Adrenocortical carcinoma is a rare cancer. Oncocytic tumors of the adrenal gland are rarer. Most Oncocytic
Adrenal Neoplasms are benign and carry favourable prognosis. They are classified as oncocytoma,
oncocytic neoplasm of uncertain malignant potential and oncocytic adrenal carcinoma. The malignant
nature of oncocytic neoplasm of adrenal gland can only be confirmed on histopathology. We report a case
of a 55-year-old male with newly diagnosed hypertension being evaluated for left adrenal mass concerning
for adrenocortical carcinoma. Open radical left adrenalectomy and nephrectomy was done and
histopathology confirmed oncocytic variant of adrenocortical carcinoma based on Lin-Weiss-Bisceglia
scoring system which has been developed particularly for oncocytic type of tumor. Though rare, oncocytic
neoplasm has to be considered as one of the differential diagnoses of adrenocortical mass, especially those
presenting as a large mass because malignant oncocytic neoplasm of adrenal gland as large as 23cm have
been reported. Imaging modalities like ultrasonography, computed tomography or magnetic resonance
imaging, though useful in evaluating an adrenocortical mass, cannot predict malignant nature of an
oncocytic neoplasm. Diagnosis of adrenocortical carcinoma is therefore reliably made only after
histopathological examination of the surgical specimen. Surgical resection in those presenting with nonmetastatic resectable disease remains the mainstay of ACC treatment. Oncocytic ACC compared with
conventional ACCs matched for age, gender, disease stage and status of surgical resection, shows significant
better overall survival thus representing more indolent variant of an aggressive and often fatal disease.