Malignant peripheral nerve sheath tumors

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Malignant Peripheral Nerve Sheath Tumors (MPNST)

INTRODUCTION Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas which originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells, or fibroblasts. Because MPNSTs can arise from multiple cell types, the overall appearance can vary greatly from one case to the next. This can make diagnosis and classification somewhat difficu...

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Intrathoracic Malignant Peripheral Nerve Sheath Tumor: Histopathological and Immunohistochemical Features

Malignant peripheral nerve sheath tumor (MPNST) is a rare nerve sheath tumor derived from Schwann cells or pleuripotent cells of neural crest. Neurogenic tumors make about 10-20% of all mediastinal tumors. Incidence of MPNST is 0.001% in general population and 0.16% in patients with neurofibromatosis I (NF I). We report a case of 60 year female presenting with progressive cough and breathlessne...

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Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

Introduction. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. There is little reported in literature on these tumors in Africa. Materials and Methods. A search of the hospital pathology database between 1992 and 2008 reveale...

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A–Z of malignant peripheral nerve sheath tumors

This article reviews the typical and atypical locations, imaging findings, local recurrence, and metastatic pattern of malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are rare soft tissue sarcomas, commonly occur in extremities, and are often associated with neurofibromatosis. Distinction between benign and malignant tumors can be challenging on imaging. MPNSTs have a poor prognosis; ...

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Malignant peripheral nerve sheath tumors in neurofibromatosis 1.

One of the most clinically aggressive cancers associated with neurofibromatosis 1 (NF1) is the malignant peripheral nerve sheath tumor (MPNST). To determine the incidence and relative risk (RR) of MPNSTs in individuals with NF1, 1,475 individuals with NF1 were included from a cohort of patients examined by a single experienced geneticist from 1977 to 1996. The end points were incidence of MPNST...

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ژورنال

عنوان ژورنال: Cancer

سال: 2006

ISSN: 0008-543X,1097-0142

DOI: 10.1002/cncr.22098