FUNCTIONAL PRIMARY EWING SARCOMA OF THE ADRENAL GLAND – A CASE REPORT shreyasurendra@gmail.com

 

FUNCTIONAL PRIMARY EWING SARCOMA OF THE ADRENAL GLAND – A CASE REPORT

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India

Poster Exhibition only

Ewing sarcoma is a rare malignant neoplasm of neuroectodermal origin affecting the bone or soft tissue in children and young adults. A minority (10-20%) can present in extra skeletal locations. It has a characteristic translocation t(11;22) on chromosome 22. There have been 66 cases of primary Ewing sarcoma of the adrenal gland published in literature with only one reported functional tumour. Here we share our experience of functional primary adrenal Ewing Sarcoma in a 26-year old gentleman that mimicked adrenal cortical carcinoma in its presentation

26 year old gentleman presented with abdominal pain with mass. Clinical diagnosis was Adrenal cortical carcinoma with cortisol production. Histopathology surprised with adrenal Ewings sarcoma with classic translocation seen on FISH.

Following surgery for adrenal Ewings sarcoma he underwent chemotherapy and radiotherapy. One year post surgery follow up PET CT shows a doubtful recurrent lesion in the adrenal bed but as he is asymptomatic and doing well, he has been kept on follow up.

Primary adrenal Ewing sarcoma is extremely rare malignancy. Preoperative distinction from ACC is difficult. Histopathology is the gold standard for diagnosis while molecular translocation defines the tumour. To the best of our knowledge this is the second case to be reported of a functional primary adrenal Ewing’s sarcoma.