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(BQ) Part 1 book Lippincotts illustrated Q&A review of rubin''s pathology presentation of content: Cell injury, inflammation, repair, regeneration, and fibrosis, immunopathology, developmental and genetic diseases, environmental and nutritional pathology, hemodynamic disorders, infectious and parasitic diseases,...
Fenderson_Index.indd 360 7/13/2010 9:01:37 PM Lippincott’s Illustrated Review of Rubin's Pathology SECOND EDITION Bruce A Fenderson, PhD Professor of Pathology Department of Pathology, Anatomy and Cell Biology Jefferson Medical College Thomas Jefferson University Philadelphia, Pennsylvania David S Strayer, MD, PhD Professor of Pathology Department of Pathology, Anatomy and Cell Biology Jefferson Medical College Thomas Jefferson University Philadelphia, Pennsylvania Raphael Rubin, MD Professor of Pathology Department of Pathology, Anatomy and Cell Biology Jefferson Medical College Thomas Jefferson University Philadelphia, Pennsylvania Emanuel Rubin, MD Founder and Consulting Editor, Rubin’s Pathology Recipient of the Tom Kent Award for Excellence in Pathology Education Gonzalo E Aponte Distinguished Professor Department of Pathology, Anatomy and Cell Biology Jefferson Medical College Thomas Jefferson University Philadelphia, Pennsylvania Fenderson_FM.indd i 7/13/2010 10:04:50 AM Acquisitions Editor: Susan Rhyner Product Manager: Catherine Noonan Vendor Manager: Bridgett Dougherty Manufacturing Manager: Margie Orzech Designer: Doug Smock Compositor: SPi Technologies Second Edition Copyright © 2011 Lippincott Williams & Wilkins 351 West Camden Street Baltimore, MD 21201 Two Commerce Square, 2001 Market Street Philadelphia, PA 19103 Printed in China All rights reserved This book is protected by copyright No part of this book may be reproduced or transmitted in any form or by any means, including as photocopies or scanned-in or other electronic copies, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews Materials appearing in this book prepared by individuals as part of their official duties as U.S government employees are not covered by the abovementioned copyright To request permission, please contact Lippincott Williams & Wilkins at Two Commerce Square, 2001 Market Street, Philadelphia, PA 19103, via email at permissions@lww.com or via website at lww.com (products and services) Library of Congress Cataloging-in-Publication Data Lippincott’s illustrated Q & A review of Rubin’s pathology / Bruce A Fenderson [et al.] — 2nd ed p ; cm Other title: Illustrated Q & A review of Rubin’s pathology Other title: Lipppincott’s illustrated Q and A review of Rubin’s pathology Rev ed of: Lippincott’s review of pathology / Bruce A Fenderson, Raphael Rubin, Emanuel Rubin c2007 A learning companion to 5th and 6th ed of Rubin’s pathology Includes index Summary: “Lippincott’s Illustrated Review of Rubin’s Pathology, Second Edition offers up-to-date, clinically relevant board-style questions-perfect for course review and board prep! Approximately 1,000 multiple-choice questions with detailed answer explanations cover frequently tested topics in general and systemic pathology The book is heavily illustrated with photos in the question or answer explanation Online access to the questions and answers provides flexible study options”—Provided by publisher ISBN 978-1-60831-640-3 (pbk.) Pathology—Examinations, questions, etc I Fenderson, Bruce A II Fenderson, Bruce A Lippincott’s review of pathology III Rubin’s pathology IV Title: Illustrated Q & A review of Rubin’s pathology V Title: Lipppincott’s illustrated Q and A review of Rubin’s pathology [DNLM: Pathology—Examination Questions QZ 18.2 L765 2011] RB31.F46 2011 616.07076—dc22 2010025179 DISCLAIMER Care has been taken to confirm the accuracy of the information present and to describe generally accepted practices However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication Application of this information in a particular situation remains the professional responsibility of the practitioner; the clinical treatments described and recommended may not be considered absolute and universal recommendations The authors, editors, and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with the current recommendations and practice at the time of publication However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions This is particularly important when the recommended agent is a new or infrequently employed drug Some drugs and medical devices presented in this publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings It is the responsibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice To purchase additional copies of this book, call our customer service department at (800) 638-3030 or fax orders to (301) 223-2320 International customers should call (301) 223-2300 Visit Lippincott Williams & Wilkins on the Internet: http://www.lww.com Lippincott Williams & Wilkins customer service representatives are available from 8:30 am to 6:00 pm, EST Fenderson_FM.indd ii 7/13/2010 10:04:51 AM Dedication We dedicate this book to our many teachers and colleagues for generously sharing their time and knowledge, and to all students of medicine for their intellectual stimulation and passion for learning Fenderson_FM.indd iii 7/13/2010 10:04:51 AM Preface Lippincott’s Illustrated Q&A Review of Rubin’s Pathology presents the key concepts of modern pathology in the form of clinical vignette-style questions Using the format of the National Board of Medical Examiners (NBME), the questions address the major topics in general and systemic pathology presented in Rubin’s Pathology: Clinicopathologic Foundations of Medicine In addition to being a learning companion to this textbook, these questions will serve as a standalone resource for self-assessment and board review The questions are prepared at a level appropriate for second-year medical students They provide a roadmap for students completing their courses in pathology and preparing for the United States Medical Licensing Examination (USMLE) Students in the allied health sciences (e.g., nursing and physical therapy) will also find considerable didactic value in clinical vignette-style questions Clinical vignette-style questions strengthen problem-solving skills Students must integrate clinical and laboratory data, thereby simulating the practice of pathology and medicine in general Case-based questions probe a level of competency that is expected for success on national licensing examinations Given below are key features of this text: • Multiple choice questions follow the USMLE template Case-based questions include (1) patients’ demographics, (2) clinical history, (3) physical examination findings, and (4) results of diagnostic tests and procedures Each clinical vignette is followed by a question stem that addresses a key concept in pathology • Questions frequently involve “two-step” logic—a strategy that probes the student’s ability to integrate basic knowledge into a clinical setting The answer choices appear homogeneous and are listed alphabetically to avoid unintended cueing • Over 200 full-color images link clinical and pathologic findings • Answers are linked to the clinical vignettes and address key concepts Incorrect answers are explained in context • Normal laboratory reference values are included for key laboratory tests • As an additional test-taking practice tool, the questions are also presented in an electronic format on our connection Web site (http://thePoint.lww.com/LIQARpathology2) Questions can be presented in both “quiz” and “test” modes In quiz mode, students receive instant feedback regarding the correctness of their answer choice, along with a rationale The test mode helps familiarize the user with the computer-generated USMLE experience We hope that this review of pathology will encourage students to think critically and formulate their own questions concerning mechanisms of disease We are mindful of the words of e e Cummings, who wrote “always the beautiful answer who asks a more beautiful question.” We wish our students success in their learning adventure Most importantly, have fun with pathology Bruce A Fenderson Raphael Rubin David S Strayer Emanuel Rubin iv Fenderson_FM.indd iv 7/13/2010 10:04:51 AM Acknowledgments The contributions of the editors and authors of Rubin’s Pathology: Clinicopathologic Foundations of Medicine, 5th and 6th editions were invaluable in the preparation of this text We are particularly indebted to Dr Ivan Damjanov and Dr Hector Lopez for their contributions Finally, we gratefully acknowledge the staff at Lippincott Williams & Wilkins for their expert help with manuscript preparation v Fenderson_FM.indd v 7/13/2010 10:04:51 AM Contents Preface .iv Acknowledgments v Chapter Cell Injury 01 Chapter Inflammation 14 Chapter Repair, Regeneration, and Fibrosis 25 Chapter Immunopathology 32 Chapter Neoplasia 40 Chapter Developmental and Genetic Diseases 52 Chapter Hemodynamic Disorders 63 Chapter Environmental and Nutritional Pathology 73 Chapter Infectious and Parasitic Diseases 81 Chapter 10 Blood Vessels 98 Chapter 11 The Heart 111 Chapter 12 The Respiratory System 125 Chapter 13 The Gastrointestinal Tract 142 Chapter 14 The Liver and Biliary System 160 Chapter 15 The Pancreas 175 Chapter 16 The Kidney 180 Chapter 17 The Lower Urinary Tract and Male Reproductive System 198 Chapter 18 The Female Reproductive System 210 Chapter 19 The Breast 225 Chapter 20 Hematopathology 234 Chapter 21 The Endocrine System 253 Chapter 22 Obesity, Diabetes Mellitus, Metabolic Syndrome 267 Chapter 23 The Amyloidoses 271 Chapter 24 The Skin 276 vi Fenderson_FM.indd vi 7/13/2010 10:04:51 AM Contents vii Chapter 25 The Head and Neck 283 Chapter 26 Bones, Joints, and Soft Tissues 291 Chapter 27 Skeletal Muscle 301 Chapter 28 The Nervous System 306 Chapter 29 The Eye 328 Chapter 30 Cytopathology 335 Appendix A: Normal Reference Range 340 Appendix B: Common Abbreviations 342 Appendix C: Figure Credits 343 Index 351 Fenderson_FM.indd vii 7/13/2010 10:04:52 AM Fenderson_FM.indd viii 7/13/2010 10:04:52 AM The Gastrointestinal