Abstract

Introduction: The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor). The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases.EWS is the second most common primary bone malignancy in childhood. In contrast to long bone involvement, delays in spinal EWS diagnosis may occur because symptoms may not be present until neurological deficits occur. To date, although there have been reported cases of EWS in the lumbosacral region, the reported cases are very less and the presentations might drastically differ. Case Presentation: A 14 year old boy comes with a 12 weeks h/o urinary incontinence and constipation. Over the next 4 months, the patient had new-onset intermittent abdominal pain with worsening urinary symptoms.MRI confirmed a diagnosis of Koch's spine. However, the histopathology reported it as an “Ewings sarcoma/Primitive Neuroectodermal Tumor”. Discussion: Ewing’s tumor of sacrum is rare, but should be suspected in low backache in children. Cauda equina syndrome can be a valid presentation for EWS spine. MRI can identify cases early and enables early treatment though it is not specific. Histopathological diagnosis is a must before any definitive management.EWS spine can mimick Tuberculosis both clinico-radiologically, only to be confirmed with a tissue biopsy. Conclusion: Ewing’s sarcoma of the lumbosacral spine can have an atypical presentation and there should be a high degree of suspicion to diagnose it early.

Highlights

  • The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor)

  • Tumors belonging to the Ewing family are devastating malignancies that appear to be more common than it has previously been reported [9]

  • Cauda equina syndrome can be a valid presentation for EWS spine

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Summary

Introduction

Ewing’s sarcoma was first described by James Ewing in 1921. It is a poorly differentiated tumor of uncertain histogenesis and variable biologic behavior. It is more common in Caucasians and rarely arises in individuals of African and Asian races. EWS is the second most common primary bone malignancy in childhood and adolescence with peak age from 15 to 17 years and a slight male predominance [8,9]. There have been reported cases of EWS in the lumbosacral region, the reported cases are very less and the presentations might drastically differ. Present this case report with a dual diagnostic atypicality of EWS clinico-radiologically and basic principles to follow

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