PRIMARY HEMANGIOSARCOMA, A RARE FORM OF AN INCIDENTAL ADRENAL MASS

A 50-year-old man with sudden onset of nausea, vomiting and diarrhoea was on ultrasonography of the upper abdomen found to have an adrenal tumour, 5.5 cm in diameter. This was excised transperitoneally with its connective-tissue pseudocapsule, there being no enlarged regional lymphnodes or other pathological findings. But the histological diagnosis was haemangiosarcoma of the adrenal. Seven months later the patient was re-admitted because of suspected acute cholecystitis. Emergency laparotomy revealed a gangrenous, stone-free gallbladder without abscess. Further exploration was not possible because of inflammatory changes and marked adhesions. Recurrent vomiting postoperatively indicated endoscopic retrograde cholangiopancreatography and computed tomography, which showed a space-occupying lesion in the right adrenal region with compression of the choledochal duct, lesser gastric curvature and duodenum. At re-laparotomy tumour recurrence was found with histologically proven intraluminal metastases of the haemangiosarcoma in the jejunum. As curative resection was not possible, a palliative gastrojejunostomy was performed. The patient died 9 months after the diagnosis had been made from the advanced malignancy. - This case of a rapidly fatal very rare malignant adrenal tumour underlines the need to remove all hormone-active asymptomatic adrenal tumours 4 cm in diameter or larger.