Tract 20 21 22 A 45-year-old woman presents with a 6-month history of fatigue and swelling in her neck Physical examination shows a goiter A CBC discloses megaloblastic anemia and a normal reticulocyte count Additionally, there is an elevated serum level of TSH and antithyroid antibodies Needle aspiration of the left lobe of the thyroid reveals benign follicular cells and numerous lymphocytes Anemia in this patient is most likely caused by antibodies directed to which of the following targets? (A) Chief cells (B) Intrinsic factor (C) Paneth cells (D) TSH receptor (E) Vitamin D A 45-year-old man describes burning epigastric pain to hours after eating Foods, antacids, and over-the-counter medications provide no relief, and prescribed inhibitors of acid secretion are only moderately effective Recently, the patient noticed that his stools were black Physical examination reveals abdominal tenderness The blood pressure is 120/80 mm Hg in the supine position and 90/50 mm Hg sitting up The patient complains of lightheadedness upon returning to a standing position CBC shows a hemoglobin of 6.3 g/dL Endoscopy reveals multiple gastric and duodenal ulcers Epigastric pain and anemia are most likely related to a neoplasm arising in which of the following anatomic locations? (A) Adrenal medulla (B) Ampulla of Vater (C) Duodenum (D) Esophagus (E) Pancreas A 60-year-old man presents with an 8-week history of progressive weight loss, nausea, and upper abdominal pain that does not respond to antacids or H2-receptor antagonists Laboratory studies show iron-deficiency anemia Gastroscopy reveals a crater-like, ulcerated lesion in the antrum, with raised, irregular, and indurated margins The patient undergoes partial gastrectomy and the surgical specimen is shown in the image Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) Acute erosive gastritis Adenocarcinoma Curling ulcer Linitis plastica Peptic ulcer disease 23 A 58-year-old woman presents with a 2-month history of abdominal discomfort and dark stools Physical examination shows pallor but no evidence of jaundice Laboratory studies disclose a microcytic, hypochromic anemia, with a hemoglobin level of 6.7 g/dL A barium swallow radiograph reveals a “leather bottle” appearance of the stomach Microscopic examination shows diffusely infiltrating malignant cells, many of which are “signet ring” cells, in the stomach wall Which of the following is the most likely diagnosis? (A) Fungating adenocarcinoma (B) Gastric leiomyosarcoma (C) Gastric lymphoma (D) Linitis plastica (E) Ménétrier disease 24 A 42-year-old man presents with long-standing abdominal pain after meals, which is relieved by over-the-counter antacids The patient has lost kg (20 lb) in the past year Physical examination reveals peripheral edema and ascites Laboratory studies show decreased serum albumin but normal serum levels of transaminases and gastrin Gross and microscopic examination of this patient’s stomach would most likely show which of the following pathologic changes? (A) Atrophic gastritis (B) Enlarged rugal folds (C) Intestinal metaplasia (D) Multiple hemorrhagic ulcers (E) Proliferation of neuroendocrine cells 25 A 55-year-old woman complains of upper gastrointestinal pain and tarry stools Upper endoscopy shows a firm, smooth, yellowish submucosal ulcerated mass in the stomach Gastroscopic biopsy reveals spindle cells with vacuolated cytoplasm The mass is removed, and the surgical specimen is shown in the image Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) Fenderson_Chap13.indd 145 145 Gastric adenocarcinoma Gastric lymphoma Gastrointestinal stromal tumor Peptic ulcer Tubular adenoma 7/12/2010 2:10:46 PM 146 26 Chapter 13 A 56-year-old woman comes to the physician after noticing multiple lumps in her neck Physical examination shows enlarged and nontender supraclavicular lymph nodes Upper endoscopy discloses thickening of the gastric mucosa, without an obvious tumor The results of gastric biopsy are shown in the image Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) 27 28 A 60-year-old man presents with epigastric pain after meals, with some nausea and vomiting A burning sensation in the midepigastrium is relieved by antacids and H2 antagonists Upper endoscopy demonstrates paired ulcers on both walls of the proximal duodenum Which of the following represents the most common complication of this patient’s duodenal disease? (A) Bleeding (B) Malignant transformation (C) Obstruction (D) Perforation (E) Peritonitis 29 A 45-year-old woman presents with sudden attacks of wheezing, shortness of breath, and episodic hot flashes She also reports abdominal cramps and diarrhea Physical examination shows facial redness, pitting edema of the lower legs, and a murmur of tricuspid regurgitation A 24-hour urine specimen contains elevated levels of 5-hydroxyindoleacetic acid (5-HIAA) A CT scan of the abdomen demonstrates multiple 1- to 2-cm nodules in distal ileum A small bowel resection is performed (shown in the image) The arrows point to submucosal tumors Microscopic examination shows nests of cells with round and uniform nuclei Which of the following is the most likely diagnosis? Adenocarcinoma Gastric lymphoma Leiomyosarcoma Linitis plastica Ménétrier disease A 23-year-old woman with a history of an eating disorder complains of vomiting, nausea, and severe abdominal pain Physical examination shows abdominal distension and constipation An X-ray film of the abdomen reveals air-fluid levels and a hyperlucent shadow at the epigastric area The material obstructing the gastrointestinal tract is removed surgically and shown Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) 30 (A) (B) (C) (D) (E) Diverticulum of the stomach Gastric lymphoma Phytobezoar Trichobezoar Volvulus of the stomach Fenderson_Chap13.indd 146 Carcinoid tumor Mediterranean intestinal lymphoma Mucosa-associated lymphoid tissue (MALT) lymphoma Peutz-Jeghers syndrome Whipple disease A 5-year-old girl is brought to the physician after her parents noticed red blood in her stool Physical examination reveals mucocutaneous pigmentation Small bowel radiography discloses multiple, small- to medium-sized polyps that are diagnosed pathologically as hamartomas Which of the following is the most likely diagnosis? (A) Congenital teratoma (B) Hyperplastic polyp (C) Peutz-Jeghers polyp (D) Tubular adenoma (E) Villous adenoma 7/12/2010 2:10:48 PM The Gastrointestinal Tract 31 A 55-year-old man undergoes routine colonoscopy A small, raised, mucosal nodule measuring 0.4 cm in diameter is identified in the rectum and resected The surgical specimen is shown in the image Microscopic examination reveals goblet cells and absorptive cells with exaggerated crypt architecture, but no signs of nuclear atypia Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) 32 Adenocarcinoma Hyperplastic polyp Inflammatory polyp Peutz-Jeghers polyp Villous adenoma A 65-year-old woman undergoes routine colonoscopy During the procedure, a 2-cm mass is identified in the rectosigmoid region and resected The surgical specimen is shown in the image Microscopic examination shows irregular crypts lined by pseudostratified epithelium with hyperchromatic nuclei, without dysplastic features Which of the following is the most likely diagnosis for this patient’s colonic lesion? (A) (B) (C) (D) (E) Fenderson_Chap13.indd 147 33 Adenocarcinoma Carcinoid tumor Hyperplastic polyp Tubular adenoma Villous adenoma A 63-year-old woman complains of rectal bleeding of week in duration Laboratory studies show hypochromic, microcytic anemia (hemoglobin = 7.6 g/dL and MCV = 70 μm3) Colonoscopy reveals a large polypoid mass, which is removed (surgical specimen shown in the image) The arrow points to a malignant tumor The patient asks about the relative risk of cancer arising in various types of gastrointestinal polyps Which of the following types of colonic polyps is most likely to undergo malignant transformation? (A) (B) (C) (D) (E) 34 147 Hyperplastic polyp Lymphoid polyp Peutz-Jeghers polyp Tubular adenoma Villous adenoma A 59-year-old man complains of progressive weakness His friends have noticed that he has become pale, and he reports that his stools are tinged with blood On abdominal palpation, there is fullness in the right lower quadrant Laboratory studies show iron-deficiency anemia, with a hemoglobin level of 7.4 g/dL Stool specimens are positive for occult blood Colonoscopy reveals an elevated and centrally ulcerated lesion of the sigmoid colon The biopsy is shown in the image Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) Adenocarcinoma Carcinoid tumor Gastrointestinal stromal tumor Lymphoma Mucinous cystadenoma 7/12/2010 2:10:52 PM 148 35 Chapter 13 A portion of the large bowel was removed from a 34-year-old man with a familial disease that affects his gastrointestinal tract The surgical specimen is shown in the image This patient most likely carries a germline mutation in which of the following genes? (A) (B) (C) (D) (E) 36 A 60-year-old woman complains of increasing abdominal girth of weeks in duration Physical examination discloses ascites, and cytologic examination of the fluid reveals malignant cells Exploratory laparotomy shows multiple tumor nodules on the serosal surface of the intestines Which of the following is the most likely diagnosis? (A) Carcinoid tumor (B) Gastrointestinal stromal tumor (C) Liposarcoma (D) MALToma (E) Metastatic carcinoma 38 A 34-year-old man with AIDS presents with a 3-month history of constipation and lower abdominal pain The patient has a history of chronic diarrhea and persistent cough Recently, he noticed blood in his stool Laboratory studies reveal mild irondeficiency anemia Stool specimens are positive for occult blood A CBC shows a CD4 count of less than 50/μL Sigmoidoscopy discloses a mass in the rectosigmoid region In addition to B-cell lymphoma, this patient is at increased risk of developing which of the following tumors of the gastrointestinal (GI) tract? (A) Carcinoid tumor (B) Colonic adenocarcinoma (C) Kaposi sarcoma (D) Leiomyosarcoma (E) Melanoma 39 A 27-year-old woman presents with a 9-month