathology

Focality/patchiness in ulcerative colitis

Appendiceal involvement

Rectal sparing

Ileal involvement in ulcerative colitis

Pan enteritis post colectomy for ulcerative colitis

Prestomal ileitis

Ulcerative colitis in children

Proctitis/ulcerative proctitis

Fulminant colitis and toxic megacolon

Rectum in ulcerative proctocolitis following ileorectal anastomosis

Effect of medical therapy on the pathology of ulcerative colitis

Ileal pouches and their complications including pouchitis

Other causes of pouch problems

When are pouch biopsies done?

Dysplasia and cancer in pouches

Other diseases affecting pouches

Crohn’s Disease

Introduction and definition

Clinical features and classification

Endoscopic features and gross pathology

Microscopic pathology

Anal and perianal disease

Management

Differentiation Between Ulcerative Colitis and Crohn’s Disease

Macroscopic differences

Microscopic differences

Indeterminate Colitis/IBD-Unclassified

Introduction

Pathology

Synchronous and Metachronous Crohn’s Disease and Ulcerative Colitis

Upper Gastrointestinal Tract Involvement in Inflammatory Bowel Disease

The role of upper gastrointestinal biopsies in differentiating Crohn’s disease from ulcerative colitis

Extragastrointestinal Manifestations of IBD

Hepatobiliary disorders

Treatment and Prognosis of IBD

Crohn’s Disease and Small Intestinal Transplantation

Colitis Associated with Primary Sclerosing Cholangitis

Table 18-5: Genomic Regions Significantly Associated with IBD in Genome-wide Association Studies of Patients of European Ancestry

Figure 18-7

Figure 18-8

Figure 18-9

Figure 18-10

Figure 18-11

Figure 18-12

Figure 18-13

Figure 18-14

Figure 18-15

Figure 18-16

Figure 18-17

Figure 18-18

Table 18-6: Scoring System for the Assessment of Severity in Ulcerative Colitis

Table 18-7a: Simplified Grading System for Chronicity and Activity in IBD (Option A)*

Table 18-7b: Simplified Grading System for Ulcerative Colitis (Option B)

Figure 18-19

Figure 18-20

Figure 18-21

Figure 18-22

Figure 18-23

Figure 18-24

Figure 18-25

Figure 18-26

Figure 18-27

Table 18-8: Causes of Proctitis

Table 18-9: Clinical Criteria for the Diagnosis of Fulminant Colitis

Figure 18-28

Figure 18-29

Figure 18-30

Figure 18-31

Figure 18-32

Figure 18-33

Table 18-10: Vienna and Montreal Classification for Crohn’s Disease

Figure 18-34

Figure 18-35

Figure 18-36

Figure 18-37

Figure 18-38

Figure 18-39

Figure 18-40

Figure 18-41

Table 18-11: Common Causes of Endoscopic Aphthoid Ulcers

Figure 18-42

Figure 18-43

Table 18-12: Granuloma-associated Diseases in the Gastrointestinal Tract

Figure 18-44

Figure 18-45

Figure 18-46

Figure 18-47

Figure 18-48

Figure 18-49

Figure 18-50

Figure 18-51

Figure 18-52

Figure 18-53

Figure 18-54

Figure 18-55

Table 18-13: Gross Features Which Can Help to Distinguish Between Ulcerative Colitis and Crohn’s Disease

Table 18-14: Microscopic Features for the Distinction Between Ulcerative Colitis, Crohn’s Disease, and Indeterminate Colitis in Fulminant Colitis

Figure 18-56

Figure 18-56

Figure 18-56

Figure 18-56

Figure 18-56

Figure 18-57

Figure 18-58

Figure 18-59

Figure 18-60

Table 18-15: Extragastrointestinal Complications Associated with Inflammatory Bowel Disease

IBD Mimics and Related Disorders

Diverticular Disease–associated Chronic Colitis (Diverticular Colitis)

Diversion Colitis

Focal Active Colitis

Incidental Terminal Ileitis and Cryptogenic Multifocal Ulcerous Stenosing Enteritis (CMUSE)

