Endocrine Abstracts

Adrenal lymphangiomatous cysts are rare, often found incidentally, but can also present in association with abdominal/loin pain or hypertension.¹ They have occasionally been reported in association with hormone over secretion of either aldosterone, cortisol or catecholamines. We present a case of a 40-year-old serving soldier who was suddenly awoken with severe left sided abdominal and flank pain. Over the course of several months, he was forced to take time off work, required opiate analgesia and experienced significant psychological burden because of the severity of symptoms. His initial work up involved a cystoscopy (due to dipstick haematuria) which was normal and a CT of the abdomen which showed a left sided 3.5 cm cystic, partially calcified adrenal lesion. Full endocrinological workup revealed the lesion to be non-functioning. He was normotensive. The size of the adrenal cyst was not thought to correlate with the severity of his pain (and a follow up CT showed no growth or intracyst haemorrhage), but in the absence of an alternative explanation, the patient chose to proceed to a laparoscopic left adrenalectomy. His pain immediately resolved postoperatively, he discontinued all analgesia and returned to work. Histology showed multiloculated cyst lined by flattened endothelial-like cells. Immunohistochemistry confirmed expression of D2-40, CD31 and CD34 consistent with cystic adrenal lymphangioma. Whilst most small adrenal cysts are asymptomatic, adrenal cystic lymphangiomas have been associated with back/abdominal/loin pain in 48% and hypertension in 14% cases.^1,2 This suggests that these endothelial cysts are more likely to cause symptoms regardless of their size. The possible mechanisms of pain in relatively small cysts with no evidence of prior bleed could include retroperitoneal irritation, local cytokine release, abnormal lymphatic drainage, but what exactly causes this phenomenon, especially if pain is severe, remains a mystery. Management of the adrenal cyst and decision on whether to proceed to surgery remains dependent on the size and clinical imperative. However, this case provides an example of small adrenal cyst having life changing implications, had it not been removed. Recognition that small adrenal cystic lymphangiomas can cause such symptoms may help inform future clinical decision making.

References: 1. Adrenal Gland Lymphangiomas. Indian J Surg. 2015 Dec;77(3):1334–1342

2. Cystic lymphangiomatous lesions of the adrenal gland: A clinicopathological study of 37 cases including previously unreported cysts with papillary endothelial proliferation. Pathol Res Pract. 2019 Jun;215(6):152385