Endocrine Abstracts

Introduction: Adrenocorticalcarcinoma (ACC) is a rare malignancy with an aggressive prognosis, an incidance of 1–2 cases/million/year. It is the cause of 0.2% of all cancer deaths. It is seen in the 4th and 5th decades in adults and mostly unilateral and sporadic. Herein, we present a case of sarcomatoid type ACC which is symptomatic with mass compression and hypertansive attacks.

Case presentation: A-52-year-old male was referred to our endocrinology clinic with and abdominal pain and abdominal distension. Patient’s history revealed intermittent episodes of hypertension responding to nifedipine treatment. Computed Tomography (CT) revealed left adrenal mass which was 9×14 cm. The patient was admitted to the endocrinology inpatient clinic with the diagnosis of pheochromocytoma. 24-hr-urinary normetanephrine level 1058 pg/24hr (N: 88–444), metanephrine 200 pg/hr (N: 276–341). 1-mg dexamethasone-suppression test serum cortisol was 0.56 mg/dL (N: 4.6–22.8), 24-hr-urinary cortisol 39 nmol/L (N:38–208), plasma renin 2.06 ng/ml/h, aldosterone 22 pg/dL, dehyroepiandesterone sulfat (dhea-s) 110 ug/dl (N: 34.5-568.9). Tumor markers were normal. With aggravation of abdominal pain CT was performed again and it revealed a 10X14 cm wide necrotic area, heterogeneous enhancement of the left diaphragm, spleen, left renal vein and artery. The patient died on first postoperative day due to hypotensive shock. The patient was diagnosed as sarcomatoid type ACC with the presence of calretinin (−), oscar-ck (+), synaptophysin (−), chromogranin (−), cytokeratin-7 (+), smooth muscle-4 (+). According to weiss criteria the mass was evaluated as high nuclear grade atypical mitotic fugures, venous sinusoidal and encapsuleted invasion.

Discussion: There are two types of adrenocortical carsinoms; functioning and nonfunctioning tumors. Nonfunctioning ones can present with metastasis, mass compression and invasion findings. Sarcomatoid carcinoma is a rare variant of ACC. When confronted with the surrenal mass, ACC is a pathology that should be kept in mind adult adrenocortical carsinoma has poor diagnosis underscoring the importance of identifying diagnostic and prognostic markers.

Keywords: Adrenocortical carsinoma, sarcomatoid