Reporting Ileitis

Neutrophil Function Defects and IBD

Hermansky-Pudlak Syndrome

Intestinal Behcet Disease

Epidemiology

Clinical features

Pathology

Cryptogenic Multifocal Ulcerous Stenosing Enteritis (CMUSE) (Chronic Non-specific Ulcer [CNSU] of the Small Bowel)

Obstructive Colitis

NK Cell Enteropathy

Eosinophilic Colitis

Figure 18-61

Figure 18-62

Figure 18-63

Figure 18-64

Figure 18-65

Figure 18-66

Figure 18-67

Figure 18-68

Figure 18-69

Table 18-16: Common Causes of Eosinophilic Colitis

Figure 18-70

Differential Diagnosis of Chronic Inflammatory Bowel Diseases

Infections

Infections in patients with IBD

Drug-induced Mucosal Injury

Ischemia

Miscellaneous

Table 18-18: Enteric Pathogens That Cause Inflammation Resembling IBD

Table 18-17: Differential Diagnosis Between Acute Self-limited or Infectious-type Colitis and IBD

Table 18-19: Some Causes of Right-sided Colitis

Figure 18-71

Figure 18-72

Malignancy in Inflammatory Bowel Disease

Introduction

Molecular Pathogenesis of Cancer in IBD

Dysplasia in IBD

Terminology of dysplastic lesions and their management

Gross pathology of dysplasia

Microscopic pathology of dysplasia

Distinction from reactive changes

The classification and grading of dysplasia

Grading dysplasia

Reproducibility of dysplasia diagnosis and grading

Summary of problematic issues related to dysplasia in IBD

Role of immunohistochemistry and other ancillary techniques

The clinical implications of dysplasia in ulcerative colitis

Cancer in IBD

Pathologic characteristics of colorectal cancer in colitis

Management of carcinoma complicating ulcerative colitis

Cancer of the small and large bowel in Crohn’s disease

Anal carcinoma in IBD

Carcinoma in Indeterminate colitis

Inflammatory bowel disease and other malignancies

Carcinoma after surgery

Table 18-20: Genetic Alterations in Colitis-associated and Sporadic CRC

Figure 18-73

Table 18-21: Macroscopic Appearance of IBD-associated Intraepithelial Neoplasia

Figure 18-74

Figure 18-75

Figure 18-76

Figure 18-77

Figure 18-78

Figure 18-79

Figure 18-80

Figure 18-81

Figure 18-82

Figure 18-83

Figure 18-84

Table 18-22: Biopsy Classification of Dysplasia in IBD: Comparison of the Two Common Systems in Use

Figure 18-85

Figure 18-86

Figure 18-87

Figure 18-88

Figure 18-89

Figure 18-90

Case Studies

Case 1

Case 2

Case 3

Case 4

Case 5

Case 6

Table 18-23: Systematic Approach to Biopsy Evaluation for Inflammatory Disorders of the Bowel

Figure 18-91

Figure 18-92

Figure 18-93

Figure 18-94

Figure 18-95

Figure 18-96

Drug-Associated, Iatrogenic and Miscellaneous Diseases of the Small and Large Intestines

Table 18-24: Effects of Some Drugs and Medications on the Intestines

Role of the Pathologist

Mucosal Changes

Antineoplastic Drugs and Drugs Causing Increased Apoptosis (“Apoptotic” Colopathy)

Acute toxic injury

Abnormal mitosis

Dysplasia mimics related to medication

Neutropenic enterocolitis (typhlitis, neutropenic appendicitis)

