Title: Malignant Peripheral Nerve Sheath Tumor: A Rare Case and Review of Literature

Authors: Dr Shashirekha C A, Dr Arvind Ramachandran, Dr Ravikiran H R

 DOI:  https://dx.doi.org/10.18535/jmscr/v5i2.105

Abstract

Introduction

Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs the brachial and lumbar plexuses or from nerves as they arise from the trunk. MPNSTs comprise approximately 5-10% of all soft tissue sarcomas. They can occur either spon-taneously or in association with neurofibromatosis-1.

The etiology is unknown but there is a higher incidence in patients with a history of radiation exposure. Up to 50% of MPNSTs occur in patients with NF1, demonstrating the tendency for this tumor to arise from a preexisting neurofibroma. Cross sectional studies have previously demonstrated a 1-2% prevalence of MPNST among NF1 patients although a recent study showed these patients have a 10% lifetime risk of ultimately developing an MPNST.

References

1.      Hruban RH, Shiu MH, Senie RT, Woodruff JM: Malignant peripheral nerve sheath tumors of the buttock and lower extremity A study of 43 cases. Cancer 1990, 66:1253-1265.

2.      Angelov L, Guha A: Peripheral Nerve Tumors. In Neuro-oncology Essentials 1st edition. Edited by: Berstein M, Berger MS. New York Theme Publishers; 2000:434-444.

3.      Wanebo JE, Malik JM, VandenBerg SR, Wanebo JH, Driesen N, Persing JA: Malignant peripheral nerve sheath tumors. A clinico-pathologic study of 28 cases. Cancer 1993, 71:1247-1253.

4.      Dasgupta TK, Choudhuri PK: Tumors of soft tissue 2nd edition. Connecticut Appleton & Lange; 1998:127-395.

5.      Ducatman SB, Bernd WS, David GP, Herbert MR, Duane MI: Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986, 57:2006-2021.

Corresponding Author

Dr Arvind Ramachandran

Post Graduate, Department of General Surgery

SDUMC, Kolar