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Related Concept Videos

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Related Experiment Video

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Malignant peripheral nerve sheath tumor: A rare malignancy.

Sheetal Sharma1, Jigna S Shah2, Harleen Bali3

  • 1Department of OMR, AIIMS, New Delhi, India.

Journal of Oral and Maxillofacial Pathology : JOMFP
|March 20, 2020
PubMed
Summary

Malignant peripheral nerve sheath tumor (MPNST), a rare and aggressive sarcoma, can be challenging to diagnose due to varied presentations. Immunohistochemistry aids in identifying this spindle cell malignancy of the peripheral nerve Schwann cell.

Keywords:
Malignant nerve sheath tumorS-100 proteinneurofibromatosisperipheral nerve originrare malignancy

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Area of Science:

  • Oncology
  • Pathology
  • Surgical Oncology

Background:

  • Malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive soft-tissue sarcoma.
  • It originates from the Schwann cell of the peripheral nerve and accounts for 10% of all sarcomas.
  • MPNST can arise de novo or from a pre-existing neurofibroma.

Observation:

  • MPNST is infrequently found in the head-and-neck region (10%-12% of cases).
  • Diagnosis is often elusive due to significant clinical and histological variability.
  • This article details a rare case of oral cavity MPNST in a 40-year-old female.

Findings:

  • Immunohistochemistry is crucial for overcoming diagnostic challenges in MPNST.
  • The case highlights the importance of considering MPNST in the differential diagnosis of oral lesions.

Implications:

  • Improved diagnostic strategies for MPNST are needed.
  • Early and accurate diagnosis can potentially improve patient outcomes.
  • Further research into the management of rare MPNST presentations is warranted.