Endocrine Abstracts

Adrenal/periadrenal sarcomas are very rare entity of adrenal tumours. An epidemiological analysis of primary adrenal cancers found prevalence of adrenal sarcoma of 1.3% compared to adrenocortical carcinoma (ACC) 43.8%, neuroblastoma 39.7%, pheochromocytoma 10.9% and non-Hodgkin’s lymphoma 4.3% (1). In 2021, 26 adrenalectomies in adults were performed in our institution. 12/26 had malignant histology. 9/26 lesions were > 6 cm. 7/9 lesions > 6 cm were malignant (2 adrenal sarcomas, 2 ACC, 2 infiltrative renal cancer, 1 metastatic melanoma). Adrenal sarcoma was detected in 2/26 adrenalectomized specimens. We present two cases of adrenal sarcoma detected over 12-month period in University Hospital Southampton, United Kingdom. Case 1: 35-year-old woman presented with right abdominal pain for over one year. Ultrasound showed right suprarenal mass. CT CAP and adrenal MRI confirmed large tumour 4.9×6.1×5.7 cm adjacent to or arising from right adrenal gland and indents IVC. No metastases. Urine metanephrines, aldosterone, renin, 1 mg ODST were normal. Working diagnosis was non-functioning ACC. Patient underwent open adrenalectomy and post-operatively her pain resolved. Histology revealed high grade leiomyosarcoma (pT2 N0 G3) adjacent to adrenal with narrow excision margin. Patient opted against adjuvant chemotherapy and remains under surveillance. Imaging 8 months post-op showed no evidence of recurrence. Case 2: 70-year-old man presented with two months history of left flank and back pain, weight loss, and elevated CRP (159 mg/l). CT detected suspicious 7.8 cm heterogenous left adrenal mass extending to splenic hilum. Metanephrines, lymphoma screen- normal, 1 mg ODST-raised (72 nmol/l). Patient underwent open adrenalectomy, splenectomy and wedge excision of the liver due to intraoperative findings of liver metastases. Histology confirmed high grade epithelioid angiosarcoma. Restaging CT post-op showed lung, liver metastases and local recurrence in resection bed. He commenced palliative paclitaxel chemotherapy, with good radiological response. Adrenal sarcomas should be considered in any age in differential diagnosis of indeterminate adrenal masses especially when > 6 cm, associated with ipsilateral pain and raised CRP. 1 mg ODST can be raised due to raised metabolic rate associated with high-grade malignancy. The duration of symptoms could range from few weeks to 12 months. In our second case with metastatic disease, surgical debulking of primary tumour provided significant pain relief and increased patient’s quality of live and prognosis. Both patients are alive at the time of writing.

Reference: (1) Chandrasekar T et al. The who, when, and why of primary adrenal malignancies: Insights into the epidemiology of a rare clinical entity. Cancer. 2019 Apr 1;125(7):1050–1059