history of bloody diarrhea and crampy abdominal pain Three weeks ago, she noticed that her left knee was swollen, red, and painful Her temperature is 38°C (101°F), respirations are 32 per minute, and blood pressure is 130/90 mm Hg Abdominal palpation reveals tenderness over the left lower quadrant Laboratory studies show moderate anemia, with a hemoglobin level of 9.3 g/dL Microscopic examination of the stool reveals numerous red and white blood cells A diffusely red, bleeding, friable colonic mucosa is visualized by colonoscopy The colon is subsequently removed and the surgical specimen is shown in the image Which of the following is the most likely diagnosis? APC C-myc DCC p53 Ras A 65-year-old woman presents with a 3-month history of diarrhea and abdominal pain She has lost kg (20 lb) in the past months The patient had two benign colonic polyps removed years ago Laboratory studies reveal mild iron-deficiency anemia, and stool specimens are positive for occult blood Sigmoidoscopy demonstrates an ulcerated mass, and a biopsy shows malignant glands A segment of the colon is resected, and the surgical specimen is shown in the image Based on current models of colonic carcinogenesis, which of the following genes was most likely mutated in the transition from benign adenoma to carcinoma in this patient? (A) (B) (C) (D) (E) 37 BRCA1 C-myc p53 Ras VHL Fenderson_Chap13.indd 148 (A) (B) (C) (D) (E) Adenocarcinoma Carcinoid tumor Crohn disease Pseudomembranous colitis Ulcerative colitis 7/12/2010 2:10:56 PM The Gastrointestinal Tract 40 41 42 43 The patient described in Question 39 is at increased risk of developing which of the following complications? (A) Adenocarcinoma (B) Fistula (C) Granulomatous lymphadenitis (D) Transmural inflammation (E) Volvulus A 44-year-old woman complains of having yellow eyes, dark urine, and recurrent fever for about months She has a long history of chronic diarrhea On physical examination, the patient is thin and jaundiced The liver edge descends cm below the right costal margin and is nontender Laboratory studies show elevated serum bilirubin of 3.8 mg/dL, normal levels of AST and ALT, and an elevated level of alkaline phosphatase (440 U/dL) Endoscopic retrograde cholangiopancreatography demonstrates a beaded appearance of the extrahepatic biliary tree Which of the following is the most likely underlying cause of diarrhea in this patient? (A) Amebiasis (B) Amyloidosis (C) Carcinoma of the ampulla of Vater (D) Celiac sprue (E) Ulcerative colitis A 25-year-old woman is brought to the emergency room with symptoms of acute intestinal obstruction The patient has an 8-month history of blood-tinged diarrhea and cramping abdominal pain Her temperature is 38°C (101°F), and respirations are 32 per minute There is abdominal tenderness to palpation Laboratory studies show moderate anemia, with serum hemoglobin of 9.3 g/dL Microscopic examination of the stool reveals numerous RBCs and WBCs A CT scan of the abdomen shows massive distention of the transverse colon Which of the following is the most likely underlying cause of this patient’s colonic disorder? (A) Adenocarcinoma (B) Carcinoid tumor (C) Crohn disease (D) Pseudomembranous colitis (E) Ulcerative colitis A 21-year-old man is brought to the emergency room with symptoms of acute intestinal obstruction His temperature is 38°C (101°F), respirations are 25 per minute, and blood pressure is 120/80 mm Hg Physical examination reveals a mass in the right lower abdominal quadrant The patient subsequently undergoes surgery, and a segmental lesion involving the terminal ileum is resected (shown in the image) Which of the following is the most likely diagnosis? Fenderson_Chap13.indd 149 (A) (B) (C) (D) (E) 149 Adenocarcinoma Carcinoid tumor Crohn disease Pseudomembranous colitis Ulcerative colitis 44 A 24-year-old man is brought to the emergency room with symptoms of acute intestinal obstruction His temperature is 38°C (101°F), respirations are 25 per minute, and blood pressure is 120/80 mm Hg Physical examination reveals a mass in the right lower abdominal quadrant At laparoscopy, there are numerous small bowel strictures and a fistula extending into a loop of small bowel Which of the following is the most likely diagnosis? (A) Adenocarcinoma (B) Carcinoid tumor (C) Crohn disease (D) Pseudomembranous colitis (E) Ulcerative colitis 45 A 30-year-old woman presents with days of abdominal cramping and diarrhea Her temperature is 38°C (101°F), respirations are 32 per minute, and blood pressure is 100/65 mm Hg Stool culture shows a toxigenic Escherichia coli infection Which of the following best explains the pathogenicity of this organism in this patient? (A) Destruction of Peyer patches (B) Invasion of the mucosa of the colon (C) Invasion of the mucosa of the ileum (D) Stimulation of acute inflammation in the superficial bowel mucosa (E) Stimulation of fluid transport into the lumen of the intestine 46 A 1-year-old girl is brought to the emergency room by her parents who report that she had a fever and diarrhea for days The child’s temperature is 38°C (101°F) The CBC shows a normal WBC count and increased hematocrit Which of the following microorganisms is the most likely cause of diarrhea in this young child? (A) Cytomegalovirus (B) Rotavirus (C) Salmonella typhi (D) Shigella dysenteriae (E) Yersinia jejuni 47 A 70-year-old man is rushed to the emergency room complaining of severe abdominal pain and rectal bleeding of hours in duration He has a history of coronary artery disease Bowel sounds are absent on physical examination A CT scan of the abdomen shows distention of the stomach and air-fluid levels in the small bowel Abdominal pain and bleeding in this patient most likely involved acute occlusion of which of the following arteries? (A) Celiac trunk (B) Gastroduodenal artery (C) Inferior mesenteric artery (D) Inferior rectal artery (E) Superior mesenteric artery 7/12/2010 2:11:00 PM 150 Chapter 13 48 A 16-year-old girl complains of chronic abdominal distention, flatulence, and diarrhea after drinking milk Elimination of milk and other dairy products from the patient’s diet relieves these symptoms This example of malabsorption is caused by a functional deficiency of which of the following enzymes associated with the intestinal brush border membrane? (A) Disaccharidase (B) Glycogen phosphorylase (C) Hyaluronidase (D) Mannosidase (E) Sphingomyelinase 49 A 2-year-old girl with a history of chronic constipation since birth is brought to the emergency room because of nausea and vomiting Physical examination shows marked abdominal distension Abdominal radiography reveals distended bowel loops with a paucity of air in the rectum A rectal biopsy shows an absence of ganglion cells Which of the following is the most likely diagnosis? (A) Acquired megacolon (B) Anorectal stenosis (C) Hirschsprung disease (D) Imperforate anus (E) Rectal atresia 50 51 A 25-year-old woman presents with persistent bloody diarrhea of weeks’ duration She has experienced severe abdominal cramping for the past days Her temperature is 38°C (101°F), respirations are 22 per minute, and blood pressure is 120/70 mm Hg Physical examination reveals abdominal tenderness and mild abdominal distension Bowel sounds are diminished Laboratory studies show mild hypochromic, normocytic anemia Stool cultures are negative for pathogens, and no ova or parasites are detected A blood test for Clostridium difficile toxin is negative Rectosigmoidoscopy shows hemorrhagic mucosal lesions in the distal colorectal region A biopsy of the colon reveals crypt abscesses, basal lymphoplasmacytosis and crypt distortion Which of the following represents the most common extraintestinal manifestation of the colonic disorder in this patient? (A) Arthritis (B) Cystitis (C) Gastritis (D) Pancreatitis (E) Sepsis 52 A 74-year-old woman presents with weeks of left lower quadrant abdominal pain, changes in bowel habits, and intermittent fever Her temperature is 38°C (101°F), respirations are 19 per minute, and blood pressure is 130/80 mm Hg Physical examination shows left lower quadrant tenderness A CBC reveals neutrophilia An abdominal-pelvic ultrasound examination is normal Which of the following is the most likely diagnosis? (A) Appendicitis (B) Diverticulitis (C) Ovarian carcinoma (D) Renal colic (E) Uterine leiomyoma 53 A 9-year-old boy undergoes emergency surgery for presumptive acute appendicitis During the operation, the surgeon notices that the boy’s ileocecal lymph nodes are enlarged and matted together One of the nodes is sent for a frozen section The pathologist finds granulomatous inflammation with central necrosis The specimen is cultured Which of the following pathogens is the most likely cause of lymphadenopathy in this patient? (A) Campylobacter jejuni (B) Shigella dysenteriae (C) Toxigenic E coli (D) Vibrio cholerae (E) Yersinia enterocolitica 54 A 36-year-old man presents with fever and painful joints for weeks Physical examination shows skin pigmentation, glossitis, angular cheilitis, and generalized lymphadenopathy The patient has lost kg (20 lb) over the past months He reports that his stools are pale and foul smelling Blood cultures are negative The patient is started on antibiotic therapy and exhibits remarkable clinical improvement Biopsy of the small intestine shows marked distortion of the intestinal villi, and a periodic acid-Schiff stain reveals large, foamy macrophages filled with glycoprotein-rich granules (shown in the image) Which of the following is the most likely diagnosis? A 10-year-old boy is brought to the emergency room after 48 hours of fever and severe abdominal pain He had developed edema of the legs several weeks previously The temperature on admission is 38.7°C (103°F) Physical examination shows rebound tenderness, guarding, and ascites An abdominal tap returns numerous segmented neutrophils This child’s spontaneous bacterial peritonitis is most often associated with which of the following underlying conditions? (A) Celiac sprue (B) Diverticulosis (C) Hirschsprung disease (D) Meconium ileus (E) Nephrotic syndrome Fenderson_Chap13.indd 150 7/12/2010 