A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors

Authors

  • Siny Vellukara Sasidharan Department of Pathology, Sree Gokulam Medical College and Research Foundation, Venjaramoodu, Trivandrum, Kerala
  • Vinu Kumar Department of Pathology, Sree Gokulam Medical College and Research Foundation, Venjaramoodu, Trivandrum, Kerala
  • Radha R. Pai Department of Pathology, Kasturba Medical College, Manipal University, Mangalore, Karnataka
  • Sheela Vasudevan Department of Pathology, Sree Gokulam Medical College and Research Foundation, Venjaramoodu, Trivandrum, Kerala

Keywords:

Malignant peripheral nerve sheath tumor, Neurofibromatosis (NF-1), Heterologous differentiation

Abstract

Background:Malignant Peripheral Nerve Sheath Tumor (MPNST) is a rare aggressive sarcoma that develops within a peripheral nerve and forms a diagnostic challenge in view of its varied histomorphology. This short series highlights the clinicopathological spectrum of 11 cases of MPNST and the incidence of neurofibromatosis 1 (NF1) association.

Methods:This retrospective and descriptive study on MPNST was done in the department of pathology, Kasturba medical college Mangalore (ManipalUniversity),India over a period of three years from January 2008 to December 2010. Cases which were histopathologically diagnosed as MPNST were reviewed & immunostains was done where ever indicated to rule out the differentials.

Results:A total of 11 cases of MPNST were documented with a wide age range of 17-85 years. Male:female ratio was 2.6:1. Extremities (63.64%) were found to be the most common site. Location wise most of the tumors were deep seated (63.64%) and maximum cases were high grade (54.55%). NF1 association was seen in 2 cases. Heterologous elements in the form of chondroid differentiation was seen in one case. Immunostain with S-100 was focally positive in all the cases.

Conclusion:MPNST is a highly aggressive sarcoma with poor prognosis characterized by a challenge in its diagnosis as it has several mimics. Its diagnosis necessitates the incorporation of clinicopathological features and IHC with S-100 protein.

 

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Published

2017-01-26

How to Cite

Sasidharan, S. V., Kumar, V., Pai, R. R., & Vasudevan, S. (2017). A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors. International Journal of Research in Medical Sciences, 2(4), 1307–1312. Retrieved from https://www.msjonline.org/index.php/ijrms/article/view/2413

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Original Research Articles