Figure 18-97

Figure 18-98

Figure 18-99

Figure 18-100

Figure 18-101

Figure 18-102

Idiopathic Small and Large Bowel Ulcers, Fibrosis, Strictures

Gross and Endoscopic Appearances

Histology

Nonsteroidal Anti-inflammatory Drugs

Clinical features

Pathogenesis

NSAIDs and small intestinal disease

NSAIDs and large intestinal disease

NSAIDs and diverticular disease

NSAIDs in idiopathic IBD

NSAIDs and left-sided colonic increase of eosinophils

Potassium Chloride

Table 18-25: Some Causes of Idiopathic Ulcers of the Intestine

Figure 18-103

Figure 18-104

Figure 18-105

Drug-Associated and Iatrogenic Vascular Injuries

Ischemia Following Angiography

Trauma to the vessel wall

Embolization

Anticoagulant-associated Hemorrhage and Hematoma

Retroperitoneal hematomas

Arterial or Venous Thrombosis

Drug-induced vasculitis

Estrogen and oral contraceptives

Oral contraceptive and IBD

Changes Secondary to Contrast Media

Perforation

Ischemic necrosis

Arterial Spasm

Vasopressin

Hypotensive and hypovolemic agents

Neonatal Perforation

Stevens-Johnson Syndrome

Figure 18-106

Figure 18-107

Iatrogenic and Drug-associated Inflammatory Changes

Pseudomembranes and Ischemic Colitis

Acute Infectious-type Colitis

Methyldopa

Iatrogenic and Drug-associated Granulomatous and Eosinophilic Enteritis/Colitis

Chronic Inflammation with/without Focal Active Colitis

Mycophenolate mofetil

Rituximab

Ipilimumab and anti PD-1

Anti-tumor necrosis factor (TNFa) drugs

Anti vascular endothelial growth factor receptor antibodies (bevacizumab)

Gold enteritis and colitis

5-Flucytosine

Penicillamine

Cimetidine

Isotretinoin

Figure 18-108

Figure 18-109

Figure 18-110

Changes due to Other Drugs and Chemicals

Changes Due to Enemas and Laxatives

Sodium phosphate (fleets enema) and bisacodyl

Hypertonic saline enemas

Chemical colitis mimicking ischemia induced by glutaraldehyde, hydrogen peroxide or peracetic acid

Soap

Polyethylene glycol

Changes Due to Laxatives

Cathartic colon

Myenteric plexus damage, pseudo-obstruction, and stercoral ulceration

Alcohol

Obstruction from Ingested Drugs

Pneumatosis Cystoides Intestinalis

Pathogenesis

Clinical features

Gross and endoscopic disease

Histology

Prognosis

Iatrogenic Gas Introduced at Endoscopy (Pseudolipomatosis)

Gross and endoscopic appearances

Histology

Figure 18-111

Figure 18-112

Figure 18-113

Figure 18-114

Figure 18-115

Figure 18-116

Other Miscellaneous Conditions, Disorders, and Diseases

Compensatory Changes

Sequelae of Instrumentation

Extrinsic Damage to the Bowel

Large-bowel Disease in Acute Pancreatitis

Colitis (and Enteritis) Cystica Profunda

Adaptation to Prolapse

Rectal Mucosal Prolapse from Other Causes

Enteritis and Colitis Cystica Superficialis

Malabsorption

Diarrhea

Chemotherapeutic agents

Mycophenolate mofetil

Proton pump inhibitors

Neoplasms (Following Immune Suppression and Radiation)

Figure 18-117

Table 18-26: Causes of Cystica Superficialis and Glandularis/Cystica Profunda Lesions

Benign Disorders Involving Histiocytes

Normal Histiocytes

Histiocytes Associated with Drug Use

Iron-containing macrophages

Pseudomelanosis coli (melanosis coli)

Gross and endoscopic appearances

Histology

Histiocytes Associated with Infections

Mycobacterium avium-intracellulare

Whipple’s disease

Malakoplakia and xanthogranulomatous inflammation

Histiocytes Associated with Storage Disease

Histiocytes Associated with Miscellaneous Disorder

Iron-containing macrophages

Muciphages

Xanthoma/xanthelasma

Black Pigments in the Terminal Ileum and Large Bowel

Aluminum silicate titanium-containing pigment in terminal ileum

Injected India ink as a tattoo

Table 18-27: Disorders Associated with an Excess of Abnormal Histiocytes/Macrophages