2:11:01 PM The Gastrointestinal Tract (A) (B) (C) (D) (E) 55 56 57 Angiodysplasia of ileum Crohn disease Ménétrier disease Peutz-Jeghers syndrome Whipple disease Which of the following cellular/biochemical mechanisms best explains the pathogenesis of malabsorption in the patient described in Question 54? (A) Bile salt inactivation (B) Blind loop syndrome (C) Extrahepatic cholestasis (D) Impaired mucosal function (E) Obstruction of the common bile duct A 4-year-old girl is brought to the physician because her parents noticed that she has been having pale, fatty, foul-smelling stools The patient is at the 50th percentile for height and 10th percentile for weight Her symptoms respond dramatically to a gluten-free diet Which of the following is the most likely diagnosis? (A) Celiac sprue (B) Cystic fibrosis of the pancreas (C) Ménétrier disease (D) Tropical sprue (E) Whipple disease the terminal bowel to which of the following anatomic structures? (A) Ganglia in the wall of the rectum (B) Inferior mesenteric artery (C) Levator ani muscle (D) Muscularis mucosae of rectum (E) Urachus 59 In addition to anorectal malformation, the infant described in Question 58 is most likely to have which of the following birth defects? (A) Cleft lip/cleft palate (B) Congenital pyloric stenosis (C) Esophageal atresia (D) Gastrointestinal fistula (E) Persistent urachus 60 A 2-year-old boy is brought to the emergency room with a 48-hour history of nausea, vomiting, and abdominal discomfort Physical examination reveals right lower quadrant guarding Ultrasound examination of the abdomen reveals a 2-cm mass in the right iliac fossa A segment of the small intestine is removed (shown in the image) Which of the following best describes this pathologic finding? A 53-year-old woman complains of acute diarrhea and severe abdominal pain She was recently treated with broad-spectrum antibiotics for community-acquired pneumonia A CBC shows a WBC count of 24,000/μL The patient subsequently develops septic shock and dies A portion of her colon is shown at autopsy These findings are typical of which of the following gastrointestinal diseases? (A) (B) (C) (D) (E) (A) (B) (C) (D) (E) 58 151 Crohn disease Diverticulitis Ischemic colitis Pseudomembranous colitis Ulcerative colitis Physical examination of a newborn female after delivery reveals an imperforate anus Meconium is visible behind the thin, cutaneous membrane The classification of this anorectal malformation is based on the relationship of Fenderson_Chap13.indd 151 61 Intestinal infarct Intussusception Meckel diverticulum Peutz-Jeghers polyps Volvulus An 85-year-old man complains of abdominal pain and bright red blood in his stool An X-ray film of the abdomen shows fecal impaction in the rectosigmoid region Which of the following pathologic lesions is most likely to be found in this patient? (A) Curling ulcer (B) Cushing ulcer (C) Melanosis coli (D) Peptic ulcer (E) Stercoral ulcer 7/12/2010 2:11:02 PM 152 62 Chapter 13 A 45-year-old woman complains of chronic, right lower quadrant pain An abdominal CT scan reveals a globular, smooth-walled mass protruding into the cecum The patient subsequently has the mass removed and the surgical specimen is shown in the image Which of the following is the most likely diagnosis? (A) (B) (C) (D) (E) 63 The answer is D: Esophageal web Esophageal rings and webs cause dysphagia Webs are thin mucosal membranes that project into the lumen of the esophagus Rings are thicker than webs and contain smooth muscle The clinical symptoms of esophageal webs and rings include dysphagia, esophageal substernal pain, and aspiration or regurgitation of foods and liquids Plummer-Vinson syndrome is characterized by a cervical esophageal web, mucosal lesions of the mouth and pharynx, and iron-deficiency anemia Carcinoma of the oropharynx and upper esophagus are complications of Plummer-Vinson syndrome The other choices are not associated with anemia Schatzki ring (choice E) occurs near the gastroesophageal junction Diagnosis: Esophageal web The answer is B: Dysphagia This patient exhibits signs of scleroderma (progressive systemic sclerosis), which is characterized by vasculopathy and excessive collage deposition in the skin and internal organs Patients often suffer from intermittent episodes of ischemia of the fingers, marked by pallor, paresthesias, and pain (Raynaud phenomenon) Anti–Scl-70 antibodies to nuclear topoisomerase are virtually specific for this autoimmune disease Scleroderma can involve any portion of the gastrointestinal tract, although esophageal dysfunction is the most common and troublesome complication The disease affects principally the lower esophageal sphincter, which may become so impaired that the lower esophagus and upper stomach are no longer distinct functional entities In some affected patients, there may be a lack of peristalsis in the entire esophagus The other choices are not associated with scleroderma Diagnosis: Scleroderma The answer is D: Glandular metaplasia The biopsy shows Barrett esophagus, which is defined as a replacement of the esophageal squamous epithelium by columnar epithelium as a result of chronic gastroesophageal reflux This disorder occurs typically in the lower third of the esophagus The lesion is characterized histologically by distinctive intestine-like epithelium composed of goblet cells and surface cells similar to those of incompletely intestinalized gastric mucosa Complete intestinal metaplasia, with Paneth cells and absorptive cells, may occur Barrett esophagus is more resistant to peptic juices than normal squamous epithelium and appears to be an adaptive mechanism that serves to limit the harmful effects of gastroesophageal reflux None of the other choices lead to metaplastic changes Diagnosis: Barrett esophagus, reflux esophagitis The answer is B: Adenocarcinoma Barrett esophagus carries a serious risk of malignant transformation to adenocarcinoma, and the risk correlates with the length of the involved esophagus and the degree of dysplasia Virtually all esophageal adenocarcinomas arise in the background of the metaplastic epithelium of Barrett esophagus The symptoms and clinical course of adenocarcinoma of the esophagus are similar to those of squamous cell carcinoma and include dysphagia, pain, and, occasionally, bleeding None of the other choices reflect a complication of Barrett esophagus Diagnosis: Barrett esophagus Acute appendicitis Adenocarcinoma Carcinoid tumor Foreign body Mucocele of the appendix A 70-year-old woman with a history of ovarian cancer presents with diarrhea She completed radiation therapy for her cancer months ago Physical examination shows cachexia, hyperactive bowel sounds, and generalized pallor The stools are found to contain blood A CBC shows decreased hemoglobin (7.8 g/dL) and decreased mean corpuscular volume (70 μm3) Which of the following is the most likely cause of GI bleeding in this patient? (A) Angiodysplasia (B) Hemorrhoids (C) Ischemic colitis (D) Radiation enterocolitis (E) Solitary rectal ulcer ANSWERS The answer is A: Achalasia Achalasia is characterized by failure of the lower esophageal sphincter to relax in response to swallowing and the absence of peristalsis in the body of the esophagus As a result of these defects in both the outflow tract and the pumping mechanisms of the esophagus, food is retained within the esophagus, and the organ hypertrophies and dilates Achalasia is associated with a depletion or absence of ganglion cells in the myenteric plexuses, which regulate contraction of the esophagus In Latin America, achalasia can be a manifestation of Chagas disease, in which the ganglion cells are destroyed by Trypanosoma cruzi The other choices are usually associated with visible mucosal abnormalities and not primarily affect peristalsis Diagnosis: Achalasia Fenderson_Chap13.indd 152 7/12/2010 2:11:04 PM The Gastrointestinal Tract The answer is E: Zenker diverticulum Zenker diverticulum is an uncommon lesion that appears high in the esophagus and affects men more than women It was once believed to result from luminal pressure exerted in a structurally weak area and was, therefore, classed as a pulsion diverticulum The cause is probably more complicated, but disordered function of the cricopharyngeal musculature is still thought to be involved in the pathogenesis of this false diverticulum Most affected persons who come to medical attention are older than 60 years, an observation that supports the belief that Zenker diverticulum is an acquired lesion Epiphrenic diverticuli (choice A) are located immediately above the diaphragm Intramural pseudodiverticulum (choice B) is characterized by numerous small diverticula in the wall of the esophagus Traction diverticuli (choice D) are outpouchings that occur principally in the midportion of the esophagus Diagnosis: Zenker diverticulum The answer is D: Hiatal hernia Hiatal hernia is a protrusion of the stomach through an enlarged esophageal hiatus in the diaphragm Two basic types of hiatal hernia are observed In sliding hiatal hernias, an enlargement of the hiatus and laxity of the circumferential connective tissue allows a cap of gastric mucosa to move upward above the diaphragm Paraesophageal hiatal hernias are characterized by herniation of a portion of the gastric fundus alongside the esophagus through a defect in the diaphragmatic connective tissue membrane that defines the esophageal hiatus Symptoms of hiatal hernia, particularly heartburn and regurgitation, are attributed to the reflux of gastric contents, which is primarily related to incompetence of the lower esophageal sphincter Classically, the symptoms are exacerbated when the affected person is recumbent Large herniations carry a risk of gastric volvulus or intrathoracic gastric dilation Boerhaave syndrome (choice A) represents rupture of the esophagus as a result of vomiting Mallory-Weiss syndrome (choice E) refers to mucosal laceration of the upper stomach and lower esophagus in the setting of severe retching Diagnosis: Paraesophagic hiatal hernia The answer is B: Coagulative necrosis Chemical injury to the esophagus