DOI: http://dx.doi.org/10.18203/issn.2454-5929.ijohns20175649

A rare case of Ewing’s sarcoma in the sinonasal tract

Deepthi Satish, Nandakumar R., Balasubramanya A. M, Nitty Mathew

Abstract


Ewing's sarcoma (ES) first described by James Ewing in 1921, is a primary neoplasm of the skeletal system. Extraskeletal Ewing’s sarcoma (EES) is a rare, rapidly growing, round cell malignant tumour that can develop in the soft tissue at any location. Extraskeletal Ewings sarcoma of the head and neck is uncommon. Nearly 80% of patients are younger than 20 years with peak incidence in the second decade. Ewings sarcoma rarely affects the sinonasal tract. Diagnosis is after histopathological examination immunohistochemical studies and cytogenetic studies. Treatment will include a multidisciplinary approach with surgery as the first line followed by chemotherapy and radiotherapy. Ewings sarcoma in the head and neck region do not metastazise early hence carries a better prognosis. Evaluation of lesion using imaging, biopsy followed by histopathology and immunohistochemistry and cytogenetic analysis are necessary for early diagnosis and treatment. We present a case of an 18-year-old male patient with left sided epistaxis and left sided nasal obstruction, on examination a mass was seen the nasal cavity, endoscopic excision and biopsy of the mass was suggestive of an Ewings sarcoma. He received post-operative chemotherapy and radiotherapy. 2 years on regular follow up patient is fine without signs of metastasis or recurrence. We are presenting this case due to the rarity of its presentation in the sinonasal tract.


Keywords


Ewings sarcoma, Extraskeletal Ewing’s sarcoma, Ewings family tumours

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References


Yeshvanth SK, Ninan K, Bhandary SK, Lakshinarayana KPH, Shetty JK, Makannavar JH. Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Cancer Res Therap. 2012;8(10):142-4.

Gupta S, Gupta OP, Mehrotra S, Mehrotra D. Ewing sarcoma of maxilla: A rare presentation. Quintessence Int. 2009;40:13.

Cotterill SJ, Ahrens S, Paulussen M, Ju¨rgens HF, Vouˆte PA, Gadner H, et al. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma study group. J Clin Oncol. 2000;18(17):3108–14.

Wenig BM, Dulguerov P, Kapadia SP, Prasad ML, Fanburgsmith JC Thompson LD. Neuroectodermal tumors. In: Barnes EL, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization classification of tumours. Pathology and genetics of head and neck tumours Lyon. France: IARC Press; 2005: 65–70.

Wenig BM. Undifferentiated malignant neoplasms of the sinonasal tract. Arch Pathol Lab Med. 2009;133(5):699–712.

Suzuki T, Yasumatsu R, Nakashima T et al. Primary Ewing's Sarcoma of the Sinonasal Tract: A Case Report, Case Rep Oncol. 2017;10(1):91-7.

Hafezi S, Seethala RR, Stelow EB, Mills SE, Leong IT, MacDuff E, et al. Ewing's family of tumors of the sinonasal tract and maxillary bone. Head Neck Pathol. 2011;5:8-16.

Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, et al. Ewing’s sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol. 1997;15(2):574–82.