Abstract
Objective: The objective of this systematic review was to state and analyze all the case reports published on extraskeletal extradural Ewing’s Sarcoma to date.
 Material and Methods: We searched PubMed, MEDLINE, EMBASE, and Google Scholar using the following search term, including Boolean operators AND and OR. We only included published case reports and series about Ewing's Sarcoma that were Extra-skeletal and Extradural. We excluded the intradural EWS cases and those that did not have both components, i.e. extraskeletal and extradural. Conference articles, commentaries, and unpublished articles were also not included. A PRISMA flowchart was also formed.
 Results: After the first case of EES in 1969, around 40 case reports and series have been reported, which sheds light on the rarity of this disease. Age of presentation varies from as early as two months, with the oldest report being 49 years of age, and most cases presenting in adolescents and young adults. Spinal Epidural extraskeletal Ewing's sarcoma has been more frequently reported in males. The level of occurrence of the tumor also varies among cases, with cervical, thoracic and lumbar regions being more frequently affected than sacral regions.
 Conclusion: Extraskeletal extradural ES is an exceedingly rare anomaly. Multicentric collaborative work is the need of the hour for such orphan illnesses, to optimize treatment paradigms as a single-center is exceedingly unlikely to amass cases.
Highlights
Primary spinal extradural Ewing's sarcoma (ES), known as primitive neuroectodermal tumor (PNET), is a rare, heterogeneous group of malignant tumors of childhood and early adulthood
Spinal Epidural extraskeletal Ewing's sarcoma has been more frequently reported in males
The level of occurrence of the tumor varies among cases, with cervical, thoracic and lumbar regions being more frequently affected than sacral regions
Summary
Primary spinal extradural Ewing's sarcoma (ES), known as primitive neuroectodermal tumor (PNET), is a rare, heterogeneous group of malignant tumors of childhood and early adulthood. According to The World Health Organization (WHO) classification, PNET is an undifferentiated round cell tumor that arises from primitive neuroepithelial cells. Depending on the presentation site, it can be a centrally located or peripheral PNET.[1] Spinal Epidural PNET/peripheral primitive neuroectodermal tumors (pPNET) have an incidence of < 1% of primary spinal tumors.[2] Extraosseous Ewing's sarcoma commonly presents in soft tissues and bones of the lower extremity, paravertebral, and retroperitoneal regions.[3] Tefft et al in 1969, were the first to describe the extraosseous form of Ewing's sarcoma, and called it ‘extraskeletal Ewing's sarcoma (EES).[4]
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