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A rare occurrence of adrenal leiomyosarcoma

 

Aditi Sharma, Elisa Lewington-Gower, Ana Pokrajac

 

Leiomyosarcomas are rare tumours that can arise from smooth muscle cells anywhere in the body; common sites include uterus, GI tract and retroperitoneum. We report an unusual case of a patient with an adrenal incidentaloma confirmed as a leiomyosarcoma on histology.


A 61-year-old Caucasian female was investigated for chronic abdominal pain. CT scan of the abdomen and pelvis did not show any pathology other than an incidental 2.3cm right adrenal nodule. She underwent CT and MRI of the adrenals to further characterise the lesion. These were reported as ‘indeterminate’ but likely a benign adrenal incidentaloma. Patient was then referred to our Endocrinology department whereby further testing confirmed a non-secretory tumour. A follow up CT adrenal scan at 6 months showed an increase in the size of the tumour to 3cm, of heterogeneous density with delayed washout of contrast with a low-enhancing centre, requiring an urgent referral for surgery. Although the pre-operative investigations suggested an adrenal tumour, at surgery the tumour was even larger and found to be invading the IVC and was clearly malignant.  A planned laparoscopic procedure was changed to an open procedure and a grade 2 right per-adrenal leiomyosarcoma was resected with resection of the lateral wall of the IVC. Interestingly, the attached adrenal gland was normal. Patient is currently followed up in a dedicated leiomyosarcoma centre.

 

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 multi-disciplinary team