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peripheral nerve sheath tumors

Monday 9 November 2009

Benign (BPNST) and malignant (MPNST) peripheral nerve sheath tumours occur either sporadically or are related to neurofibromatosis (NF).

The mechanisms involved are well known in NF-related tumours, but still remain unclear in sporadic cases.

Types

- peripheral nerve sheath tumors

Neurofibromas are sporadic or associated with type 1 neurofibromatosis (NF1), with a higher risk of malignant progression.

Molecular biology

Somatic BRAF and KRAS mutations represent the most frequent genetic events in melanocytic neoplastic lesions.

BRAF and KRAS mutations influence BPNST and MPNST development. RAS pathway activation due to BRAF V600E and KRAS mutations is an important event in a subset of peripheral nerve sheath tumours not related to NF.

Immunochemistry

- CD10 is useful in the assessment of peripheral sheath tumors and could give evidence that atypical myxoid and/or diffuse neurofibromas, sometimes histologically difficult to distinguish from low-grade MPNST, represent not only a histological but also an immunohistochemical continuum with MPNST. (19661328)

References

- BRAF V600E and KRAS G12S mutations in peripheral nerve sheath tumours. Serrano C, Simonetti S, Hernández-Losa J, Valverde C, Carrato C, Bagué S, Orellana R, Somoza R, Moliné T, Carles J, Huguet P, Romagosa C, Ramón Y Cajal S. Histopathology. 2012 Aug 31. PMID: 23190154

- Diagnostic and prognostic value of CD10 in peripheral nerve sheath tumors. Cabibi D, Zerilli M, Caradonna G, Schillaci L, Belmonte B, Rodolico V. Anticancer Res. 2009 Aug;29(8):3149-55. PMID: 19661328