Endocrine Abstracts

Introduction: Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells. They are benign endocrinologically inactive tumours with a histological structure consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. Commonly, angiomyolipomas occur in the kidney with few extra renal case reports in adrenals.

Case Report: We report the case of a 76-year-old-man with personal history of hypertension and hypercholesterolemia, who presents as an incidental finding on ultrasound performed by urology due to an episode of acute urine retention, a 91 mm right adrenal mass, which on scan is suggestive of adenoma. The hormonal study (cortisoluria, metanephrines and aldosterone) was negative for functionality and, DHEA sulfate revealed normal results. Due to the large size, the patient underwent an open right adrenalectomy and was discharged home just over 1 week post-operatively without incidences. Histology showed a soft tissue tumour composed of mature adipose tissue, proliferating thick-walled vessels and in a smaller quantity proliferation of smooth muscle, with focal spindle and epithelioid cells Immunostains for Melan-A (Fig. 4) and HMB-45 were focally positive. Desmin and smooth muscle actin were also focally positive, confirming the presence of smooth muscle in the tumour, also there were present cystic areas occupied by devitalized tissue and fibrino-hematic material. These histological features are consistent with an spontaneous adrenal hematoma of an angiomyolipoma.

Discussion: This case report describes spontaneous haemorrhage of an adrenal angiomyolipoma picked up incidentally on imaging. A review of the literature revealed 18 previously reported cases of adrenal angiomyolipoma. Of these, 15 were sporadic and 3 were reported in patients with tuberous sclerosis or lymphangioleiomyomatosis]. There were 2 reported cases of spontaneous haemorrhage. Management of these lesions has been guided by the limited number of case reports in the literature. It is general consensus that smaller lesions detected incidentally should be managed non-surgically and their size should be kept under surveillance. Surgical management has been proposed for lesions which are either symptomatic and/or greater than 5 cm. Laparoscopic adrenalectomy has successfully been used, but larger lesions are best removed using open adrenalectomy

Conclusion: Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here we report the 3er case reported on literature of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.