Malignant Peripheral Nerve Sheath Tumour in a Patient of Type 1 Neurofibromatosis

Authors

  • Amitava Sengupta NRS Medical College and Hospital, West Bengal
  • Ankan Bandyopadhyay NRS Medical College and Hospital, West Bengal
  • Debraj Jash NRS Medical College and Hospital West Bengal
  • Kaushik Saha NRS Medical College and Hospital,West Bengal

DOI:

https://doi.org/10.3329/jom.v13i2.12761

Keywords:

Malignant peripheral nerve sheath tumour, Neurofibromatosis type 1, Upper abdominal mass

Abstract

Malignant peripheral nerve sheath tumours (MPNST) constitute a rare type of soft tissue sarcoma which arises from peripheral nerves or Schwann cells, perineural cells or fibroblasts. MPNST is a rare neoplasm affecting 1 in 1,00,000 population. Here we report a case of a 36 year old male who presented to us with an upper abdominal swelling for last 3 months. At first ,a diagnosis of type 1 neurofibromatosis type 1(NF-1) was made. Contrast enhanced CT scan abdomen and thorax revealed presence of a complex, heterogenous mass in upper abdomen with extension towards left hemithorax. Histopathological examination done from true cut biopsy specimen of the mass was suggestive of malignant peripheral nerve sheath tumour. Positivity of tumour cells on immunohistochemistry to S-100 established the diagnosis of MPNST in type 1 neurofibromatosis. In conclusion, it was a rare case of MPNST in a patient of NF-1.

DOI: http://dx.doi.org/10.3329/jom.v13i2.12761

J Medicine 2012; 13 : 219-221

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Author Biographies

Amitava Sengupta, NRS Medical College and Hospital, West Bengal

Associate Professor, Department of Pulmonary Medicine

Ankan Bandyopadhyay, NRS Medical College and Hospital, West Bengal

RMO cum Clinical Tutor, Department of Pulmonary
Medicine

Debraj Jash, NRS Medical College and Hospital West Bengal

Post Graduate Trainee, Department of Pulmonary Medicine,

Kaushik Saha, NRS Medical College and Hospital,West Bengal

RMO cum Clinical Tutor, Department of Pulmonary
Medicine,

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Published

2012-11-26

How to Cite

Sengupta, A., Bandyopadhyay, A., Jash, D., & Saha, K. (2012). Malignant Peripheral Nerve Sheath Tumour in a Patient of Type 1 Neurofibromatosis. Journal of Medicine, 13(2), 219–221. https://doi.org/10.3329/jom.v13i2.12761

Issue

Section

Case Reports