A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors.
Article
in English
| IMSEAR
| ID: sea-165433
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 (Manipal University), 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. Malefemale ratio was 2.61. 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.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Language:
English
Year:
2014
Type:
Article
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