Ewing’s sarcoma arising from the adrenal gland in a young male: a case report

BMC Research Notes

Shahid Pervez¹  Wasim Memon²  Tariq Moatter¹  Tayyaba Zehra Ansari³  Muhammad Nauman Zahir³ 

[1] Department of Pathology and Microbiology, Aga Khan University, Stadium Road, PO BOX: 3500, Karachi 74800, Pakistan;Department of Radiology, Aga Khan University Hospital, Stadium Road, PO BOX: 3500, Karachi 74800, Pakistan;Department of Oncology, Aga Khan University Hospital, Stadium Road, PO BOX: 3500, Karachi 74800, Pakistan

关键词: CD99;    Adrenal;    Ewing’s sarcoma;   

Others  :  1140494
DOI  :  10.1186/1756-0500-6-533

 received in 2013-06-13, accepted in 2013-12-10,  发布年份 2013

【 摘 要 】

Background

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.

Case presentation

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.

Conclusion

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.

【 授权许可】

   
2013 Zahir et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

Figure 3.

【 参考文献 】

• [1]de Alava E, Gerald WL: Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 2000, 18(1):204-213.
• [2]Zagar TM, Triche TJ, Kinsella TJ: Extraosseous Ewing's sarcoma: 25 years later. J Clin Oncol 2008, 26(26):4230-4232.
• [3]Ahmed AA, Nava VE, Pham T, Taubenberger JK, Lichy JH, Sorbara L, et al.: Ewing sarcoma family of tumors in unusual sites: confirmation by rt-PCR. Pediatr Dev Pathol 2006, 9(6):488-495.
• [4]Gonin J, Larousserie F, Dousset B, Rousseau J, Delattre O, Waintrop C, et al.: An unusual adrenal tumor: Ewing tumor. Ann Pathol 2011, 31(1):28-31.
• [5]Kato K, Kato Y, Ijiri R, Misugi K, Nanba I, Nagai J, et al.: Ewing's sarcoma family of tumor arising in the adrenal gland–possible diagnostic pitfall in pediatric pathology: histologic, immunohistochemical, ultrastructural, and molecular study. Hum Pathol 2001, 32(9):1012-1016.
• [6]Matsuoka Y, Fujii Y, Akashi T, Gosehi N, Kihara K: Primitive neuroectodermal tumour of the adrenal gland. BJU Int 1999, 83(4):515-516.
• [7]Pirani JF, Woolums CS, Dishop MK, Herman JR: Primitive neuroectodermal tumor of the adrenal gland. J Urol 2000, 163(6):1855-1856.
• [8]Zhang Y, Li H: Primitive neuroectodermal tumors of adrenal gland. Jpn J Clin Oncol 2010, 40(8):800-804.
• [9]Kushner BH, Cheung NK: Neuroblastoma–from genetic profiles to clinical challenge. N Engl J Med 2005, 353(21):2215-2217.
• [10]Marina NM, Etcubanas E, Parham DM, Bowman LC, Green A: Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management. Cancer 1989, 64(9):1952-1960.
• [11]Balamuth NJ, Womer RB: Ewing's sarcoma. Lancet Oncol 2010, 11(2):184-192.