Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Adrenal Gland: Report of a Rare Case Posing Diagnostic Cha
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Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Adrenal Gland: Report of a Rare Case Posing Diagnostic Challenge with the Role of Immunohistochemistry in the Diagnosis Leela Pant & Dipti Kalita & Ratna Chopra & Abhijit Das & Gaurav Jain
# Springer Science+Business Media New York 2015
Abstract Histological diagnosis of adrenal tumors is often challenging as diverse groups of tumors, both primaries and metastatic, may be seen in the adrenal gland with overlapping morphological features. Immunohistochemistry (IHC) plays the most important role in their diagnosis. Perivascular epithelioid cell tumor (PEComa), a rarely reported tumor in the adrenal gland, shares many features with another rare tumor sarcomatoid adrenocortical carcinoma (ACC). Extensive immunohistochemical study is required to distinguish this tumor from adrenocortical carcinoma and from other morphologically similar tumors. The unique combination of immunoreactivity for melanocytic markers, such as HMB-45 and Melan A, and myogenic markers, such as smooth muscle actin, is the hallmark of PEComas biological behavior, and prognosis of malignant PEComas is yet to be fully understood. Few cases of malignant PEComa have been reported in the adrenal gland. We report a case of malignant PEComa of the adrenal gland posing diagnostic challenge and compare its morphological and immunohistochemical features with those of sarcomatoid ACC.
Keywords PEComa . Sarcomatoid adrenocortical . Carcinoma . Immunohistochemistry
L. Pant : D. Kalita (*) : A. Das : G. Jain Department of Pathology, North DMC Medical College and Hindu Rao Hospital, Delhi 110007, India e-mail: [email protected] R. Chopra Department of Surgery, North DMC Medical College and Hindu Rao Hospital, Delhi, India
Introduction Histological diagnosis of adrenal tumors is often challenging as diverse groups of tumors, both primaries and metastatic, may be seen in adrenal gland with overlapping morphological features. Immunohistochemistry (IHC) plays the most important role in their diagnosis. Among primary tumors, adrenal cortical carcinoma (ACC) with sarcomatoid features and malignant perivascular epithelioid cell tumor (PEComa) are rare, with few cases reported in literature. These tumors share histomorphological and immunohistological features with many other tumors. An extensive panel of IHC markers is required to distinguish them from each other. Here, we report a case of an adrenal gland tumor showing both epithelioid and spindle cell patterns posing diagnostic challenge, and our aim is to discuss the differential diagnosis based on morphology and immunohistochemical features.
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