Abstract
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.
Highlights
Adrenocortical carcinoma (ACC) is a rare primary adrenal neoplasm with an incidence of 1-2 cases per million per year [1]
We report a case of a 55-year-old male with newly diagnosed hypertension being evaluated for left adrenal mass concerning for adrenocortical carcinoma
We report a case of oncocytic ACC with emphasis on management, histopathological features and immunohistochemical profile of the tumor aiding in its distinction from the benign adrenocortical oncocytoma and conventional ACC
Summary
Adrenocortical carcinoma (ACC) is a rare primary adrenal neoplasm with an incidence of 1-2 cases per million per year [1]. A 55-year-old man was referred to our hospital for management of a left suprarenal mass detected on ultrasonography done for evaluation of persistent pain in his left hypochondrium. He was recently diagnosed to have hypertension and diabetes mellitus. Examination of the abdomen revealed a mass occupying the left hypochondrium and the left lumbar region. His serum cortisol, metanephrine and normetanephrine levels were within normal limits while dehydroepiandrosterone (DHEA) was elevated. Positive for melan A and negative for inhibin, CD117, and chromogranin (Figure 2)
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