Abstract
ABSTRACTAndrogen secreting adrenocortical carcinoma (ACC) is a very rare disease with a poor prognosis. Approximately 80% of tumors are functional, most commonly secreting glucocorticoids. We herewith report a case of a huge functional ACC of the right adrenal gland in a 33-year-old female who presented with complaints of hirsutism, amenorrhea and an abdominal lump. On abdominal examination a large lump was palpable in the right hypochondrium reaching up to the umbilicus. Contrast-enhance computed tomography (CECT) revealed a mass in the right suprarenal region. The tumor measured 29 cm × 20 cm × 12 cm and weighed 7.8 kg, the largest reported case of ACC in the world to the best of our knowledge.
Highlights
Adrenocortical carcinomas (ACC) are rare tumors with an incidence of 0.5-2 cases per one million population per year and constitute 0.05-0.2% of all malignancies.[1]
We report a case of the largest androgenproducing, non-metastasizing ACC mass till date
After performing an extensive google search of all the articles published on ACCs, to date, we report this case as the largest ACC in size in the world
Summary
Adrenocortical carcinomas (ACC) are rare tumors with an incidence of 0.5-2 cases per one million population per year and constitute 0.05-0.2% of all malignancies.[1]. An abdominal lump was found in the right hypochondrium reaching up to the umbilicus and midline measuring ~ 20×20×15 cm. It was soft in consistency, non-tender with no ascites. A triphasic abdominal and pelvic contrast-enhanced computed tomography (CECT) demonstrated a large solid hypodense mass measuring 24×20×16 cm, with areas of necrosis and increased arterial blood supply (mostly direct branches of aorta) in the right adrenal topography. Per operatively a large adrenal mass (right) reaching up to the pelvis crossing midline, and touching but not invading the lower border of liver and inferior vena cava and encroaching right kidney was found and sent for histopathological examination. On two year follow up the patient had no recurrence and is doing well with a normal hormonal status
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