Skull and Spinal Ewing's Sarcoma in Children: An Institutional Study

Ewing's sarcoma is a disease of children and young adults and occurs most often in bone and soft tissues. The intracranial and spinal manifestation of the disease is rare and reported incidence is 1%-6%.

We conducted this study to determine the surgical outcome of children with skull and spine Ewing's sarcoma (SSES).

This is a prospective analysis of 13 patients of SSES who reported to the Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India, between 2014 and 2016. All cases after detailed examination, magnetic resonance imaging, and computed tomography scan were subjected to surgery followed by adjuvant therapy comprising chemotherapy and radiotherapy. Outcome was analyzed at 6 months as well at latest follow-up. Neurological function, local recurrence, primary or secondary nature of the disease, distant relapse, and treatment-related complications were analyzed in this study.

There were eight female and five male patients with a mean age of 12 years (ranging from 4 to 8 years). Pain was the common presenting feature in all cases. Focal neurological deficits corresponding to the anatomical location was seen in six patients. These 13 cases were distributed anatomically as four cases involving the cloves, two cases with occipital bone and lobe involvement, one case of parietal bone and lobe involvement, and six cases of spinal involvement. Surgery was performed in all cases where gross total excision (Ozge C, Calikoglu M, Cinel L, Apaydin FD, Ozgür ES. Massive pleural effusion in an 18-year-old girl with Ewing sarcoma. Can Respir J 2004;11:363-5), near-total excision, and subtotal excision was achieved in these 13 cases (Steinbok P, Flodmark O, Norman MG, Chan KW, Fryer CJ. Primary Ewing's sarcoma of the base of the skull. Neurosurgery 1986;19:104-7). Subsequently all cases underwent multiagent chemoradiotherapy. Postsurgery pain subsided in 12 (92%) of patients. Ten patients maintained or improved motor function. In seven cranial cases and in six spinal cases, four cases showed improvement whereas three (23%) had deterioration of motor function.

Surgical outcome of SSES in short-term follow-up is good with current recommended management regimen of maximum excision followed by chemo and radiotherapy. However, metastasis is not uncommon.