Abstract
Adrenocortical carcinosarcoma is an extremely rare and aggressive variant of adrenocortical carcinoma characterized by the presence of both carcinomatous and sarcomatous components, with the latter often showing heterologous differentiation. Due to the rarity and unusual histology, it may pose a diagnostic challenge. In order to increase awareness and identify potential diagnostic pitfalls, we report the ninth case of non-functioning adrenocortical carcinosarcoma in a 45-year-old man who presented with worsening epigastric pain and a left large retroperitoneal mass in close proximity to the body/tail of pancreas and third portion of the duodenum with displacement of the kidney without parenchymal invasion and multiple liver nodules detected by computed tomographic scan. On en bloc resection, the tumor grossly did not involve the pancreas, kidney or colon. Histologically, the tumor was composed of two distinct components - an epithelioid component with granular cytoplasm that stained for synaptophysin, Melan-A, calretinin, and vimentin compatible with adrenocortical differentiation, and a pleomorphic to spindled component that was positive for desmin and myogenin, compatible with rhabdomyosarcomatous differentiation. A wedge biopsy of a liver nodule showed morphologic features similar to the epithelial component of the primary tumor. The patient died three months after surgery due to locoregional and distant recurrence. Adrenocortical carcinosarcoma is a rare malignancy that adds to the differential diagnostic considerations for a retroperitoneal epithelioid malignancy. Awareness of this as a possibility will help in distinguishing this tumor from other carcinomas, melanomas, and true sarcomas.
Highlights
Adrenocortical carcinoma is a rare but highly aggressive malignancy with an estimated annual incidence of between 1.5 to 2 per million population [1]
According to WHO classification 2004 in other epithelial malignant neoplasms, these tumors are classified as sarcomatoid carcinoma
We showed that sarcomatous component of the tumor focally retains positivity for Melan-A, synaptophysin and calretinin, supporting the notion that sarcomatous area of the tumor has originated from the adrenocortical carcinoma rather than representing a
Summary
Adrenocortical carcinoma is a rare but highly aggressive malignancy with an estimated annual incidence of between 1.5 to 2 per million population [1]. The epithelioid component consisted of sheet and nests of loosely cohesive polygonal cells with clear and eosinophilic cytoplasm resembling adrenocortical cells (Fig. 3A) These cells showed highly atypical nuclei and large eosinophilic nucleoli (Fuhrman’s grade III) with high mitotic activity (average of seven mitoses per 10 highpower fields). The pleomorphic/spindled component, comprising approximately 75% of viable tumor, showed predominantly spindle-shaped cells arranged in fascicular pattern with highly pleomorphic nuclei with dense irregularly clumped chromatin and prominent dense eosinophilic nucleoli (Fig. 3B) and occasional anaplastic multinucleated neoplastic giant cells. Identified in these areas were large elongated or ovoid cells with abundant deeply eosinophilic cytoplasm and eccentrically located nuclei and prominent nucleoli, suggestive of rhabdomyosarcomatous differentiation (Fig. 3C). Based on the histologic and immunohistochemical profile, the tumor was diagnosed as an adrenocortical carcinosarcoma
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