AJSP: Reviews and Reports

Langerhans cell histiocytosis (LCH) is a clonal neoplasm characterized by aberrant proliferation of S100, CD1a, and langerin-positive Langerhans cells. Langerhans cell histiocytosis most commonly presents in the lung as a solitary pulmonary eosinophilic granuloma, but can be disseminated involving the skin and central nervous system. Hepatic involvement by LCH in adults is extremely rare and considered at high risk for mortality. The early stage is easily recognizable with sinusoidal involvement by Langerhans cells. However, the later manifestation of sclerosing cholangitis predominantly affects the perihilar bile ducts and has patchy involvement of the distal bile ducts. This histologically can overlap with primary sclerosing cholangitis and creates a diagnostic dilemma in adults. Sclerosing cholangitis associated with LCH requires liver transplantation for definitive cure. We present the case of a 48-year-old man who had a biopsy with findings of sclerosing cholangitis but no definitive involvement by LCH. He underwent an orthotopic liver transplant showing obliteration of the perihilar bile ducts by Langerhans cells and associated histologic features of sclerosing cholangitis, 16 years after an initial diagnosis of pulmonary LCH.

Krithika Shenoy

Kathleen Byrnes

AJSP Review and Reports

Adult Langerhans Cell Histiocytosis Presenting as Sclerosing Cholangitis in a Liver Explant

成人朗格汉斯细胞组织细胞增生症表现为肝移植中的硬化性胆管炎

Disseminated mucormycosis is a rare, life-threatening disease generally seen in immunocompromised patients. We report the case of a 34-year-old alcoholic man who presented to the hospital with abdominal pain, chills, and hypotension and subsequently passed 2 weeks later. Postmortem examination showed multiorgan necrosis and infarction. The stomach mucosa was notable for an encompassing, granular, yellow-tinged, velvety pseudomembrane. Histopathologic examination showed lungs, spleen, stomach, and kidneys with angioinvasive branching fungal hyphae, which was confirmed as Mucorales species through metagenomic sequencing. To our knowledge, this is the first reported case of pseudomembranous gastritis secondary to mucormycosis.

Tiffany Lin

Paige M.K. Larkin

Shangxin Yang

Gregory A. Fishbein

Pseudomembranous Gastritis Secondary to Mucormycosis in Patient With Chronic Alcoholism

慢性酒精中毒患者继发于毛霉菌病的假膜性胃炎

Abstract The morphologic overlap between low-grade spindle cell lesions can lead to diagnostic dilemmas, particularly when attempting to interpret limited material. Evaluation of such specimens frequently requires correlating with clinical and imaging findings, as well as ancillary studies. We discuss the case of a 78-year-old woman with a remote history of breast carcinoma who presented with a left base of neck nodule. While imaging findings were highly suggestive of schwannoma, fine-needle aspiration biopsy of the nodule was performed for diagnostic confirmation. The patient reported tingling and an electrical shock sensation traveling down her arm during the fine-needle aspiration procedure. Cytomorphologic findings demonstrating a low-grade spindle cell neoplasm further supported a diagnosis of schwannoma, despite early immunohistochemistry results not being supportive. A last-minute suggestion that the differential diagnosis be broadened prompted additional immunohistochemical workup and follow-up molecular testing that confirmed an alternative diagnosis of solitary fibrous tumor. This case demonstrates the importance of correlating clinical, radiologic, and pathologic findings when approaching the differential diagnosis of low-grade spindle cell lesions on fine-needle biopsy specimens. Ancillary testing including immunohistochemistry, molecular studies, or fluorescence in situ hybridization is frequently utilized to establish a definitive diagnosis.

Taylor Bronson

Bonnie Choy

Wolves in Sheep's Clothing and Vice Versa: Fine-Needle Aspiration of Low-Grade Spindle Cell Lesions

披着羊皮的狼与反之：低级别梭形细胞病变的细针穿刺

Abstract Typical and atypical carcinoids account for 2% of all pulmonary neoplasms and are usually diagnosed based on characteristic neuroendocrine granules, architecture, and overall bland cytomorphology. However, there are rare carcinoids with unusual morphology mimicking features seen in non–small cell lung carcinoma, which can be a significant challenge on small biopsy or cytology specimens with major therapeutic implications. We report a case of a transbronchial needle aspirate that was initially misinterpreted as non–small cell lung carcinoma due to the presence of cohesive clusters of tumor cells showing striking pleomorphism and prominent nucleoli in a background of necrosis. In addition, tumor cells showed multifocal brown cytoplasmic melanin pigment that has rarely been reported in carcinoids. The diagnostic pitfalls of a pleomorphic carcinoid and the possible limitations of reaching this diagnosis on small biopsy specimens are discussed.

