Primary Intradural Extramedullary Ewing Sarcoma of spine: Systematic Review of Literature

Abstract

Introduction

The incidence of extraosseous Ewing sarcoma, a highly malignant mesenchymal tumor, is rare in the spinal cord

and its clinical outcomes unknown. To date, few cases of primary intradural extramedullary Ewing sarcoma

(PIEES) have been reported in the literature, with few follow-ups.

Material and Methods

Here, we aimed to perform a comprehensive review of all cases published in the literature and update previously

reported cases with our single institution case. Institutional medical records were searched for cases of PIEES of

the spine managed at our institution between the years 2017 and 2023. We performed a systematic search of

two electronic databases (PubMed and Medline) from inception to December 2023 to obtain all published cases

of PIEES. We used our institutional medical records to update cases reported from our institution.

Results

We identified a total of 40 cases with PIEES reported in the existing literature. Of the 41 cases, the median age

of diagnosis was 31 years. The most common presentation pattern was PIEES in the lumbar/sacral region (61%,

n = 27), with a majority (59%, n = 25) presenting initially with pain. The most common modality of treatment

reported was surgery (41/41, 100%), followed by adjuvant chemotherapy (31/41, 75%) and local radiation therapy

(29/41, 70%). Overall, recurrence was reported in 17/41 (34%) cases, with median progression free survival (PFS)

of 12 months (range, 1–72). There were 12/37 (29.4%) deaths reported, with median overall survival (OS) of 14

months (range, 1–72).

Conclusion

We presented the most updated review of all reported cases of PIEES. While surgical resection is the mainstay

of treatment, tumor recurrence is a great concern given the adhesive nature of the lesion preventing complete

resection. Adjuvant chemotherapy and radiotherapy should be carefully considered to prevent recurrence and

improve survival outcome.