usually reflects accidental poisoning in children, attempted suicide in adults, or contact with medication Ingestion of strong acids produces an immediate coagulative necrosis in the esophagus, which results in a protective eschar that limits injury and further chemical penetration By contrast, ingestion of strong alkaline solutions is accompanied by liquefactive necrosis (choice E), with inflammation and saponification of membrane lipids Alkaline solutions are particularly insidious because they are generally odorless and tasteless and, therefore, easily swallowed before protective reflexes come into play Diagnosis: Chemical esophagitis The answer is A: Adenocarcinoma Adenocarcinoma of the esophagus is now more common (60%) in the United States than squamous carcinoma Adenocarcinoma originates in the glandular metaplasia of Barrett esophagus Endoscopic surveillance for adenocarcinoma is now commonly done in patients with Barrett esophagus, particularly those with dysplasia Tumors tend to grow into the lumen of the esophagus Fenderson_Chap13.indd 153 153 The affected region of the esophagus is typically indurated and ulcerated, causing pain and bleeding The other choices not exhibit the histologic features described Diagnosis: Adenocarcinoma of the esophagus 10 The answer is D: Squamous cell carcinoma Most cancers of the esophagus worldwide are squamous cell carcinomas, although adenocarcinoma is now more common in the United States Squamous cell tumors range from well-differentiated cancers with “epithelial pearls” to poorly differentiated neoplasms that lack evidence of squamous differentiation The most common presenting complaint of patients with esophageal cancer is dysphagia, but by this time, most tumors are unresectable Adenocarcinoma (choice A) and Barrett esophagus (choice B) are incorrect because the biopsy does not show glandular features Primary malignant melanoma (choice C) of the esophagus is extremely rare, although melanoma metastases to the esophagus may occur Diagnosis: Squamous cell carcinoma of the esophagus 11 The answer is A: Cigarette smoking Risk factors for squamous cell carcinoma of the esophagus include chronic alcoholism, tobacco use, diets lacking in fresh fruits, exposure to aniline dyes, chronic esophagitis, and congenital disorders of the esophagus (e.g., Plummer-Vinson syndrome) Cigarette smoking is associated with a 5- to 10-fold increased risk of esophageal cancer, and the number of cigarettes smoked correlates with the presence of dysplasia in the esophageal epithelium Epidemiologic data suggest that there are additional, as yet unidentified risk factors prevalent in certain geographical regions of the world (China, Iran, and South Africa) Reflux esophagitis (choice E) leads to Barrett esophagus and adenocarcinoma Aflatoxin (choice B) is a well-known hepatotoxin linked to the development of hepatocellular carcinoma Herpetic esophagitis (choice C) frequently occurs in immunocompromised persons but is not associated with the development of carcinoma Diagnosis: Esophageal cancer 12 The answer is C: Reflux esophagitis Esophagitis may be caused by infections, reflux of gastric juice, or exogenous irritants Of these, the most common type is reflux esophagitis, which is often found in conjunction with a sliding hiatal hernia but may also arise through an incompetent lower esophageal sphincter without any demonstrable anatomical lesion Chronic exposure to stomach juice causes reactive thickening of the squamous epithelium (leukoplakia) and the underlying stroma Areas affected by gastric reflux are susceptible to mucosal erosions and ulcers which appear as linear vertical streaks Neutrophils and lymphocytes accumulate in the mucosa The other choices are not typical complications of hiatal hernia Diagnosis: Reflux esophagitis, hiatal hernia 13 The answer is B: Candida esophagitis Candida esophagitis has become commonplace because of an increasing number of immunocompromised persons Esophageal candidiasis also occurs in patients with diabetes and in those receiving antibiotic therapy The pseudomembranes are composed of fungal mycelia, fibrin, and necrotic debris Involvement of deeper layers of the esophageal wall can lead to disseminated candidiasis, as well as fibrosis, which is sometimes severe enough 7/12/2010 2:11:05 PM 154 Chapter 13 to create esophageal stricture Symptoms include dysphagia and odynophagia (pain on swallowing) Herpetic esophagitis (choice C) features mucosal vesicles The other choices are not characterized by the formation of elevated white plaques on the esophageal mucosa Diagnosis: Infective esophagitis 14 The answer is E: Portal hypertension Esophageal varices are dilated (varicose) veins immediately beneath the mucosa, which are prone to rupture and hemorrhage They arise in the lower third of the esophagus, most often in the setting of portal hypertension, secondary to cirrhosis The lower esophageal veins are linked to the portal system through gastroesophageal anastomoses If the portal blood pressure exceeds a critical level, these anastomoses become prominent When varices become greater than mm in diameter, they are likely to rupture, in which case life-threatening hemorrhage may ensue The other choices are not associated with bleeding esophageal varices Alcoholic hepatitis (choice A) by itself does not cause varices, but long-term alcohol abuse often leads to cirrhosis and esophageal varices Diagnosis: Esophageal varices, cirrhosis 15 The answer is B: Congenital pyloric stenosis Congenital pyloric stenosis is a concentric enlargement of the pyloric canal that obstructs the outlet of the stomach The disorder is the most common indication for abdominal surgery in the first months of life Congenital pyloric stenosis has a familial tendency, and the condition is more common in identical twins than in fraternal ones The only consistent microscopic abnormality is hypertrophy of the circular muscle coat Projectile vomiting is not characteristic of the other choices, particularly in neonates Diagnosis: Congenital pyloric stenosis 16 17 The answer is A: Acute erosive gastritis Acute hemorrhagic gastritis is characterized by necrosis of the mucosa and is commonly associated with the intake of aspirin, other NSAIDs, alcohol, or ischemic injury The factor common to all forms of acute hemorrhagic gastritis is thought to be the breakdown of the mucosal barrier, which permits acid-induced injury Mucosal injury causes bleeding from superficial erosions Defects in the mucosa may extend into the deeper tissues to form an ulcer The necrosis is accompanied by an acute inflammatory response and hemorrhage, which may be severe enough to result in exsanguination and hypovolemic shock The other choices are not associated with the use of NSAIDs Diagnosis: Acute erosive gastritis The answer is B: Autoimmune gastritis Autoimmune gastritis refers to chronic, diffuse inflammatory disease of the stomach that is restricted to the body and fundus and is associated with other autoimmune phenomena This disorder typically features diffuse atrophic gastritis, antibodies to parietal cells and the intrinsic factor, and increased serum gastrin due to G-cell hyperplasia Immunologic destruction of parietal cells and antibody targeting of intrinsic factor interfere with intestinal absorption of vitamin B12 As a result, all lineages of bone marrow precursors show asynchronous maturation between the nucleus and cytoplasm (megaloblastic cells), and the peripheral blood displays megaloblastic anemia Megaloblastic Fenderson_Chap13.indd 154 anemia that is caused by malabsorption of vitamin B12, occasioned by a deficiency of the intrinsic factor, is referred to as “pernicious anemia.” The other choices are not causes of pernicious anemia Diagnosis: Autoimmune atrophic gastritis, pernicious anemia 18 The answer is E: Helicobacter pylori infection Peptic ulcer disease refers to breaks in the mucosa of the stomach and small intestine, principally the proximal duodenum, which are produced by the action of gastric secretions The pathogenesis of peptic ulcer disease is believed to involve an underlying chronic gastritis caused by H pylori This pathogen has been isolated from the gastric antrum of virtually all patients with duodenal ulcers and from about 75% of those with gastric ulcers H pylori gastritis is the most common type of gastritis in the United States and is characterized by prominent chronic inflammation of the antrum and body of the stomach In addition to peptic ulcer disease, H pylori gastritis is a risk factor for development of gastric adenocarcinoma and lymphoma Eradication of H pylori infection is curative of peptic ulcer disease in most patients Gastrinoma (choice D) is a rare cause of peptic ulcers Achlorhydria (choice A) is incorrect because the formation of peptic ulcers requires at least some gastric acid secretion Diagnosis: Chronic infectious gastritis, peptic ulcer disease, gastric ulcer 19 The answer is C: Melena Peptic ulcers of the stomach and duodenum are estimated to afflict 10% of the population of Western industrialized countries at some time during their lives Peptic ulcers appear as punched out, rounded ulcers Erosion through arteries causes bleeding and iron-deficiency anemia Melena refers to black, tarry stools composed largely of blood from the upper digestive tract that has been processed by the action of gastric juices Melena is commonly seen in patients who suffer from chronic peptic ulcer disease Unlike duodenal ulcers, most patients with gastric ulcers secrete normal or decreased amounts of gastric acid (see choice B) Diagnosis: Peptic ulcer disease, gastric ulcer 20 The answer is B: Intrinsic factor This patient has chronic lymphocytic thyroiditis (Hashimoto thyroiditis) and pernicious anemia Pernicious anemia is a megaloblastic anemia that is caused by malabsorption of vitamin B12 due to a deficiency of the intrinsic factor In many cases, pernicious anemia is associated with other autoimmune diseases (e.g., Hashimoto thyroiditis, Graves disease, Addison disease, or diabetes mellitus type 1) Circulating antibodies to parietal