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Analyzes�hundreds of diagnoses, each of which include critical diagnostic information such as definitions, epidemiology, clinical presentation, macro- and microscopic features, differential diagnoses, and pathologic interpretation pearls.�
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Features�thorough updates throughout, including novel immunohistochemical markers and other ancillary techniques (such as BAP1, INSM1, Rb, albumin in situ hybridization) that play an increasingly critical role in improving diagnoses�
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Addresses�recent advances in molecular pathology-new diagnostic, immunohistochemical, molecular, and genetic techniques used for diagnosis, as well as new details about the genesis and activity of tumors in the liver, pancreas, and biliary systems�
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Includes details from the 2019 WHO Classification of Tumors of the Digestive System regarding�important changes to diagnostic techniques, identification of new variants of cancers, and immunohistochemical markers and ancillary techniques that improve diagnosis and treatment options�
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Reflects�recent WHO updates�and�updates from the AJCC's 8th Edition Cancer Staging Manual�such as new terminology, new grading of neuroendocrine tumors and neuroendocrine carcinomas, and characteristic molecular alterations in hepatocellular adenoma, cholangiocarcinoma, and other tumors�
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Uses a�templated page layout, bulleted text, and a generous number of�high-quality images�to help practicing and in-training pathologists reach a confident diagnosis�
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Includes the enhanced eBook version, which allows you to search all text, figures, and references on a variety of devices
Table of Contents
SECTION 1: LIVER
INHERITED, METABOLIC, AND DEVELOPMENTAL DISORDERS
4�Glycogen Storage Disease, Hepatic Joseph Misdraji, MD, Lisa Yerian, MD, and Grace E. Kim, MD 10�Tyrosinemia Joseph Misdraji, MD 12�Niemann-Pick Disease Joseph Misdraji, MD 14�Gaucher Disease Joseph Misdraji, MD 16�Neonatal Hemochromatosis Joseph Misdraji, MD 18�Porphyrin Metabolism Disorders Maria Westerhoff, MD and Joseph Misdraji, MD 24�Dubin-Johnson Syndrome Joseph Misdraji, MD and Sanjay Kakar, MD 26�Gilbert Disease Joseph Misdraji, MD and Sanjay Kakar, MD 28�Progressive Familial Intrahepatic Cholestasis Joseph Misdraji, MD 36�Cystic Fibrosis, Hepatic Joseph Misdraji, MD 40�Hereditary Hemochromatosis Joseph Misdraji, MD and Laura W. Lamps, MD 44�Wilson Disease Joseph Misdraji, MD and Laura W. Lamps, MD 50�Alpha-1-Antitrypsin Deficiency Nicole C. Panarelli, MD, Kari D. Caradine, MD, and Laura W. Lamps, MD 54�Congenital Hepatic Fibrosis Romil Saxena, MBBS, MD, FRCPath (UK) and Hanlin L. Wang, MD, PhD 56�Polycystic Liver Disease Romil Saxena, MBBS, MD, FRCPath (UK) and Hanlin L. Wang, MD, PhD 58�Caroli Disease Romil Saxena, MBBS, MD, FRCPath (UK) � INFECTIOUS DISORDERS� 60�Overview of Hepatitis Nicole