Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and ...

The identification of cancer drivers is a major goal of current cancer research. Finding driver genes within large chromosomal events is especially challenging because such alterations encompass many genes. Previously, we demonstrated that zebrafish malignant peripheral nerve sheath tumors (MPNSTs) are highly aneuploid, much like human tumors. In this study, we examined 147 zebrafish MPNSTs by ...

Although the head and neck region is recognized as the most common location for peripheral nerve sheath tumors, central involvement, particularly in the jaw bones, is quite unusual. Neurofibroma is one of the most common nerve sheath tumors occurring in the soft tissue and generally appears in neurofibromatosis 1 (NF1 or von Recklinghausen's disease). Malignant peripheral nerve sheath tumors (M...

Ganglioneuroma is a benign tumor, affecting the central nervous system, commonly seen in children and young adults. Non-cranial ganglioneuromas are seen predominantly in the posterior mediastinum and retroperitoneum. Paratesticular neoplasms are extremely rare and are susceptible to clinical misinterpretation as malignancy. Tumors of nerve origin are a rarity in the paratesticul...

[This corrects the article DOI: 10.1371/journal.pone.0178064.].

OBJECTIVE To assess prevalence of plexiform neurofibroma in children and adolescents with type I neurofibromatosis and its malignant potential. METHODS A retrospective study was conducted through analysis of the database at Centro Nacional de Neurofibromatose [Brazilian Neurofibromatosis Center], collected from the following reference services between 1996 and 2004: Instituto de Dermatologia ...

The original version of this Article contained an error in the title where: " KANK1 inhibits cell growth by inducing apoptosis though regulating CXXC5 in human malignant peripheral nerve sheath tumors ". Now reads: " KANK1 inhibits cell growth by inducing apoptosis through regulating CXXC5 in human malignant peripheral nerve sheath tumors ". There were also typographical errors in the legends o...

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...

schwannoma is a solitary, slow growing,  benign tumour of the peripheral  nerve sheath, and it is most common locations are the head, neck, the flexor surfaces of the extremities, retroperitoneal and posterior mediastinium. external female genital organs are the least common location site of the schwannoma. only a few cases of vulvar schwannoma have been reported.we report 65 year- old woman pr...