cells, some of which are cytotoxic in the presence of complement, occur in 90% of patients with pernicious anemia Two thirds of patients display an antibody to the intrinsic factor that prevents its combination with vitamin B12, thereby preventing formation of the complex that is later absorbed in the ileum Half of all patients with pernicious anemia have circulating antibodies to thyroid tissue Diagnosis: Pernicious anemia, Hashimoto thyroiditis 21 The answer is E: Pancreas Zollinger-Ellison syndrome is characterized by unrelenting peptic ulceration in the stomach or duodenum (or even proximal jejunum) by the action of tumor-derived gastrin Gastrin-producing neuroendocrine 7/12/2010 2:11:05 PM The Gastrointestinal Tract tumors (gastrinomas) usually arise in the pancreatic islets Among islet cell tumors, pancreatic gastrinomas are second in frequency only to insulinomas Duodenum (choice C) is incorrect because only 15% of cases of Zollinger-Ellison syndrome are due to gastrinomas outside the pancreas (e.g., duodenum) Most gastrinomas are malignant Diagnosis: Zollinger-Ellison syndrome, peptic ulcer disease, gastric ulcer 22 23 24 25 The answer is B: Adenocarcinoma Adenocarcinoma of the stomach accounts for more than 95% of all malignant gastric tumors Most patients have metastases by the time they are seen for examination The most frequent initial symptom of gastric cancer is weight loss, usually associated with anorexia and nausea Most patients complain of epigastric pain—a symptom that mimics benign gastric ulcer disease, and is often relieved by antacids or H2-receptor antagonists On gross inspection, gastric cancer appears as a polypoid, fungating, or ulcerated mass, or diffuse infiltration of the stomach wall This patient has an ulcerating carcinoma Acute erosive gastritis (choice A) and peptic ulcer disease (choice E) not typically present with rapid weight loss, and these benign ulcers usually not have heaped-up (raised), ragged margins Curling ulcers (choice C) occur in severely burned patients Diagnosis: Gastric adenocarcinoma The answer is D: Linitis plastica Diffuse adenocarcinoma constitutes10% of all stomach cancers No true tumor mass is seen macroscopically Instead, the wall of the stomach is conspicuously thickened and firm, accounting for the radiologic “leather bottle” appearance When the entire stomach is involved, the term linitis plastica is applied The invading tumor cells induce extensive fibrosis in the submucosa and muscularis of the stomach wall Gastric carcinomas typically metastasize to regional lymph nodes and the liver Signet ring cells are so named because intracellular mucin displaces the nuclei to the periphery of the tumor cells Gastric carcinomas and linitis plastica, in particular, have a poor prognosis The other choices not show the characteristic morphologic appearance of linitis plastica and generally not exhibit signet ring cells Diagnosis: Gastric adenocarcinoma The answer is B: Enlarged rugal folds Ménétrier disease (hyperplastic hypersecretory gastropathy) is an uncommon disorder of the stomach characterized by enlarged rugae It is often accompanied by a severe loss of plasma proteins (including albumin) from the altered gastric mucosa The disease occurs in two forms: a childhood form due to cytomegalovirus infection and an adult form attributed to overexpression of TGF-α The folds of the greater curvature in the fundus and body of the stomach (occasionally in the antrum) are increased in height and thickness, forming a convoluted brain-like surface The other choices not feature protein-losing enteropathy Diagnosis: Ménétrier disease The answer is C: Gastrointestinal stromal tumor (GIST) GISTs are derived from the pacemaker cells of Cajal They include the vast majority of mesenchyme-derived stromal tumors of the entire gastrointestinal tract Gastric GISTs are usually Fenderson_Chap13.indd 155 155 submucosal and covered by intact mucosa Microscopically, the tumors show spindle cells with vacuolated cytoplasms GISTs are considered to be of low malignant potential and are removed surgically Gastric adenocarcinoma (choice A) does not often dedifferentiate to a spindle cell morphology Diagnosis: Gastrointestinal stromal tumor 26 The answer is B: Gastric lymphoma Gastric lymphoma is the most common form of extranodal lymphoma, accounting for 20% of all such tumors Gastric lymphoma has a considerably better prognosis than gastric carcinoma (45% 5-year survival) The clinical symptoms of gastric lymphoma are nonspecific and indistinguishable from those of gastric carcinoma (e.g., weight loss, dyspepsia, and abdominal pain) Most gastric lymphomas are low-grade B-cell neoplasms of the MALToma (mucosa-associated lymphoid tissue) type, which arise in the setting of chronic Helicobacter pylori gastritis with lymphoid hyperplasia Some of these lymphomas regress after eradication of the infection The other choices not demonstrate the lymphoid lesion depicted here Diagnosis: Gastric lymphoma, MALToma 27 The answer is D: Trichobezoar Bezoars are foreign bodies in the stomach that are composed of food or hair that have been altered by the digestive process The mass removed from the stomach in this patient is a hairball (trichobezoar) within a gelatinous matrix Trichobezoar is usually seen in long-haired girls or young women who eat their own hair as a nervous habit (trichotillomania; also called “Rapunzel” syndrome) Such a trichobezoar may grow by accretion to form a complete cast of the stomach Strands of hair may extend into the bowel as far as the transverse colon (Rapunzel syndrome) Phytobezoars (choice C) are concretions of plant material, which usually occur in persons with conditions that interfere with gastric emptying Diagnosis: Trichobezoar, bezoar, Rapunzel syndrome 28 The answer is A: Bleeding Bleeding is the most common complication of peptic ulcer disease, occurring in about 20% of patients Chronic blood loss due to occult bleeding is often a feature of peptic ulcers, whereas massive bleeding occurs less often Perforation (choice D) is a serious complication that occurs in 5% of patients Perforating ulcers are commonly encountered in the duodenum Duodenal peptic ulcers not undergo malignant transformation (choice B) The other choices are uncommon Diseases associated with peptic ulcers include cirrhosis, chronic renal failure, hereditary endocrine syndromes (MEN-1), α1–antitrypsin deficiency, and chronic pulmonary disease Diagnosis: Duodenal ulcer, peptic ulcer disease 29 The answer is A: Carcinoid tumor Carcinoid tumors are low-grade malignant neoplasms composed of neuroendocrine cells, which usually show considerable nuclear uniformity They are most commonly located in the submucosa of the intestines (appendix, terminal ileum, and rectum) Carcinoids are distinguished from intestinal carcinomas based on their location, histologic features, malignant potential, endocrine activity, and clinical features Carcinoid syndrome is a systemic paraneoplastic disease caused by the release of hormones from carcinoid tumors into venous blood Clinical features of carcinoid tumors (e.g., flushing, bronchial wheezing, watery 7/12/2010 2:11:05 PM 156 Chapter 13 diarrhea, and abdominal colic) are presumably caused by the release of serotonin, bradykinin, and histamine Release of tumor secretions from hepatic metastases leads to the formation of fibrous plaques in the tricuspid and pulmonic valves and may result in tricuspid insufficiency or pulmonic stenosis The other choices are not associated with secretion of 5-HIAA acid or other neuroendocrine peptides Diagnosis: Carcinoid syndrome 30 31 32 33 The answer is C: Peutz-Jeghers polyp Peutz-Jeghers syndrome is an autosomal dominant, hereditary disorder characterized by intestinal hamartomatous polyps and mucocutaneous melanin pigmentation, which is particularly evident on the face, buccal mucosa, hands, feet, and perianal and genital regions The polyps seen in Peutz-Jeghers syndrome are hamartomatous, characterized by a branching network of smooth muscle fibers continuous with the muscularis mucosa that support the glandular epithelium of the polyp Congenital teratoma (choice A) does not involve the intestine The other choices are principally colonic polyps that derive from the luminal epithelium Diagnosis: Gastrointestinal polyp, Peutz-Jeghers polyp The answer is B: Hyperplastic polyp Hyperplastic polyps are small, sessile mucosal excrescences that display exaggerated crypt architecture They are the most common polypoid lesions of the colon and are particularly frequent in the rectum The crypts of hyperplastic polyps are elongated and may exhibit cystic dilations The epithelium is composed of goblet cells and absorptive cells, without any dysplasia There are no dysplastic features indicative of adenocarcinoma (choice A) Villous adenomas (choice E) are considerably larger and exhibit prominent thin, tall, fingerlike processes PeutzJeghers polyps (choice D) are hamartomatous Diagnosis: Gastrointestinal polyp, hyperplastic polyp The answer is D: Tubular adenoma Tubular adenomas constitute two thirds of benign colonic adenomas They are typically smooth-surface lesions, usually less than cm in diameter, and often have a stalk Microscopically, tubular adenomas exhibit closely packed epithelial tubules, which may be uniform or irregular with excessive branching Dysplasia and carcinoma often develop in tubular adenomas As long as dysplastic foci remain confined to the polyp mucosa, the lesion is almost always cured by resection Adenocarcinoma (choice A) is incorrect because the lesion does not have dysplastic features Pseudostratified epithelium is not a feature of carcinoid tumor (choice B) or hyperplastic polyp (choice C) The incorrect choices not typically exhibit a stalk Diagnosis: Gastrointestinal polyp, tubular adenoma The answer is E: Villous adenoma These polyps comprise one third of colonic adenomas and are found predominantly in the rectosigmoid region They are typically large, broadbased, elevated lesions that display a shaggy, cauliflower-like surface Microscopically, villous adenomas are composed of