A rare occurence of adrenal leiomyosarcoma

Abstract

❖ Mesenchymal tumours like leiomyosarcoma are associated with non-islet cell tumour-induced hypoglycaemia caused by the unregulated production of IGF-II and extensive glucose metabolism.❖ This case highlights the importance of appropriate radiological assessment in adrenal incidentalomas by experienced adrenal radiologists.❖ Monitoring of patients with suspicious looking lesions even if not meeting the initial criteria for surgery should be rigorous and discussed in a dedicated multidisciplinary team.A Rare Occurrence of Adrenal Leiomyosarcoma She reported pain and nausea triggered by sweets.•CT scan of the abdomen and pelvis did not show any pathology other than an incidental 2.3cm right adrenal nodule. She was then referred to our endocrine team.•She underwent CT and MRI of the adrenals to further characterise the lesion. These were reported as 'indeterminate' but likely a benign adrenal incidentaloma.•Further testing confirmed a non-secretory tumour (24 hour urine metanephrines [3 samples]: negative; normal aldosterone/renin ratio, ODST: 71 nmol/L, normal LDDST)• A follow up CT adrenal scan at 6 months showed an increase in the size of the adrenal nodule to 3cm.• It was of heterogeneous density with delayed washout of contrast with a low-enhancing centre.• The increase in size of the tumour within 6 months and the characteristics seen on repeat CT adrenal scan were suspicious of malignancy and she was referred urgently to the tertiary centre for a surgical opinion.• As the pre-operative investigations suggested an adrenal tumour, she was booked for a retroperitoneoscopic/laparoscopic right adrenalectomy.• However at surgery the tumour was even larger and found to be invading the IVC and clearly malignant.• A planned laparoscopic procedure was changed to an open procedure and a grade 2 right peri-adrenal leiomyosarcoma was resected with resection of the lateral wall of the IVC. • Interestingly, the attached adrenal gland was normal.• Her sweet intolerance resolved after surgery.• She is currently followed up in a dedicated leiomyosarcoma centre.