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Malignant peripheral nerve sheath tumour-A case report.

Senthilkumar A C1, S Sridharan2, B Mahendra1

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Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive cancers often linked to neurofibromatosis I. Early detection and surgical removal are key for successful treatment and disease-free survival in these challenging cases.

Keywords:
CaseMPNSTMalignantNervePeripheralPre-sacralReportSheathTumour

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Area of Science:

  • Oncology
  • Genetics
  • Surgical Pathology

Background:

  • Malignant peripheral nerve sheath tumors (MPNST) are aggressive sarcomas originating from peripheral nerve sheaths, frequently associated with neurofibromatosis I or prior radiation.
  • Diagnosis is aided by high-resolution PET scans, with surgical excision as the primary treatment.

Purpose of the Study:

  • To present a case report of a patient with Von Recklinghausen disease diagnosed with MPNST.
  • To highlight the importance of early diagnosis and multidisciplinary management for MPNST in this patient population.

Main Methods:

  • A case report detailing a 47-year-old female with Von Recklinghausen disease presenting with pelvic mass and thigh pain.
  • Clinical examination, radiological assessment, and intraoperative findings were utilized for diagnosis and treatment planning.
  • Surgical excision of the pelvic mass was performed.

Main Results:

  • Intraoperative findings correlated with clinical and radiological assessments, confirming the presence of a pelvic mass.
  • The patient underwent successful surgical excision and is currently disease-free with regular follow-up.

Conclusions:

  • MPNSTs are rare tumors strongly associated with neurofibromatosis I, driven by genetic mutations.
  • Prompt diagnosis, radical surgery, and adjuvant chemoradiation are crucial for achieving near-complete cure.
  • Malignancy, specifically MPNST, should be highly suspected in Von Recklinghausen patients presenting with similar symptoms.