Naomi Hardy

Jonathan Jacobs

Paul Staats

Allen Burke

Kristen Stashek

Laura Malone

Pleomorphic Carcinoid: A Pitfall in Diagnosis on Small Biopsy Specimens

多形性类癌：小活检标本诊断中的陷阱

Abstract We report a renal tumor originally diagnosed as oncocytoma that recurred with biopsy-proven multiple liver metastases. Review of the initial tumor revealed a predominantly CK7-negative neoplasm that had areas resembling oncocytoma, areas resembling eosinophilic chromophobe renal cell carcinoma, and areas with intermediate features. The case illustrates that the distinction between eosinophilic chromophobe renal cell carcinoma and “oncocytic tumor of uncertain malignant potential” is in some cases subjective. Criteria need better definition and should not be based on cytokeratin 7 staining pattern alone. A discussion of the current classification of oncocytic renal tumors is included, as well as a discussion of the differential diagnosis of renal neoplasms with eosinophilic cytoplasm.

Teklu Legesse

Adina Paulk

Low-Grade Oncocytic Renal Tumor With Massive Liver Metastasis: Do Not Trust a Negative CK7 and Misdiagnose Oncocytoma: A Cautionary Tale and Current Classification of Oncocytic Renal Tumors

低级别嗜酸性肾肿瘤伴大量肝转移：不要因CK7阴性而误诊为嗜酸性细胞瘤：一个警示故事及当前嗜酸性肾肿瘤分类

Abstract Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a rare primary liver tumor with neoplastic components of both hepatocytic and cholangiocytic differentiation within the same tumor, such as the case reported herein. There is a need to standardize the pathological diagnosis of cHCC-CCA and to improve the concordance of diagnosis among pathologists based on consensus paper because of its histological and phenotypic diversity that leads to confusion in terminology and classification. This case review addresses the terminology, classification, and clinical and pathological characteristics of cHCC-CCA and its differential diagnosis.

Reiichiro Kondo

Jun Akiba

Hirohisa Yano

A Case Review on Combined Hepatocellular Cholangiocarcinoma

肝细胞胆管细胞癌联合病例回顾

Abstract Hepatocellular adenoma (HCA) is a benign liver neoplasm that typically arises in the noncirrhotic liver. Based on molecular findings, HCA can be subclassified into the following subtypes: hepatocyte nuclear factor 1α–inactivated HCA, inflammatory HCA, β-catenin–activated HCA, sonic hedgehog-activated HCA, and unclassified HCA. Mixed β-catenin–activated inflammatory HCA may rarely occur. We report a case of HCA that was initially diagnosed as inflammatory HCA on a biopsy but finally was confirmed to be β-catenin–activated inflammatory HCA on the resection specimen. Most recent updates on molecular classification, key histologic features of each subtype, and common diagnostic pitfalls are discussed.

Geunyoung Jung

Yongjun Liu

Hepatocellular Adenoma: A Case Report, Current Updates on Subtyping, and Diagnostic Pitfalls

肝细胞腺瘤：病例报告、亚型分类的最新进展及诊断陷阱

Abstract Primary liver cancers comprise a heterogeneous group of neoplasms, with the 2 main entities being hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (iCCA). Over the past decades, HCC and iCCA have been recognized to be heterogeneous in morphology, molecular features, and biological behavior, and the most recent World Health Organization classification of digestive system tumors describes various histomorphological subtypes of HCC and iCCA, some of which also have characteristic molecular features, clinical correlates, and prognostic implications. In this review, we discuss 4 recently described subtypes of HCC and iCCA—macrotrabecular HCC, steatohepatitic HCC, scirrhous HCC, and small duct iCCA—based on a series of cases.

