A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors

Siny Vellukara Sasidharan, Vinu Kumar, Radha R. Pai, Sheela Vasudevan

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.

 


Keywords


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

Full Text:

PDF

References


Suresh TN, Kumar H, Prasad CSBR, Kalyani R, Borappa K. Malignant peripheral nerve sheath tumor with divergent differentiation. Indian J Pathol Microbiol. 2009;52:74-6.

Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral nerve sheath tumor (MPNST). A clinicopathologic, immunohistochemical and ultrastructural study of seven cases. Am J Surg Pathol. 1998;22:1368-78.

Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors: a clinicopathological study of 120 cases. Cancer. 1986;57:2006-21.

Weiss SV, Enzinger FM. Malignant tumors of peripheral nerves. In: Weiss SV, Goldblum R, eds. Enzinger and Weiss’s Soft Tissue Tumors. 4th ed. St Louis: CV Mosby; 2001: 1209-1263.

Rekhi B, Ingle A, Kumar R, DeSouza MA, Dikshit R, Jambhekar NA. Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at tertiary cancer referral centre in Mumbai, India. Indian J Pathol Microbiol. 2010;53:611-8.

Kar M, Deo AV, Shukla NK, Malik A, Gupta SD, Mohanti BK, et al. Malignant peripheral nerve sheath tumors (MPNST) - clinicopathological study and treatment outcome of twenty-four cases. World J Surg Oncol. 2006;4:55.

Wanebo J, Malik J, Vandenberg S, Wanebo H, Driesen N, Persing J. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer. 1993;71:1247-53.

Gabhane SK, Kotwal MN, Bobhate SK. Morphologic spectrum of peripheral nerve sheath tumors: a series of 126 cases. Indian J Pathol Microbiol. 2009;52:29-33.

Hirose T, Hasegawa T, Kudo E, Seiki K, Sano T, Hisawa K. Malignant peripheral nerve sheath tumors: an immunohistochemical study in relation to ultrastructural features. Hum Pathol. 1992;23:865-70.

Theaker J, Gatter K, Puddle J. Epithelial membrane antigen expression by the perineurium of peripheral nerve and in peripheral nerve sheath tumors. Histopathology. 1988;13:171-9.

Smith TA, Machen SK, Fischer C, Goldblum JR. Usefulness of cytokeratin subsets for distinguishing monophasic synovial sarcoma from malignant peripheral nerve sheath tumor. Am J Clin Pathol. 1999;112:641-8.