title: A case report of adrenocortical carcinosarcoma with oncocytic and primitive neuroectodermal-like features.
abstract: Adrenocortical carcinosarcomas are rare aggressive neoplasms; only a few have been reported to date, all with dismal prognosis. These were reported as having varying morphology. We have encountered a case of adrenal carcinosarcoma with an undifferentiated component bearing similarities to primitive neuroectodermal tumors and other areas of oncocytic differentiation. The 48-year-old woman patient presented with abdominal pain and unintended, excessive weight loss. Computed tomographic imaging revealed a tumor located adjacent to the liver and kidney necessitating a partial nephrectomy and hepatectomy. Histologically, the tumor exhibited malignant features. Melan-A, inhibin, calretinin, cytokeratin AE1/AE3, synaptophysin, and neuron-specific enolase were positive immunohistochemically. The patient developed metastasis within 2 months of surgery and is currently alive with disease after chemotherapy. Adrenal carcinosarcoma is a rare highly aggressive malignancy with a wide morphologic spectrum. Recognition of variant morphology and applying correct immunohistochemical studies will aid in reaching an accurate diagnosis.
authors.affiliation: Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA. yutkao@iupui.edu
authors.forename: Chia-Sui
authors.initials: CS
authors.lastname: Kao
authors.forename: David J
authors.initials: DJ
authors.lastname: Grignon
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Ulbright
authors.forename: Muhammad T
authors.initials: MT
authors.lastname: Idrees
mesh_terms: D018249:Adenoma, Oxyphilic / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D000306:Adrenal Cortex Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D014408:Biomarkers, Tumor / Q000378:metabolism; D002296:Carcinosarcoma / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000556:secondary; D008113:Liver Neoplasms / Q000556:secondary; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D018242:Neuroectodermal Tumors, Primitive / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D024862:Oxyphil Cells / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: Adrenocortical; Carcinosarcoma; Immunohistochemistry; Morphology; PNET; Primitive neuroectodermal tumor; Sarcomatoid carcinoma