Figure 18-118

Figure 18-119

Figure 18-120

Figure 18-121

Table 18-28: The Involvement of Various Cell Types in a Range of Storage Diseases

Table 18-29: The Staining Patterns of the Neuronal Storage Material

Figure 18-122

Table 18-30: Iatrogenic Causes of Ischemia

Figure 18-123

Figure 18-124

Abnormal Deposits (Crystal Deposition)

Barium Granuloma

Oleogranulomas (Paraffinomas)

Radiologic Masses, Baroliths, and Acute Appendicitis

Mercury

Figure 18-125

Chapter 19: Enteric Infections and Associated Diseases

Chapter 19 Introduction

Introduction

Table 19-1: Important Enteric Infections Classified According to Major Classes or Organisms

Host Defense Mechanisms

Acidity of Gastric Juices

Intestinal Motility

Mucus

Normal Resident Bacterial Flora

Gut-Associated Immune System

Infection of the Normal Host

Mechanisms of Infection

Adherence, Initial Attachment, and Colonization

Tissue Invasion

Toxin Production

Enteroadherent and Other Mechanisms of Diarrhea

Persistence of Infection

Approach to Diagnosis of GI Infections

Introduction

Clinical Features

Endoscopic and Gross Findings

Stool Examination, Culture, and Other Studies

Biopsy Diagnosis

No change

Typical acute infectious (self-limited) colitis

Mild acute or chronic inflammation

Specific histologic pattern

Detection of specific organisms or their cytopathic changes

Special stains

Electron microscopy

Molecular tests

Gastrointestinal Infections in Specific Clinical Circumstances

Traveler’s diarrhea

Point outbreaks of infection

Health care–associated (“nosocomial”) infection

Oral–anal sexual practices

Table 19-2: Pathogens Causing Distinctive Cellular Changes or Tissue Reactions

Figure 19-1

Table 19-3: Infectious Agents for Which Monoclonal or Polyclonal Antibodies or Both Are Available for Immunofluorescence or Immunohistochemistry

Table 19-4: Infectious Agents for Which DNA Probes for In Situ Hybridization Assays Are Available

Table 19-5: Infectious Agents for Which PCR Assays Are Available

Table 19-6: Identification of Specific Enteropathogens Shown as Percentage of Total Cases in Studies of the Etiology of Traveler’s Diarrhea Carried Out in Latin America/Caribbean, Africa, and Southern Asia, 1973–2004

Table 19-7: Infections in Male Homosexuals and Sites of Involvement

Acute Infectious (Self-Limited) Colitis and Proctitis

Pathogenesis and Clinical Features

Exacerbations of Inflammatory Bowel Disease

Gross and Endoscopic Appearances

Histology

Diagnosis

Bacterial Infections

Vibrios

Epidemiology

Clinical features

Pathogenesis

Pathology

Diagnosis

Treatment and follow-up

Escherichia coli

Enterotoxigenic E. coli

Enteroinvasive E. coli

Enteropathogenic organisms

Enteroadherent E. coli

Enterohemorrhagic E. coli

Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura

Verotoxin-producing E. coli in inflammatory bowel disease

Shigella

Pathogenesis

Clinical features

Gross and endoscopic appearances

Histology

Diagnosis, differential diagnosis, and treatment

Salmonella

Classification of Salmonella

Salmonella enterocolitis

Fulminant infections

Salmonella infection in inflammatory bowel disease

Typhoid fever

Campylobacter

Pathogenesis

Clinical and endoscopic features

Gross and endoscopic findings

Histology

Complications

Aeromonas hydrophila, Plesiomonas shigelloides, and Edwardsiella tarda

Pathology

Legionella

Yersinia Infection

Pathogenesis and clinical features

Gross pathology and endoscopic appearances

Histology

Yersinia pseudotuberculosis infection

Klebsiella Granulomatis (Granuloma Inuinale)

Differential diagnosis

Prognosis

Clostridial Infections

Clostridium difficile

Antibiotic-associated colitis

Clostridium Perfringens (Previously Called C. welchii)

