Primary Ewing's Sarcoma affecting the Central Nervous System: A single-center experience and Narrative review

Area of Science:

• Neuro-oncology
• Pediatric oncology
• Skeletal system tumors

Background:

• Ewing sarcoma (EWS) is a rare malignant round-cell tumor affecting bone and soft tissue, primarily in children and young adults.
• Central nervous system (CNS) involvement by primary EWS is exceptionally rare, with an incidence of approximately one per million.
• Limited literature exists on CNS EWS, necessitating case series to understand its management and outcomes.

Purpose of the Study:

• To present the clinical experience of managing 21 cases of primary CNS EWS.
• To highlight the clinical and radiological findings, treatment strategies, and surgical outcomes.
• To evaluate the efficacy of current treatment protocols for this rare condition.

Main Methods:

• Retrospective collection of hospital records for surgically treated primary CNS EWS patients (2015-2023).
• Analysis of patient demographics, clinical presentation, radiological findings, and treatment details (surgery, adjuvant therapy).
• Evaluation of outcomes at discharge and latest follow-up.

Main Results:

• Sixteen males and five females, mean age 18.2 years. Common symptoms included headache/vomiting (cranial) and back pain (spinal).
• Cranial lesions in 13 patients (61.9%), spinal in 8 (38.1%). Tumor resection achieved in 18 patients (85.71%), with gross-total excision in 9 (42.86%).
• 15 patients received chemo-radiotherapy; 5 with poor KPS expired early. 9 patients remained asymptomatic at follow-up; metastasis occurred in some cases.

Conclusions:

• Primary CNS EWS is a rare entity requiring detailed radiological assessment for surgical planning.
• Timely diagnosis and radical surgical excision followed by adjuvant therapy are crucial for favorable outcomes.
• Adjuvant chemo-radiotherapy improves disease control, but regular monitoring for metastasis is essential.