Sarcomatoid Adrenal Cortical Carcinoma: Report of a Rare Case

Abstract

Abstract Introduction/Objective Adrenal cortical carcinoma (ACC) is a rare neoplasm with oncocytic, myxoid, and sarcomatoid variants. Sarcomatoid is the least common variant. To our knowledge, there are only 23 cases of sarcomatoid ACC reported in the English literature. Typically, ACC’s express SF1, Melan A, inhibin, and synaptophysin. It has been reported that the sarcomatoid component does not express the aforementioned immunomarkers; however, it can display mutational expression of p53 and β-catenin. Methods/Case Report A 77-year-old man was incidentally found to have a left adrenal gland mass. Hormone levels were unremarkable, and adrenalectomy was performed. Gross examination of the specimen revealed a 4.5 cm well-circumscribed tan and focally hemorrhagic mass confined to the adrenal gland. Histologically, the mass was composed of pleomorphic round to polygonal cells with clear to eosinophilic cytoplasm and spindle cells. The mitotic count exceeded 20 in 50 high power fields. Lymphovascular invasion and extensive necrosis were present. Immunohistochemical studies revealed that the sarcomatoid component expressed SF1 and synaptophysin focally. The non-sarcomatoid component exhibited focal expression of synaptophysin and diffuse expression of SF1 and Melan A. Both components displayed null expression of p53, and neither expressed β-catenin. Ki67 proliferation index was > 95% in the sarcomatoid component. Results (if a Case Study enter NA) NA Conclusion In sarcomatoid ACC, both sarcomatoid and non-sarcomatoid components have been shown to harbor TP53 and CTNNB1 mutations, with the latter leading to activation of Wnt pathway and causing diffuse and strong nuclear expression of β-catenin. In the present case, tumor cells lacked expression of p53, which might have played a role in the evolution of the tumor.