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

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.

The Pathologic Point of View of Laparoscopic Adrenalectomy in the Era of Radiologic Imaging: A Multicenter Retrospective Study

“…The remaining consists of benign cysts, adrenocortical hyperplasias, schwannomas, ganglioneuromas, lymphangiomas, liposarcomas, lymphomas, cavernous hemangiomas, angiomyolipomas, angiosarcomas and leiomyosarcomas [5,9]. Additionally, there are some rarely seen pathologies, most of which have been presented as case reports, including plasmacytoma, ganglioneuroblastoma, sarcomatoid carcinoma, leiomyosarcoma, cavernous hemangioma, malignant perivascular epithelioid tumor, primitive neuroectodermal tumor, oncocytoma, lymphangioma, fibrous tumor and lymphoma [10,11,12,13,14,15,16,17,18,19,20]. In addition to these, in the present study, 1 malignant mesenchymal tumor, 1 meningomyelocele and 1 granulomatous lymphadenitis were observed.…”

Surprising genetic and pathological findings in a patient with giant bilateral periadrenal tumours: PEComas and mutations of PTCH1 in Gorlin-Goltz syndrome

“…A case of bilateral cystic adrenal lymphangiomas in GGS has been described [12]. Rare cases of adrenal PEComa including malignant tumors have been documented without clinical suspicion for GGS [13,14]. In our case, however, despite the vicinity of both adrenal glands, the retroperitoneal PEComas were ultimately found to be independent of the adrenals.…”

PEComa of the Adrenal Gland

“…Although PEComas have a predilection for deep soft tissue, they can also arise at visceral sites, with the most common being the uterus and gastrointestinal tract 2,3 . Adrenal gland PEComas are very rare and, to our knowledge, have only been documented as case reports 4–6 . Some of these have been referred to as epithelioid angiomyolipomas; however, they lack the classic triphasic morphology of angiomyolipoma and, more importantly, have the potential for aggressive clinical behavior.…”

“…2,3 Adrenal gland PEComas are very rare and, to our knowledge, have only been documented as case reports. [4][5][6] Some of these have been referred to as epithelioid angiomyolipomas; however, they lack the classic triphasic morphology of angiomyolipoma and, more importantly, have the potential for aggressive clinical behavior. In the adrenal gland, PEComas may be particularly difficult to recognize, given their morphologic overlap with much more common adrenal cortical adenomas and carcinomas.…”