Enteritis necroticans

Clostridium botulinum

Neutropenic enterocolitis

Phlegmonous enterocolitis

Mycobacterial Infections

Mycobacterium tuberculosis and bovis

Mycobacterium avium-intracellulare

Gonococcal Proctitis

Gross and endoscopic appearances

Histology

Spirochetes

Syphilis

Intestinal spirochetosis

Brucella

Actinomyces

Rhodococcus equi (formerly known as Corynebacterium equi)

Chlamydia

Lymphogranuloma venereum

Chlamydial proctitis

Rickettsial Disease

Rocky mountain spotted fever

Table 19-8: The Various Pathotypes and the Common Clinical Syndromes Associated with E. coli

Figure 19-2

Figure 19-3

Figure 19-4

Figure 19-5

Figure 19-6

Figure 19-7

Figure 19-8

Figure 19-9

Figure 19-10

Figure 19-11

Figure 19-12

Figure 19-13

Figure 19-14

Figure 19-15

Figure 19-16

Figure 19-17

Table 19-9: Pathologic Features Differentiating Tuberculosis and Crohn’s Disease

Figure 19-18

Figure 19-19

Figure 19-20

Figure 19-21

Figure 19-22

Figure 19-23

Figure 19-24

Figure 19-25

Viral Infections

Herpesvirus Infections

Human herpesviridae infections

Herpes simplex virus

Cytomegalovirus infection

Measles

Enteric Viruses

Nonpathogenic enteric viruses

Pathogenic enteric viruses causing diarrhea

Rotavirus infections

Norovirus (formerly called Norwalk virus)

Enteric adenoviruses

Other causes of viral diarrhea

Figure 19-26

Figure 19-27

Figure 19-28

Figure 19-29

Figure 19-30

Figure 19-31

Parasitic Infections

Protozoa

Giardia

Amoeba

Potentially pathogenic protozoa

Dientamoeba fragilis

Balantidium coli

Blastocystis hominis

Coccidiosis

Nematodes

Ancylostomiasis

Angiostrongylus costaricensis

Anisakiasis

Ascariasis (round worm)

Capillariasis

Enterobiasis

Esophagostomiasis

Strongyloidiasis

Trichinosis

Trichuriasis

Trematodes

Schistosomiasis

Fasciolopsis buski

Cestodes

Diphyllobothrium latum (fish tapeworm)

Taenia saginata (beef tapeworm)

Taenia solium (pork tapeworm)

Hymenolepis nana (dwarf tapeworm)

Figure 19-32

Figure 19-33

Figure 19-34

Figure 19-35

Figure 19-36

Figure 19-37

Figure 19-38

Figure 19-39

Figure 19-40

Figure 19-41

Figure 19-42

Figure 19-43

Figure 19-44

Figure 19-45

Fungal Infections

Candidiasis

Histoplasmosis

Cryptococcosis

Phycomycosis/Mucormycosis

Aspergillosis

South American Blastomycosis

Pneumocystis

Figure 19-46

Figure 19-47

Figure 19-48

Figure 19-49

Acknowledgment

Chapter 20: Small and Large Bowel Polyps and Tumors

Chapter 20 Introduction

Introduction

Small Bowel Polyps

Nondysplastic Small Bowel Mucosal Polyps

Brunner’s gland nodules and polyps (“hyperplasia”)

Brunner’s gland adenoma

Heterotopic gastric mucosa

Heterotopic pancreatic tissue (myoepithelial hamartoma, ectopic pancreas, and adenomyoma)

Lymphoid nodules and tumors

Other duodenal mucosal bumps

Dysplastic Mucosal Polyps (Adenomas)

Clinical features

Gross and endoscopic appearances

Histology

Management

Serrated Polyps of the Small Bowel

Figure 20-1

Figure 20-2

Figure 20-3

Figure 20-4

Figure 20-5

Figure 20-6

Figure 20-7

Figure 20-8

Figure 20-9

Figure 20-10

Small Bowel Carcinoma

Introduction

Pathogenesis

Clinical features

Gross and endoscopic appearances

Histology

Immunophenotype and molecular pathology

Problems in diagnosis and management of early invasive carcinoma

Treatment

Staging and prognosis

Figure 20-11

Figure 20-12

Figure 20-13

Figure 20-14

Figure 20-15

Figure 20-16

Table 20-1: TNM Staging of Nonampullary Small Bowel Tumors

Table 20-2: Staging of Ampullary Tumors (Excluding Well-differentiated Endocrine Tumors)

