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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