thin, tall, fingerlike processes, which superficially resemble the villi of small intestine Compared to tubular adenomas (choice D), villous adenomas more frequently contain foci of carcinoma Hyperplastic polyps (choice A) have a much lower risk for malignant transformation Diagnosis: Gastrointestinal polyp, villous adenoma Fenderson_Chap13.indd 156 34 The answer is A: Adenocarcinoma Adenocarcinoma of the rectum or sigmoid colon often presents as a circumferential mass narrowing the intestinal lumen The gross appearance of the colorectal cancer is similar to that seen elsewhere in the gastrointestinal tract The most important risk factors associated with the development of colonic adenocarcinoma are age, prior colorectal cancer, ulcerative colitis, genetic factors, and perhaps diet Colorectal cancer invades lymphatic channels and initially involves the lymph nodes immediately underlying the tumor As the tumor grows, the most common sign is occult blood in feces Bright red blood more often occurs in distal lesions In either case, bleeding typically causes iron-deficiency anemia Choices B, C, and D are principally lesions of the intestinal wall Choice E (mucinous cystadenoma) is an ovarian tumor Diagnosis: Colorectal cancer, adenocarcinoma of colon 35 The answer is A: APC The photograph shows numerous adenomas of the colon, consistent with familial adenomatous polyposis (FAP), also termed adenomatous polyposis coli (APC) This autosomal dominant inherited disease accounts for about 1% of colorectal cancers It is characterized by the progressive development of innumerable adenomatous polyps of the colorectum, particularly in the rectosigmoid region Germline mutations in the APC gene, a putative tumor suppressor gene, are responsible for FAP Carcinoma of the colon and rectum is inevitable in these patients, and the mean age of onset is 40 years The DCC gene (“deleted in colon cancer”— choice C) is a putative tumor suppressor gene that is often missing in colorectal cancers Activating mutations of the ras protooncogene (choice E) occur early in tubular adenomas of the colon Diagnosis: Adenomatous polyposis coli 36 The answer is C: p53 In most cases of colorectal carcinoma, it has been estimated that a minimum of eight to ten mutational events must accumulate before the development of invasive cancer This process is initiated in morphologically normal mucosa, proceeds through an adenomatous precursor, and terminates as invasive adenocarcinoma The APC gene is considered to play an important role in the early development of most colorectal neoplasms, whereas mutations in the p53 tumor suppressor gene are thought to participate in the late transition from adenoma to carcinoma BRCA1 (choice A) has been implicated in the pathogenesis of breast and ovarian cancers VHL (choice E) has been incriminated in the pathogenesis of clear cell renal cell carcinoma Diagnosis: Adenocarcinoma of colon 37 The answer is E: Metastatic carcinoma Metastatic carcinoma is by far the most common malignant disorder affecting the peritoneum Ovarian, gastric, and pancreatic carcinomas are particularly likely to seed the peritoneum, but any intra-abdominal carcinoma can spread to the peritoneum Metastatic carcinoma to the abdomen presents in the form of multiple serosal nodules and ascites fluid that contains malignant cells Diagnosis: Metastatic carcinoma 38 The answer is C: Kaposi sarcoma Kaposi sarcoma of the GI tract is found almost exclusively in patients with AIDS One third to one half of AIDS patients, with cutaneous Kaposi 7/12/2010 2:11:05 PM The Gastrointestinal Tract sarcoma, exhibit involvement of the GI tract In most patients, intestinal Kaposi sarcoma does not lead to symptoms, although GI bleeding, obstruction, and malabsorption have been reported The other choices are not specifically associated with AIDS Diagnosis: AIDS, Kaposi sarcoma 39 40 41 The answer is E: Ulcerative colitis Ulcerative colitis is an inflammatory disease of the large intestine characterized by chronic diarrhea and rectal bleeding It is associated with a pattern of remission and exacerbations and the possibility of serious local and systemic complications The disorder occurs principally, but not exclusively, in young adults Ulcerative colitis is essentially a disease of the mucosa The process extends from the rectum for a variable distance proximally and is limited to the colon and rectum Pseudomembranous colitis (choice D) is usually a complication of antibiotic therapy, and the mucosal surface of the colon is covered by raised, irregular plaques composed of necrotic debris and an acute inflammatory exudate Crohn disease (choice C) typically affects the colon in a patchy distribution with transmural inflammation Diagnosis: Ulcerative colitis The answer is A: Adenocarcinoma Patients with longstanding ulcerative colitis have a higher risk of developing colorectal cancer (adenocarcinoma) than does the general population This risk is related to the extent of colorectal involvement and the duration of the inflammatory process Thus, people with involvement of the entire colon are at the greatest risk of developing colorectal cancer Intestinal fistula (choice B) is a complication of Crohn disease Ulcerations in ulcerative colitis are largely confined to the mucosa (not transmural, choice D) Diagnosis: Ulcerative colitis The correct answer is E: Ulcerative colitis Primary sclerosing cholangitis (PSC) is characterized by inflammation and obliterative fibrosis of intrahepatic and extrahepatic bile ducts Approximately 70% of patients with PSC have longstanding ulcerative colitis, although the prevalence of PSC in such patients is only 4% The clinicopathologic findings are complemented by a characteristic radiographic appearance of a beaded biliary tree, representing sporadic strictures The other choices are not associated with PSC Diagnosis: Primary sclerosing cholangitis, ulcerative colitis 42 The answer is E: Ulcerative colitis Local complications of ulcerative colitis include toxic megacolon, perforation, inflammatory pseudopolyps, hemorrhage, and adenocarcinoma The other choices are not associated with the development of toxic megacolon Diagnosis: Toxic megacolon, ulcerative colitis 43 The answer is C: Crohn disease Crohn disease is a transmural, chronic inflammatory disease that may affect any part of the digestive tract but occurs principally in the distal small intestine and occasionally the right colon It has variously been referred to as terminal ileitis and regional ileitis when it involves the ileum and granulomatous colitis when Fenderson_Chap13.indd 157 157 it principally affects the colon Skip lesions are common The affected mucosa has a characteristic “cobblestone” appearance (shown in the image) due to the presence of linear ulcerations and edema, and inflammation of the intervening tissue The other choices not show the characteristic cobblestone morphology that is seen in this case Diagnosis: Crohn disease 44 The answer is C: Crohn disease Crohn disease is a transmural, chronic inflammatory disease that may affect any part of the digestive tract Intestinal obstruction and fistulas are the most common intestinal complications of Crohn disease Occasionally, free perforation of the bowel occurs The risk of small bowel cancer is increased at least threefold in patients with Crohn disease Pseudomembranous colitis (choice D) and ulcerative colitis (choice E) are not associated with fistula formation Adenocarcinoma (choice A) rarely, if ever, arises in the terminal ileum Diagnosis: Crohn disease 45 The answer is E: Stimulation of fluid transport into the lumen of the intestine The most significant factor in infectious diarrhea is increased intestinal secretion, stimulated by bacterial toxins and enteric hormones Organisms that produce diarrhea by secreting specific toxins include Vibrio cholera and toxigenic strains of E coli There is minimal or absent damage to the intestinal mucosa (choices A to D) in cases of toxigenic diarrhea The organisms remain attached to the intestinal mucosa and elaborate toxins, which stimulate the transmucosal transport of fluid into the lumen, resulting in diarrhea Patients may become severely dehydrated, particularly in the case of cholera Diagnosis: Bacterial diarrhea 46 The answer is B: Rotavirus Rotavirus is the most common cause of infantile diarrhea and can be demonstrated in duodenal biopsy specimens in half the cases of acute diarrhea in hospitalized children under the age of years Choices C, D, and E can cause diarrhea, but are uncommon in developed countries Diagnosis: Viral diarrhea 47 The answer is E: Superior mesenteric artery Sudden occlusion of a large artery by thrombosis or embolization leads to small bowel infarction before collateral circulation comes into play Depending on the size of the artery, infarction may be segmental or may lead to gangrene of virtually the entire small bowel The small intestine, which is supplied by the superior mesenteric artery, is more likely to suffer transmural hemorrhagic infarction than the large intestine The inferior mesenteric artery (choice C), which supplies blood to the colon, is a less common site for atherosclerotic embolization than the superior mesenteric artery because of the smaller size of the latter and its oblique origin from the aorta Pathologically, ischemic bowel disease is classified as occlusive or nonocclusive Occlusive disease is caused by thrombi or emboli, whereas nonocclusive disease is secondary to arterial narrowing by atherosclerosis The other choices are not specifically associated with small bowel ischemia Diagnosis: Ischemic colitis 48 The answer is A: Disaccharidase Acquired lactase deficiency is a widespread disorder of carbohydrate absorption The symptoms of this disease typically begin in adolescence, when 7/12/2010 2:11:05 PM 158 Chapter 13 patients complain of flatulence and diarrhea after the ingestion of dairy products Lactose is one of the most common disaccharides in dairy products The intestinal brush border contains disaccharidases that are important for cleavage of lactose to free glucose and galactose