Secondary Tumors of the Small Bowel

Miscellaneous Other Tumors and Tumor-Like Conditions

Large Bowel Polyps

Serrated/Hyperplastic Polyps

Introduction

Nomenclature and classification

Hyperplastic polyps

Sessile serrated polyps (synonym sessile serrated adenoma) (SSP, SSA, SSP/A)

Serrated polyps with dysplasia

Traditional serrated adenomas

Unclassified serrated polyps

Immunophenotype and molecular pathology of serrated polyps

Interobserver variability

Differential diagnosis and approach to diagnostic problems

Serrated polyp and associated mesenchymal tumors

Treatment and follow-up

Large Bowel Adenomas

Definition

Age, sex, and distribution

Invasive Carcinoma in Endoscopically Resected Polyps (Malignant Polyps)

Role of the pathologist

Gross and endoscopic appearances

Pathology

Histology

Problematic issues and approach to diagnosis

Histologic risk assessment of malignant polyps

Prognosis and management

Suggested follow-up for malignant polyps

Role of the pathologist in malignant polyps

Other Nondysplastic Polyps

Inflammatory polyps

Colonic mucosal–submucosal elongated polyp (mucosubmucosal polyp)

Polypoid prolapsing mucosal fold

Colonic elastosis and elastofibroma

Miscellaneous and other unclassifiable nonneoplastic polyps (benign unclassifiable mucosal bumps [BUMPs])

Other Distinct Polyps and Associated Polyposis Syndromes Involving the Small and Large Bowel

Peutz-Jeghers polyps and polyposis

Solitary Peutz-Jehjers polyps

Juvenile polyps and juvenile polyposis syndrome

Cowden’s syndrome

Cronkhite-Canada syndrome

Serrated/hyperplastic polyposis syndrome

Familial adenomatous polyposis (familial adenomatosis, familial polyposis coli) and its variants

Variants of FAP

MUTYH-associated polyposis (MYH-associated polyposis)

Lynch syndrome (HNPCC)

Multiple adenomas

Lymphoid polyposis with reactive lymphoid hyperplasia

Table 20-3: Classification of Serrated Polyps

Figure 20-18

Figure 20-17

Figure 20-20

Figure 20-19

Figure 20-21

Figure 20-22

Figure 20-23

Figure 20-24

Figure 20-25

Figure 20-26

Figure 20-27

Figure 20-28

Figure 20-29

Table 20-4: Serrated Polyps with Key Molecular Alterations

Figure 20-30

Table 20-5: Comparison of Microvesicular Hyperplastic Polyps with Sessile Serrated Polyps

Table 20-6: Comparison of Key Histologic Features Between Various Types of Serrated Polyps

Figure 20-31

Figure 20-32

Table 20-7: Guidelines for Surveillance Colonoscopy

Table 20-8: Gross Appearance of Polyps and Early Cancer-Paris Classification

Figure 20-33

Figure 20-34

Figure 20-35

Figure 20-36

Figure 20-37

Figure 20-38

Figure 20-39

Figure 20-40

Figure 20-41

Figure 20-42

Figure 20-43

Figure 20-44

Figure 20-45

Figure 20-47

Figure 20-46

Figure 20-48

Figure 20-49

Figure 20-50

Table 20-9: High-risk Histologic Features in Malignant Polyps That Require Colectomy

Figure 20-51

Figure 20-52

Figure 20-53

Figure 20-54

Figure 20-55

Figure 20-56

Table 20-10: Various Polyposis Syndromes of the GI Tract, Genes Involved, and Associated Cancer Risk

