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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

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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.

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Acknowledgments

The authors thank Dept. of Pathology, All India Institute of Medical Science, New Delhi, India, and Core Diagnostics for providing consultation.

Conflict of Interests

The authors declare no conflict of interest.

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Authors and Affiliations

  1. Department of Pathology, North DMC Medical College and Hindu Rao Hospital, Delhi, 110007, India

    Leela Pant, Dipti Kalita, Abhijit Das & Gaurav Jain

  2. Department of Surgery, North DMC Medical College and Hindu Rao Hospital, Delhi, India

    Ratna Chopra

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  1. Leela Pant
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  2. Dipti Kalita
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  3. Ratna Chopra
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  4. Abhijit Das
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  5. Gaurav Jain
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Correspondence to Dipti Kalita.

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Pant, L., Kalita, D., Chopra, R. et al. 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. Endocr Pathol 26, 129–134 (2015). https://doi.org/10.1007/s12022-015-9360-0

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