Yoon Jung Hwang

Haeryoung Kim

Histomorphological Subtypes of Hepatocellular Carcinoma and Intrahepatic Cholangiocarcinoma: Review and Update

肝细胞癌和肝内胆管癌的组织形态学亚型：综述与更新

Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with a high mortality. Liver involvement is common, with most patients demonstrating acute hepatitis. Therefore, liver biopsies are frequently obtained in the setting of suspected HLH. Several studies have described the clinicopathologic manifestations of liver involvement by HLH. The histology is highly variable, as are rates of demonstrated hemophagocytosis. This review summarizes the clinical characteristics of HLH, with particular emphasis on hepatic manifestations thereof. We then describe the histologic patterns that have been commonly and uncommonly reported. Finally, we address difficulties faced by liver pathologists when considering this entity and offer our opinions regarding the worst “pain points.”

Yuqing Xiong

Armando Del Portillo

Ladan Fazlollahi

Helen E. Remotti

Michael Lee

Stephen M. Lagana

Hemophagocytic Lymphohistiocytosis: A Practical Review for Liver Pathologists

噬血细胞性淋巴组织细胞增多症：肝脏病理学家的实用综述

This issue of the American Journal of Surgical Pathology, Reviews and Reports is devoted to recent advances and update in the pathology of the liver. The articles provide insights into the current nomenclature, classification, diagnosis, differential diagnoses, clinical and prognostic aspects of various liver diseases, and lesions that are informative to pathologists in their daily practice. Hepatocellular adenomas (HCAs) is generally a benign liver neoplasm that typically arises in the noncirrhotic liver; however, malignant transformation does occur, and hence, subtyping is critical. Drs Jung and Liu present a case of HCA that was initially diagnosed as inflammatory HCA on a biopsy and subsequently was confirmed to be β-catenin–activated inflammatory HCA on the resection specimen. They then comprehensively review recent updates on molecular classification and the key histologic features of each subtype and discuss common diagnostic pitfalls. Over the past decades, hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (iCCA) have been recognized to be heterogeneous in morphology, molecular features, and biological behavior. Recent World Health Organization classification describes various histomorphological subtypes of HCC and iCCA, some with characteristic molecular profiles, clinical correlates, and prognostic implications. In their review, Drs Hwang and Kim nicely discuss recently described subtypes of HCC and iCCA: macrotrabecular HCC, steatohepatitic HCC, scirrhous HCC, and small duct iCCA. Congestive hepatopathy shows a unique pattern of fibrosis, that is, pericentral fibrosis at early stage, which may progress to bridging fibrosis and cirrhosis. Making a diagnosis of congestive hepatopathy and staging its fibrosis are important tasks for pathologists, as patients may undergo liver biopsies before cardiac transplantation to assess the degree of liver fibrosis, which may impact clinical decision-making. Besides patients' own cardiac dysfunction, congestive hepatopathy can also be seen after a Fontan procedure. Drs Gosse and Bosch describe a case of congestive hepatopathy due to altered circulation with a Fontan procedure. They then discuss the fibrosis scoring systems recently described for Fontan-associated liver disease and congestive heart disease–associated hepatopathy. Combined hepatocellular-cholangiocarcinoma is a rare primary liver tumor with neoplastic components of both hepatocytic and cholangiocytic differentiation within the same tumor. There is recent consensus paper and World Health Organization classification of this unique tumor. Drs Kondo, Akiba, and Yano herein report a case of this tumor and thoughtfully review the terminology, classification, and clinical and pathological characteristics of combined hepatocellular-cholangiocarcinoma and its differential diagnosis. Hemophagocytic lymphohistiocytosis is a rare disease with a high mortality. Liver involvement is common, with most patients demonstrating acute hepatitis. Therefore, liver biopsies are frequently performed when this entity is suspected. Dr Xiong and colleagues summarize the clinical features of hemophagocytic lymphohistiocytosis, emphasizing its hepatic manifestations. They describe the highly variable histologic patterns and address difficulties encountered by pathologists. We are extremely grateful to all the contributors for sharing their expertise and knowledge in forming this issue. We hope that the information provides highlights and elucidates the practically encountered lesions in the liver. Guest Editors

Matthew M. Yeh

Glenda Pua

Update in Pathology of Practically Encountered Lesions and Diseases in the Liver

肝脏常见病变及疾病病理学进展

ISSN	2381-5949, 2381-652X
h-index	12

Publication Information	Publisher: Wolters Kluwer Health，Publishing cycle: ，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 2020，Proportion of original research papers: ，Self Citation Rate:， Gold OA Rate:

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