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
Highlights
Malignant peripheral nerve sheath tumours (MPNST) are sarcoma which arises from peripheral nerves or from cells associated with nerve sheath
(2) Axillary or inguinal freckles,Two or more neurofibromas of any type or one plexiform neurofibroma, (3) Sphenoid wing dysplasia or congenital bowing of long bone cortex with or without pseudoarthrosis, (4) Bilateral optic nerve glioma, two or more Lisch nodules on slit lamp examination, (5) First degree relative with Neurofibromatosis type 1 (NF1) diagnosed by preceding criteria
(1) It arises from a peripheral nerve, (2) It arises from a pre-existing benign nerve sheath tumour, (3) It demonstrates Schwann cell differentiation on histological examination
Summary
Introduction: Malignant peripheral nerve sheath tumours (MPNST) are sarcoma which arises from peripheral nerves or from cells associated with nerve sheath. Sarcoma arising from peripheral nerve or neurofibroma is considered to be a MPNST. The incidence of malignant peripheral nerve sheath tumour (MPNST) in general population is 0.001%.
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