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Related Experiment Video

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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

Published on: May 17, 2024

Malignant peripheral nerve cell tumour.

M E Sham1, Ghorpade, Akshya Shetty

  • 1Dept. of Oral and Maxillofacial Surgery, Vydehi Institute of Medical and Dental Sciences, Bangalore, India ; No. 37, 1st Cross, Silver Oak Street, JP Nagar, 7th phase, Bangalore-78, India.

Journal of Maxillofacial and Oral Surgery
|November 10, 2012
PubMed
Summary

Malignant Peripheral Nerve Sheath Tumour (MPNST) is a rare soft tissue sarcoma. This report details an unusual intraosseous MPNST in the mandible, discussing its surgical and treatment strategies.

Keywords:
Malignant triton tumoursNerve sheath tumourNeurilemmomaNeurofibromatosis-ISarcoma/bone neoplasms

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Orthotopic Xenografting of Human Luciferase-Tagged Malignant Peripheral Nerve Sheath Tumor Cells for in vivo Testing of Candidate Therapeutic Agents

Published on: March 7, 2011

Area of Science:

  • Oncology
  • Surgical Pathology
  • Oral & Maxillofacial Surgery

Background:

  • Malignant Peripheral Nerve Sheath Tumour (MPNST) comprises 10% of soft tissue sarcomas, typically affecting lower extremities.
  • MPNST in the head and neck region is rare (10-20%), with central intraosseous involvement of the jaw bones being highly unusual.
  • Neurofibromas are common nerve sheath tumors, often associated with Neurofibromatosis I (NF-I).

Purpose of the Study:

  • To report a rare case of intraosseous Malignant Peripheral Nerve Sheath Tumour (MPNST) originating in the mandible.
  • To discuss the comprehensive surgical management approach for this rare intraosseous tumor.
  • To outline potential adjuvant and neoadjuvant treatment strategies for intraosseous MPNST.

Main Methods:

  • Case report detailing a patient with an intraosseous peripheral nerve sheath tumor.
  • Review of diagnostic findings and imaging studies.
  • Discussion of surgical resection techniques and reconstruction.
  • Consideration of adjuvant and neoadjuvant therapeutic options.

Main Results:

  • Successful surgical management of an intraosseous MPNST in the mandible.
  • Identification of the tumor's origin and characteristics.
  • Discussion of the challenges and outcomes associated with treating rare intraosseous sarcomas.

Conclusions:

  • Intraosseous MPNST of the mandible is an exceptionally rare presentation requiring specialized surgical and oncological management.
  • Multidisciplinary treatment planning is crucial for optimizing outcomes in such rare cases.
  • Further research into neoadjuvant and adjuvant therapies may improve prognosis for intraosseous MPNST.