for absorption Congenital lactase deficiency is rare but may be lethal if not recognized The other choices not hydrolyze lactose Diagnosis: Lactose intolerance 49 The answer is C: Hirschsprung disease Hirschsprung disease, also referred to as congenital megacolon, results from a congenital defect in the innervation of the large intestine, usually in the rectum Severe chronic constipation is typical Marked dilation of the colon occurs proximal to the stenotic rectum, with clinical signs of intestinal obstruction The other choices are not associated with loss of ganglion cells Diagnosis: Hirschsprung disease 50 The answer is A: Arthritis The case history is indicative of ulcerative colitis Arthritis is seen in 25% of patients with ulcerative colitis Uveitis and skin lesions develop in approximately 10% of patients The most common cutaneous lesions are erythema nodosum and pyoderma gangrenosum Liver disease occurs in about 4% of patients, the most common pathologic findings being pericholangitis and fatty liver The other choices not represent extraintestinal manifestations of ulcerative colitis Diagnosis: Ulcerative colitis, arthritis 51 The answer is E: Nephrotic syndrome Most cases of peritonitis are caused by bacteria that enter the abdominal cavity from a perforated viscus or through an abdominal wound However, spontaneous bacterial peritonitis occurs in children without an obvious perforation Most of these patients have a nephrotic syndrome and a systemic infection that seeds the ascitic fluid with bacteria In adults, spontaneous bacterial peritonitis is a feared complication of cirrhosis The other choices are not associated with the development of spontaneous bacterial peritonitis Diagnosis: Spontaneous bacterial peritonitis, nephrotic syndrome 52 53 The answer is B: Diverticulitis Diverticular disease refers to two entities: a condition termed diverticulosis and an inflammatory complication called diverticulitis Diverticulosis is generally asymptomatic Diverticulitis results from the irritation caused by retained fecal material that obstructs the lumen of a diverticulum Clinically, the most common symptoms of diverticulitis usually follow microscopic or gross perforation of the diverticulum Diverticula are most common in the sigmoid colon, which is affected in 95% of cases Peritonitis and sepsis are serious complications Appendicitis (choice A) usually presents with right lower quadrant pain None of the other choices presents with gastrointestinal symptoms and fever Diagnosis: Diverticulitis The answer is E: Yersinia enterocolitica Yersinia can cause mesenteric adenitis and pain in the right lower quadrant (pseudoappendicitis) Infected children not infrequently have undergone laparotomy because of a mistaken diagnosis of Fenderson_Chap13.indd 158 appendicitis The lymph nodes show a granulomatous inflammation Other symptoms are diarrhea, reactive arthritis, erythema nodosum, and septicemia The other choices are not associated with the development of mesenteric adenitis and not present with symptoms that mimic acute appendicitis Diagnosis: Yersinia lymphadenitis 54 The answer is E: Whipple disease Whipple disease is a rare infectious disorder of the small intestine in which malabsorption is the most prominent feature The disorder typically features infiltration of the small bowel mucosa by macrophages that are packed with small, rod-shaped bacilli (Tropheryma whippelii) Infiltrates of macrophages containing bacilli may be found in other organs, including regional lymph nodes and the heart The other choices not feature these distinctive aggregates of foamy macrophages Diagnosis: Whipple disease 55 The answer is D: Impaired mucosal function Normal intestinal absorption is characterized by a luminal phase and an intestinal phase The intestinal phase includes those processes that occur in epithelial cells and transport channels Injury to the mucosa in patients with Whipple disease (secondary to inflammation) results in impaired transport of nutrients through the intestinal wall Histologic examination of the small intestine reveals flat, thickened villi and extensive infiltration of the lamina propria with foamy macrophages The other choices pertain to luminal phase processes that are unaffected in patients with Whipple disease Diagnosis: Whipple disease 56 The answer is A: Celiac sprue Celiac sprue, which is also referred to as gluten-sensitive enteropathy, is characterized by (1) generalized malabsorption, (2) small intestinal mucosal lesions, and (3) prompt clinical and histopathologic response to the withdrawal of gluten-containing food Critical factors in the development of celiac sprue include genetic predisposition and gliadin exposure The hallmark of celiac disease is a flat mucosa, with blunting of villi, damaged epithelial cells, intraepithelial T cells, and increased plasma cells in the lamina propria The other choices not respond to a gluten-free diet Diagnosis: Celiac sprue 57 The answer is D: Pseudomembranous colitis Pseudomembranous colitis is a generic term for an inflammatory disease of the colon that is characterized by exudative plaques on the mucosa Antibiotic therapy eliminates the normal mixed flora of the colon and facilitates the overgrowth of Clostridium difficile, leading to an acute infection of the colon The exotoxins produced by C difficile cause intestinal necrosis, with superficial ulcers covered by a thick fibropurulent exudate The other choices are not related to antibiotic therapy and are not associated with the development of these exudative plaques Diagnosis: Pseudomembranous colitis 58 The answer is C: Levator ani muscle Anorectal malformations are among the most common anomalies and vary from minor narrowing to serious and complex effects The classification of these anomalies is based on the relation of the terminal bowel to the levator ani muscle The other choices are not associated with anorectal malformations Diagnosis: Anorectal malformation 7/12/2010 2:11:06 PM The Gastrointestinal Tract 159 59 The answer is D: Gastrointestinal fistula Anorectal malformations result from arrested development of the caudal region of the gut in the first months of fetal life The cause is unknown Fistulas between the malformation and the bladder, urethra, vagina, or skin may occur in all types of anorectal anomalies The other choices are not associated with anorectal malformations Diagnosis: Anorectal malformation 61 The answer is E: Stercoral ulcer Incomplete evacuation of the feces, usually in association with debilitating disease or old age, may lead to the formation of a large mass of stool that cannot be passed, termed fecal impaction Stercoral ulcers result from pressure necrosis of the mucosa caused by the fecal mass Complications include rectal bleeding and perforation The other ulcers not occur in the rectum Diagnosis: Stercoral ulcer 60 The answer is B: Intussesception Mechanical obstruction to the passage of intestinal contents can be caused by (1) a luminal mass, (2) an intrinsic lesion of the bowel wall, or (3) extrinsic compression Obstruction in this case was caused by intussusception, in which a segment of bowel (intussusceptum) protruded distally into a surrounding outer portion (intussuscipiens) This condition is usually a disorder of infants or young children, in whom it occurs without a known cause In adults, the leading point of an intussusception is usually a lesion in the bowel wall, such as Meckel diverticulum or a tumor Once the leading point is entrapped in the intussuscipiens, peristalsis drives the intussusceptum forward In addition to acute intestinal obstruction, intussusception compresses the blood supply to the intussusceptum, which may become infarcted If the obstruction is not relieved spontaneously, treatment requires surgery None of the other choices display “telescoping” of the small intestine Meckle diverticulum (choice C) is an outpouching of the gut caused by perisitence of the embryonic vitelline duct It is the most common congenital anomaly of the small intestine and is usually asymptomatic Peutz-Jeghers polyps (choice D) are hamartomas of the small intestine Volvulus (choice E) is an example of intestinal obstruction and acute abdomen, in which a segment of the gut twists on its mesentery, kinking the bowel and usually interrupting its blood supply Diagnosis: Intussusception 62 The answer is E: Mucocele of the appendix Mucocele refers to a dilated mucous-filled appendix The pathogenesis may be neoplastic or nonneoplastic In the nonneoplastic variety, chronic obstruction leads to the retention of mucus in the appendiceal lumen In the presence of a mucinous cystadenoma (in this case) or mucinous cystadenocarcinoma, the dilated appendix is lined by a villous adenomatous mucosa A mucocele may become secondarily infected and rupture Rupture of a neoplastic mucocele may seed the peritoneal cavity with mucus-secreting tumor cells, a condition referred to as “pseudomyxoma peritonei.” Diagnosis: Mucocele 63 The answer is D: Radiation enterocolitis Radiation therapy for malignant disease of the pelvis or abdomen may be complicated by injury to the small intestine and colon Clinically significant radiation enterocolitis is most common in the rectum The lesions produced by radiation therapy range from a reversible injury of the intestinal mucosa to chronic inflammation, ulceration, and fibrosis of the intestine Diagnosis: Radiation enterocolitis, ovarian cancer Fenderson_Chap13.indd 159 7/12/2010 2:11:06 PM ... pathology V Title: Lipppincott’s illustrated Q and A review of Rubin’s pathology [DNLM: Pathology Examination Questions QZ 18 .2 L765 20 11] RB31.F46 20 11 616.07076—dc 22 2010 025 179 DISCLAIMER Care has... Lippincott’s illustrated Q & A review of Rubin’s pathology / Bruce A Fenderson [et al.] — 2nd ed p ; cm Other title: Illustrated Q & A review of Rubin’s pathology Other title: Lipppincott’s illustrated. ..Fenderson_Index.indd 360 7/13 /20 10 9:01:37 PM Lippincott’s Illustrated Review of Rubin's Pathology SECOND EDITION Bruce A Fenderson, PhD Professor of Pathology Department of Pathology, Anatomy and Cell