Cranial and Spinal Malignant Peripheral Nerve Sheath Tumor: A Pathological Enigma

Area of Science:

• Neuro-oncology
• Radiology
• Surgical Oncology

Background:

• Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms originating from nerve sheaths.
• Limited literature exists on MPNST treatment strategies and clinical outcomes.
• Differentiating MPNSTs from benign variants requires improved diagnostic approaches.

Purpose of the Study:

• To review institutional cases of MPNST, focusing on radiological features for early differentiation.
• To analyze clinicoradiological findings, treatment parameters, and patient outcomes.
• To enhance understanding of MPNST diagnosis and prognosis.

Main Methods:

• Retrospective analysis of pathologically diagnosed MPNST cases (January 2007 - December 2018).
• Collection of clinicoradiological data and treatment parameters from medical records.
• Telephone follow-up for final clinical status assessment.

Main Results:

• Seven MPNST cases diagnosed: four intracranial, three spinal.
• Mean patient age was 34.3 years; five out of seven patients were female.
• Gross total resection achieved in 57.1%, subtotal resection in 28.6%; 57.1% overall survival at 28.2 months average follow-up.

Conclusions:

• MPNST is a rare tumor with a poor prognosis and high fatality rate.
• Radical surgical resection is the primary treatment but often limited by tumor location and size.
• Preoperative radiological findings may aid in diagnosis, though challenging.