Functional Adrenocortical Oncocytoma: A Case Report of Rare Neoplasm of Uncertain Malignant Potential

Adrenocortical oncocytoma is a rare adrenal neoplasm with only 25 cases having been reported in the English medical literature, of which only seven were functional tumors. Since these adrenal tumors are usually nonfunctional, they are mostly incidentally detected, and most of them are benign. Herein, we report on a rare case of a functional adrenocortical oncocytoma of an uncertain malignant potential and this tumor was located in the left adrenal gland in a 59-year-old woman who presented with hypertension. The tumor size was large with foci of necrosis in the cut surface and it exclusively had oncocytic histologic features.

Beckwith-Wiedemann sydrome with left adrenal cortical neoplasm

Beckwith-Wiedemann sydrome is a multisystemic pattern of congenital anomalies with overgrowth. This syndrome is first described independently by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We experienced a case of Beckwith-Wiedemann syndrome who developed left adrenal cortical neoplasm of indeterminate malignant potential.

Adrenocortical Oncocytoma: A case report

Adrenal gland is a rare location for an oncocytic neoplasm. In English literature less than 10 cases of adrenocortical oncocytoma have been reported. We have experienced a case of adrenocortical oncocytoma in a 35-year-old man which was detected incidentally during the ultra-sonographic evaluation of the abdomen for a routine physical examination. This case did not demonstrate any clinical evidence of adrenocortical abnomalities, such as virilization or hypertension. Grossly, the tumor was light to dark tan on cut surface. Light-microscopic examination revealed tumor cells with abundant lipid- sparse eosinophilic cytoplasm and occasional pleomorphic nuclei. Mitotic figures were less than 5/50 HPFs. Tumor cells were positive for vimentin but negative for pancytokeratin, CAM 5.2, chromogranin and synaptophysin. Ultrastructural examination demonstrated abundant mitochondria containing occasional intramitochondrial dense bodies or inclusions.