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Malignant Peripheral Nerve Sheath Tumor: Treat or Not Treat?

Muhamad Fairuz Bin Samsuddin1, Mohamad Azhari Bin Omar1

  • 1Department of Neurosurgery, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia.

Asian Journal of Neurosurgery
|April 3, 2019
PubMed

Insights

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas with poor prognosis. This case highlights typical MPNST presentation and challenges in treatment.

Area of Science:

  • Oncology
  • Pathology
  • Neurosurgery

Background:

  • Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas originating from nerve cells.
  • These tumors present significant therapeutic challenges due to their aggressive nature and poor prognosis.

Observation:

  • A 23-year-old female presented with a month-long right temporal swelling and worsening constitutional symptoms.
  • Initial biopsy was suggestive of MPNST, confirmed by histopathological examination.

Findings:

  • Advanced imaging revealed widespread metastatic lesions in the spine, retroperitoneum, pelvis, chest wall, and lungs.
  • The case exemplifies the typical, aggressive presentation and metastatic potential of MPNST.

Implications:

  • This case underscores the difficulties in managing MPNSTs, emphasizing their aggressive behavior and resistance to conventional therapies.
  • It highlights the importance of early diagnosis and comprehensive staging for patients with MPNST.
Keywords:
Malignant peripheral nerve sheath tumorsneurofibromatosis type 1prognosissurvival

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