Zahir Muhammad Nauman, Ansari Tayyaba Zehra, Moatter Tariq, Memon Wasim, Pervez Shahid
Department of Oncology, Aga Khan University Hospital, Stadium Road, PO BOX: 3500, Karachi 74800, Pakistan.
BMC Res Notes. 2013 Dec 13;6:533. doi: 10.1186/1756-0500-6-533.
Ewing's sarcoma uncommonly arises from extraosseous soft tissue or parenchymal organs. Primary adrenal Ewing's Sarcoma, although very rare, is extremely aggressive and commonly fatal.
A 17 year old Pakistani male was referred to the outpatient oncology clinic at our center with a three month history of concomitant pain, swelling and dragging sensation in the right hypochondrium. Abdominal examination revealed a large, firm mass in the right hypochondrium extending into the right lumbar region and epigastrium. His genital exam was unremarkable and there were no stigmata of hepatic or adrenal disease.Computed tomography scans revealed a large peripherally enhancing mass in the hepatorenal area, biopsy of which showed a neoplastic lesion composed of small round blue cells which exhibited abundance of glycogen and stained diffusely positive for CD99 (MIC2 antigen). Fluorescence in situ hybridization demonstrated gene rearrangement at chromosome 22q12 which confirmed the diagnosis of Ewing's sarcoma. Staging scans revealed pulmonary metastasis and hence he was commenced on systemic chemotherapy.
This case report highlights the importance of keeping Ewing's sarcoma in mind when a young patient presents with a large non-functional adrenal mass.
尤因肉瘤很少起源于骨外软组织或实质器官。原发性肾上腺尤因肉瘤虽然极为罕见,但极具侵袭性,通常会致命。
一名17岁的巴基斯坦男性被转诊至我们中心的门诊肿瘤科,有三个月右季肋部疼痛、肿胀及牵拉感的病史。腹部检查发现右季肋部有一个大的、质地坚硬的肿块,延伸至右腰部和上腹部。他的生殖器检查无异常,也没有肝脏或肾上腺疾病的体征。计算机断层扫描显示肝肾区域有一个周边强化的大肿块,对其进行活检显示肿瘤性病变由小圆形蓝色细胞组成,这些细胞含有大量糖原,CD99(MIC2抗原)弥漫性染色呈阳性。荧光原位杂交显示22q12染色体发生基因重排,这证实了尤因肉瘤的诊断。分期扫描显示有肺转移,因此他开始接受全身化疗。
本病例报告强调了年轻患者出现大型无功能肾上腺肿块时,考虑尤因肉瘤的重要性。