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

Downloads

Download data is not yet available.
Abstract
113
PDF
65

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,

Downloads

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

Vol. 13 No. 2 (2012)

Section

Case Reports

License

Authors who publish with this journal agree to the following terms:

    1. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.

    1. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.

  1. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).