C. Panarelli, MD and Laura W. Lamps, MD 66�Acute Viral Hepatitis Nicole C. Panarelli, MD and Lisa Yerian, MD 70�Hepatitis B Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD 74�Hepatitis C Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD 78�Hepatitis E Maria Westerhoff, MD 82�Epstein-Barr Virus Laura W. Lamps, MD, Maria Westerhoff, MD, and Hanlin L. Wang, MD, PhD 86�Cytomegalovirus Laura W. Lamps, MD 88�Herpes Simplex Virus Sanjay Kakar, MD 90�Adenovirus Maria Westerhoff, MD and Hanlin L. Wang, MD, PhD 92�Pyogenic Abscess Laura W. Lamps, MD 96�Sepsis in Liver Laura W. Lamps, MD 98�Mycobacterium tuberculosis Laura W. Lamps, MD 102�Atypical Mycobacteria Laura W. Lamps, MD 106�Cat-Scratch Disease Laura W. Lamps, MD 108�Candidiasis Laura W. Lamps, MD 110�Histoplasmosis Joseph Misdraji, MD and Laura W. Lamps, MD 114�Cryptococcosis Laura W. Lamps, MD 116�Amebiasis Laura W. Lamps, MD 120�Schistosomiasis Laura W. Lamps, MD 122�Echinococcosis Laura W. Lamps, MD
CHRONIC CHOLESTATIC AND AUTOIMMUNE DISORDERS 124�Autoimmune Hepatitis Nicole C. Panarelli, MD and Lisa Yerian, MD 130�Primary Biliary Cholangitis Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD 136�Primary Sclerosing Cholangitis Hanlin L. Wang, MD, PhD 142�Ischemic Cholangitis Maria Westerhoff, MD and Lisa Yerian, MD 144�Large Bile Duct Obstruction Maria Westerhoff, MD and Laura W. Lamps, MD 148�Idiopathic Adulthood Ductopenia Hanlin L. Wang, MD, PhD PEDIATRIC CHOLESTATIC DISORDERS� 150�Biliary Atresia Romil Saxena, MBBS, MD, FRCPath (UK), Grace E. Kim, MD, and Matthew M. Yeh, MD, PhD 154�Idiopathic Neonatal Hepatitis Romil Saxena, MBBS, MD, FRCPath (UK), Grace E. Kim, MD, and Matthew M. Yeh, MD, PhD 156�Syndromic Paucity of Intrahepatic Bile Ducts (Alagille Syndrome) Joseph Misdraji, MD, Romil Saxena, MBBS, MD, FRCPath (UK), and Matthew M. Yeh, MD, PhD 160�Nonsyndromic Paucity of Intrahepatic Bile Ducts Romil Saxena, MBBS, MD, FRCPath (UK), Joseph Misdraji, MD, and Matthew M. Yeh, MD, PhD � DRUG/TOXIN-RELATED HEPATITIS� 164�Drug-Related Acute Hepatitis Sanjay Kakar, MD 170�Drug-Induced Acute Hepatic Failure Sanjay Kakar, MD 174�Drug-Induced Cholestatic Liver Injury Sanjay Kakar, MD 176�Drug-Related Granulomatous Hepatitis Laura W. Lamps, MD 180�Drug-Related Steatohepatitis/Phospholipidosis Laura W. Lamps, MD 184�Reye Syndrome Romil Saxena, MBBS, MD, FRCPath (UK) and Lisa Yerian, MD 186�Drug-Related Cholangitis/Ductopenia Maria Westerhoff, MD 192�Hepatic Stellate Cell Hyperplasia Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD � FATTY LIVER DISEASES� 194�Alcoholic Liver Disease Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD 200�Nonalcoholic Steatohepatitis Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD 208�Glycogenic Hepatopathy Maria Westerhoff, MD and Lisa Yerian, MD 210�Fatty Liver of Pregnancy Nicole C. Panarelli, MD and Lisa Yerian, MD
VASCULAR DISORDERS�
212�Portal Venous Obstruction Hanlin L. Wang, MD, PhD 214�Hepatoportal Sclerosis Hanlin L. Wang, MD, PhD 216�Hepatic Venous Outflow Obstruction Sanjay Kakar, MD 220�Venoocclusive Disease Sanjay Kakar, MD 222 �Amyloidosis Sanjay Kakar, MD 224�Ischemia �Rondell Graham, MBBS and Lisa Yerian, MD 226�Nodular Regenerative Hyperplasia Sanjay Kakar, MD � TRANSPLANTATION PATHOLOGY� 228�Ischemia Reperfusion Injury Romil Saxena, MBBS, MD, FRCPath (UK) and Hanlin L. Wang, MD, PhD 230�Antibody-Mediated Rejection Hanlin L. Wang, MD, PhD and Romil Saxena, MBBS, MD, FRCPath (UK) 232�T-Cell-Mediated Rejection (Acute Cellular Rejection) Romil Saxena, MBBS, MD, FRCPath (UK) 236�Chronic Rejection Romil Saxena, MBBS, MD, FRCPath (UK) and Matthew M. Yeh, MD, PhD 238�Central Perivenulitis/Parenchymal Rejection Rondell Graham, MBBS 240�Hepatic Artery Thrombosis Rondell Graham, MBBS and Lisa Yerian, MD 242�Graft-vs.-Host Disease Rondell Graham, MBBS and Lisa Yerian, MD � TUMORS OF THE LIVER� 244�Hepatic Adenoma Sanjay Kakar, MD and Matthew M. Yeh, MD, PhD 252�Focal Nodular Hyperplasia Sanjay Kakar, MD 256�Regenerative and Dysplastic Nodules Sanjay Kakar, MD 262�Hepatocellular Carcinoma and Variants Sanjay Kakar, MD 274�Fibrolamellar Carcinoma Rondell Graham, MBBS 278�Combined Hepatocellular-Cholangiocarcinoma Maria Westerhoff, MD 282�Hepatoblastoma Rondell Graham, MBBS 286�Bile Duct Adenoma Rondell Graham, MBBS and Sanjay Kakar, MD 288�von Meyenburg Complex (Biliary Microhamartoma) Sanjay Kakar, MD, Laura W. Lamps, MD, and Rondell Graham, MBBS 290�Mucinous Cystic Neoplasm, Liver Maria Westerhoff, MD and Matthew M. Yeh, MD, PhD 294�Intrahepatic Cholangiocarcinoma Rondell Graham, MBBS and Sanjay Kakar, MD 298�Intrahepatic Cholangiocarcinoma Histologic Subtypes Rondell Graham, MBBS 302�Intraductal Papillary Neoplasm Rondell Graham, MBBS 304�Hemangioma Rondell Graham, MBBS 306�Angiomyolipoma Rondell Graham, MBBS and Lisa Yerian, MD 310�Hepatic Small Vessel Neoplasm/Anastamosing Hemangioma Maria Westerhoff, MD 314�Epithelioid Hemangioendothelioma Sanjay Kakar, MD 316�Infantile Hemangioma Sanjay Kakar, MD 318�Angiosarcoma Sanjay Kakar, MD 320�Mesenchymal Hamartoma Nicole C. Panarelli, MD, Hanlin L. Wang, MD, PhD, and Sanjay Kakar, MD 322�Embryonal Sarcoma Nicole C. Panarelli, MD 324�Inflammatory Pseudotumor Joseph Misdraji, MD 330�Segmental Atrophy (Nodular Elastosis) Joseph Misdraji, MD 332�Hepatectomy Specimen Handling Rondell Graham, MBBS and Laura W. Lamps, MD � MISCELLANEOUS HEPATIC DISORDERS� 334�Langerhans Cell Histiocytosis Maria Westerhoff, MD and Hanlin L. Wang, MD, PhD 338�Hemophagocytic Syndromes Hanlin L. Wang, MD, PhD
� DEVELOPMENTAL/CONGENITAL� 342�Congenital Pancreatic Cyst Hanlin L. Wang, MD, PhD 344�Cystic Fibrosis, Pancreas Hanlin L. Wang, MD, PhD 346�Nesidioblastosis Hanlin L. Wang, MD, PhD 348�Choledochal Cyst Romil Saxena, MBBS, MD, FRCPath (UK) and Laura W. Lamps, MD 350�Cholelithiasis Laura W. Lamps, MD 352�Acute Cholecystitis Sanjay Kakar, MD 354�Chronic Cholecystitis Sanjay Kakar, MD 356�Xanthogranulomatous Cholecystitis Laura W. Lamps, MD 358�Eosinophilic Cholecystitis Laura W. Lamps, MD 360�Polyarteritis Nodosa and Other Vasculitides Nicole C. Panarelli, MD 362�Parasitic Infection Laura W. Lamps, MD
NONNEOPLASTIC AND INFLAMMATORY DISORDERS OF THE PANCREAS
