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Primary Spinal Extradural Extraosseous Primitive Neuroectodermal Tumor/Ewing's Sarcoma: A Critical Analysis and
Akshay Shrirang Patil1, Prasheelkumar Premnarayan Gupta2, Sandeep Wasudeorao Iratwar1
1Department of Neurourgery, JNMC and AVBRH, Wardha, Maharashtra, India.
Asian Journal of Neurosurgery
|July 16, 2021
Summary
Primary spinal Ewing's sarcoma/primitive neuroectodermal tumor (PNET) is a rare, aggressive childhood cancer. Early detection and treatment are crucial, especially in rural populations, due to poor prognosis and limited management guidelines.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Cancer Research
Background:
- Spinal extradural Ewing's sarcoma/primitive neuroectodermal tumor (PNET) is a rare malignant neoplasm.
- WHO classification defines PNET as an undifferentiated round cell tumor of primitive neuroepithelial origin.
- Spinal PNET presents with symptoms like chronic back pain and myelopathy, often with a dismal prognosis.
Purpose of the Study:
- To report on cases of spinal PNET at a rural Indian hospital.
- To highlight challenges in diagnosis and management.
- To emphasize the need for standardized treatment guidelines.
Main Methods:
- Retrospective review of medical records at Acharaya Vinoba Bhave Rural Hospital.
- Identification and analysis of four patients diagnosed with spinal PNET.
- Evaluation of patient demographics, tumor characteristics, and presentation delays.
Main Results:
- Four patients with spinal PNET were identified, aged 15-26 years (mean 20).
- All tumors were epidural; two were Askin type with spinal cord compression.
- Late presentation was attributed to rural factors like low literacy and financial constraints.
Conclusions:
- Spinal PNET is a rare and aggressive tumor with poor outcomes.
- Rural populations face significant barriers to early diagnosis and treatment.
- Multicentric trials are essential to establish management guidelines for spinal PNET.