Figure 20-57

Figure 20-58

Figure 20-59

Figure 20-60

Figure 20-61

Figure 20-62

Figure 20-62

Figure 20-62

Figure 20-62

Figure 20-63

Figure 20-64

Figure 20-65

Figure 20-66

Figure 20-67

Figure 20-68

Table 20-11: Genotype–Phenotype Correlations in FAP

Figure 20-69

Table 20-12: Overview of Clinical Criteria for Lynch syndrome: Amsterdam I and II and Revised Bethesda Criteria

Figure 20-70

Figure 20-71

Colorectal Carcinoma

Introduction and Terminology

Incidence and Demographics

Predisposing Factors

Predisposing Conditions

Polyposis syndromes and family history of CRC

Prior colorectal neoplasia

IBD and chronic infection

Prior local irradiation

Prior cholecystectomy

Prior ureterosigmoidostomy

Other possible associations

Premalignant Lesions

Molecular Genetics and Pathogenesis

Chromosomal instability pathway

Microsatellite instability pathway

Methylation pathways

Other pathways

Major genes and signaling pathways involved in colorectal carcinogenesis

Cytogenetic abnormalities

Genotype–phenotype correlations in CRC

Growth and Development of Adenomas

The adenoma–carcinoma (dysplasia–carcinoma) sequence

Aberrant crypt foci

Growth and kinetics of colorectal adenomas and carcinomas

Spontaneous disappearance of colorectal adenomas and carcinomas

“De novo” carcinoma

Clinical Features

Distribution within the Large Bowel

Gross and Endoscopic Appearances

Handling the resected carcinoma specimen

Histology

Usual/conventional-type colorectal carcinoma

MSI-H Carcinoma

MSS CRCs with CpG island methylation

Post–Chemotherapy and Radiation Changes

Other Histologic Variants of Colon Carcinoma

Mucinous (colloid) carcinoma

Diffuse-type signet ring carcinomas

Goblet cell carcinoma (adenocarcinoid, goblet cell carcinoid, crypt cell carcinoma—see chapter 15, appendix)

Micropapillary colon carcinoma

Medullary carcinoma (lymphoepithelioma-like carcinoma)

Serrated adenocarcinoma

Clear cell carcinoma

Adenosquamous carcinoma

Squamous carcinoma and nonanal basaloid (cloacogenic) carcinoma

Carcinosarcoma (spindle cell, sarcomatoid or metaplastic carcinoma)

Choriocarcinoma-like carcinoma (“carcinoma with trophoblastic differentiation”)

Small cell carcinoma and composite adenoma/carcinoma–carcinoid tumors

Melanotic adenocarcinoma and malignant melanoma

Cribriform or comedocarcinoma

Rhabdoid tumors

Intramucosal carcinoma

Colon Cancer in the Young

Prognostic Factors for Colorectal Carcinoma

Clinical factors

Pathologic features influencing prognosis

Treatment and Prognosis

Figure 20-72

Figure 20-73

Table 20-13: Emerging Molecular Classification of CRC Based on Genotype–Phenotype Correlations

Figure 20-74

Figure 20-75

Figure 20-76

Figure 20-77

Figure 20-78

Figure 20-79

Figure 20-80

Figure 20-81

Figure 20-82

Figure 20-83

Figure 20-84

Figure 20-85

Figure 20-86

Figure 20-87

Figure 20-88

Figure 20-89

Figure 20-90

Figure 20-91

Figure 20-92

Figure 20-93

Figure 20-94

Figure 20-95

Figure 20-96

Figure 20-97

Figure 20-98

Figure 20-99

Figure 20-100

Figure 20-101

Figure 20-102

Figure 20-103

Figure 20-104

Table 20-14: Pathologic Staging for Colorectal Cancer

Table 20-15: Colon Cancer: Expanded Changes in AJCC Substaging for Stages II and III Based on Expanded SEER Data

Table 20-16: Rectal Cancer: Expanded Changes in AJCC Substaging for Stages II and III Based on Expanded SEER Data