366�Acute Pancreatitis Grace E. Kim, MD and Alyssa M. Krasinskas, MD 368�Chronic Pancreatitis Alyssa M. Krasinskas, MD and Lisa Yerian, MD 372�Autoimmune Pancreatitis Alyssa M. Krasinskas, MD and Lisa Yerian, MD 376�Groove Pancreatitis Alyssa M. Krasinskas, MD, Lisa Yerian, MD, and Vikram Deshpande, MD 378�Infectious Pancreatitis Laura W. Lamps, MD 380�Pseudocysts Alyssa M. Krasinskas, MD, Vikram Deshpande, MD, and Lisa Yerian, MD 382�Diabetes Mellitus Laura W. Lamps, MD 384�Lymphoepithelial Cyst of the Pancreas Alyssa M. Krasinskas, MD and Lisa Yerian, MD
TUMORS OF THE GALLBLADDER AND EXTRAHEPATIC BILIARY TREE 386�Noninvasive Epithelial Polypoid Lesions
390�Nicole C. Panarelli, MD and Sanjay Kakar, MD Carcinoma of Gallbladder
396�Nicole C. Panarelli, MD and Laura W. Lamps, MD Carcinoma of Extrahepatic Bile Ducts
402�Rondell Graham, MBBS and Laura W. Lamps, MD Squamous/Adenosquamous Carcinoma, Gallbladder
404�Hanlin L. Wang, MD, PhD Neuroendocrine Neoplasms of Gallbladder
408�Hanlin L. Wang, MD, PhD Granular Cell Tumor
410�Nicole C. Panarelli, MD Embryonal Rhabdomyosarcoma
412�Hanlin L. Wang, MD, PhD Adenomyoma
414�Nicole C. Panarelli, MD and Lisa Yerian, MD Nonneoplastic Polypoid Gallbladder Lesions �Nicole C. Panarelli, MD and Laura W. Lamps, MD � TUMORS OF THE PANCREAS� 418�Pancreatic Intraepithelial Neoplasia Alyssa M. Krasinskas, MD and Sanjay Kakar, MD 420�Ductal Adenocarcinoma, Including Variants Alyssa M. Krasinskas, MD, Sanjay Kakar, MD, and Mari Mino-Kenudson, MD 424�Undifferentiated Carcinoma Wei Zheng, MD, Sanjay Kakar, MD, and Alyssa M. Krasinskas, MD 426�Adenosquamous Carcinoma, Pancreas Alyssa M. Krasinskas, MD 428�Serous Cystadenoma Sanjay Kakar, MD and Grace E. Kim, MD 432�Acinar Cell Cystadenoma Maria Westerhoff, MD �
436�Simple Mucinous Cyst Maria Westerhoff, MD 438�Mucinous Cystic Neoplasm, Pancreas Maria Westerhoff, MD 442�Intraductal Papillary Mucinous Neoplasm Mari Mino-Kenudson, MD, Zaid Mahdi, MD, and Alyssa M. Krasinskas, MD 448�Intraductal Oncocytic Papillary Neoplasm Alyssa M. Krasinskas, MD and Mari Mino-Kenudson, MD 450�Intraductal Tubulopapillary Neoplasm Alyssa M. Krasinskas, MD and Mari Mino-Kenudson, MD 452�Acinar Cell Carcinoma Laura W. Lamps, MD, Wei Zheng, MD, and Alyssa M. Krasinskas, MD 456�Pancreatoblastoma Alyssa M. Krasinskas, MD, Laura W. Lamps, MD, and Matthew M. Yeh, MD, PhD 458�Dermoid Cyst Zaid Mahdi, MD, Vikram Deshpande, MD, and Alyssa M. Krasinskas, MD 460�Poorly Differentiated Neuroendocrine Carcinoma, Pancreas Sanjay Kakar, MD 464�Well-Differentiated Neuroendocrine Tumor, Pancreas Sanjay Kakar, MD 470�Solid-Pseudopapillary Tumors Sanjay Kakar, MD � TUMORS OF THE AMPULLA� 474�Ampullary Adenoma Hanlin L. Wang, MD, PhD 476�Ampullary Adenocarcinoma and Variants Hanlin L. Wang, MD, PhD 480�Well-Differentiated Neuroendocrine Tumor, Ampulla Hanlin L. Wang, MD, PhD 482�Gangliocytic Paraganglioma Maria Westerhoff, MD � SPECIMEN HANDLING, WHIPPLE� 484�Specimen Handling, Whipple Alyssa M. Krasinskas, MD and Laura W. Lamps, MD
Authors
Laura W. Lamps Professor and Vice-Chair for Academic Affairs, Department of Pathology,
University of Arkansas for Medical Sciences,
Little Rock, Arkansas. Maria Westerhoff Associate Professor, Department of Pathology, University of Michigan. Sanjay Kakar
