Hirsutismo y amenorrea en carcinoma adrenocortical oncocítico hormonalmente activo. Aportación de un caso y revisión de la literatura

Adrenocortical carcinoma is a rare and aggressive cancer and its prognosis is frequently unsatisfactory. Oncocytic neoplasms are an exceptional variant of adrenocortical carcinoma and most rarely found in the adrenal gland. They are usually benign and non-functioning. We present a case of a testosterone-secreting oncocytic adrenocortical carcinoma in a 37-year-old female who presented with acne, hirsutism and irregular menses. Clinical investigations revealed an elevated testosterone and DHEA-S and a 11 × 8 cm left adrenal mass. The tumour was successfully excised. Histopathological result of adrenal mass showed the tumour to be comprised of oncocytic cells with granular, eosinophilic cytoplasm, features consistent with an oncocytic carcinoma. However, follow-up computed tomography at 2 months after the left adrenalectomy revealed lung metastasis. Surgery is associated with improved survival, even in metastatic disease. Therefore, surgery should be considered for select patients as part of multimodality treatment. Cytotoxic chemotherapy and mitotane have been utilized with a variable degree of benefit and few long-term responses. Then we deal with a literature review to highlight and summarize most significant aspects of epidemiology, clinical, diagnosis, prognosis and therapy.