Tumors in Urinary Conduits

Tumors in Ileal Conduits

Tumors at Ureterosigmoidostomy Sites

Polyps

Carcinomas

Management

Nephrogenic adenoma of the large intestine

Secondary Tumors of the Large Bowel

Figure 20-105

Miscellaneous Conditions Mimicking Carcinoma

Endometriosis

Figure 20-106

Figure 20-107

Chapter 21: The Anal Canal

Chapter 21 Introduction

Anatomic and Histologic Characteristics

Terminology Used for the Anus, Distal Rectum, and Perianal Skin and Problems with These Terms

Surface Anatomy

Musculature

Blood Supply

Lymphatic Supply

Microscopic Anatomy

Columnar zone

Anal transition zone

Squamous zone distal to the dentate line

Perianal skin

Anal ducts and glands

Nerves and Biopsy Implications for Hirschsprung’s Disease

Figure 21-1

Figure 21-2

Figure 21-3

Figure 21-4

Figure 21-5

Figure 21-6

Developmental Abnormalities

Table 21-1: Classification of Anorectal Anomalies

Hemorrhoids

Pathogenesis

Clinical Features

Histologic Features

Role of the Pathologist in Dealing with Hemorrhoidectomy Specimens

Figure 21-7

Figure 21-8

Perianal Hematoma (Clotted Venous Saccule)

Inflammatory Lesions

Fissures and Fistulae

Infections

Viral infections—herpes and papillomavirus

Syphilis

Lymphogranuloma venereum

Granuloma inguinale

Figure 21-9

Figure 21-10

Prolapse and Inflammatory Cloacogenic Polyps

Figure 21-11

Anal Tags (Fibroepithelial Polyps)

Figure 21-12

Tumors of the Anus

Epithelial Hyperplasia Without Dysplasia (Pseudoepitheliomatous Hyperplasia)

Condyloma Acuminatum

Verrucous Carcinoma (Giant Condyloma of Bushke and Lowenstein)

Anal Intraepithelial Neoplasia, Dysplasias, Including Bowen’s Disease of the Perianal Skin, and Their Relation to Human Papillomavirus infection

Use of biomarkers in AIN

Unsuspected AIN Found in Hemorrhoidectomy Specimens or in Tags or Other Polyps (Referred to as “Minor Surgical Specimens”)

Bowen’s Disease (Squamous Carcinoma In Situ) of Perianal Skin

Invasive Squamous Cell Carcinoma

Risk factors for anal squamous cell carcinoma

Premalignant lesions

Clinical features

Gross appearances

Histologic features

Biopsy of irradiated tumors

Spread, staging, treatment, and prognosis

Minimally invasive/Superficial carcinoma of the anal and perianal region

Treatment with chemotherapy, radiotherapy, or both

Role of the pathologist in anal squamous neoplasia

Adenomas and Adenocarcinoma

Anal gland hidrocystoma and adenoma

Surface colorectal-type adenomas

Adenocarcinoma

Anal gland carcinoma

Other unusual adenoma-like columnar dysplasias and carcinomas

Mucoepidermoid carcinoma

Malignant Melanoma

Mesenchymal Tumors

Figure 21-13

Figure 21-14

Figure 21-15

Figure 21-16

Figure 21-17

Figure 21-18

Figure 21-19

Figure 21-20

Figure 21-21

Figure 21-22

Figure 21-23

Figure 21-24

Figure 21-25

Figure 21-26

Figure 21-27

Lesions of the Perianal Skin

Keratoacanthoma

Bowen’s Disease (Squamous Cell Carcinoma In Situ) and Bowenoid Papulosis

Invasive Squamous Cell Carcinoma of the Perianal Skin

Basal Cell Carcinoma

Paget’s Disease

Lichen Sclerosus

Hidradenitis Suppurativa

Figure 21-28

Appendix

Remarks

 

An aparitie	2014
Autor	Robert Riddell MD, Dhanpat Jain MD
Dimensiuni	21.29 x 27.64 cm
Editura	LWW
Format	Hardcover
ISBN	9780781722162
Limba	Engleza
Nr pag	1488
Versiune digitala	DA