Adrenal Perivascular Epithelioid Cell Tumor: A Case Report With Discussion of Differential Diagnoses

Abstract

A 50-year-old man presented with nausea and abdominal bloating sensation of a few months' duration and was found to have a large adrenal mass that was resected after computed tomography scan evaluation. The histopathologic examination showed epithelioid and spindle cells having clear to granular eosinophilic cytoplasm, and small, central, round to oval nuclei with small nucleoli. The tumor cells demonstrated reactivity for smooth muscle actin, HMB-45, Melan-A, and vimentin, with focal positivity for desmin and S100 protein and negative staining for inhibin, synaptophysin, and chromogranin. A diagnosis of perivascular epithelioid cell tumor was made. Perivascular epithelioid cell tumors can arise in a variety of locations, including omentum/mesentery, female genital tract, pelvic soft tissues, skin, and falciform ligament, but an adrenal primary tumor has not been previously reported, to the best of our knowledge. The differential diagnosis is broad and site-dependant and in our case includes several neoplasms with clear cells that can involve the adrenal gland, including a primary adrenocortical neoplasm. A review of the related literature and discussion of the differential diagnoses are presented.

Metastatic Adrenal PEComa: Case Report and Short Review of the Literature

“…The present case represents one of few examples of malignant PEComa that arise in the adrenal gland. In the literature, only three cases have been reported in the adrenal gland to the best of our knowledge [ 10 , 11 ]. The literature reports that PEComa has a higher incidence in females (6–7:1), and the age at diagnosis ranges from 8–89 years.…”

Diagnostic dilemma for an adrenal mass: perivascular epithelioid cell neoplasm versus adrenocortical carcinoma

“…They are composed of perivascular epithelioid cells (with no known normal counterpart), distributed as nest and sheets with clear to granular eosinophilic cytoplasm and an association with blood vessel walls 1 . Furthermore, PEComas stain positive for HMB45, HMSA‐1, Melan A, MITF, actin and less commonly desmin 2 . ACCs originate from cells of the adrenal cortex and should be diagnosed by an experienced pathologist using the Weiss criteria for adrenocortical tumours, with local invasion or distant metastasis confirming malignancy 3 .…”

“…R0 surgical resection is the management of choice for isolated PEComas and ACCs 2,5 . There is no standard approach to the resection of a retroperitoneal mass.…”

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.…”