malignant peripheral nerve sheath tumour

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malignant peripheral nerve sheath tumour

A poorly understood malignancy, presumably of Schwann cell origin, which is thought to arise from the neural crest. It is the most common malignancy of peripheral nerves, and represents 5 to 10% of all soft-tissue tumours. It occurs in the lower extremities in relatively young patients (age 20 to 50). Half of cases occur in patients with neurofibromatosis type 1, in which the male:female ratio is 4:1; 2/3 of MPNSTs arise from neurofibromas, 1/3 arise de novo. Origin from pre-existing schwannomas, ganglioneuromas or phaeochromocytomas are rare; 10% arise in a background of therapeutic radiation.
 
Management
Wide local excision; radiation and chemotherapy have been used with varying degrees of failure.
References in periodicals archive ?
All 14 clinically and surgically proven malignant lesions (including the MPNST) were diagnosed as such by FNACBP, thus, giving a sensitivity, specificity, and positive predictive value of 100% for malignancy.
Most of MPNST are high grade and have highly aggressive in its clinical course.
Gelderblom et al., "First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: An EORTC Soft Tissue and Bone Sarcoma Group study," Annals of Oncology, vol.
They are a major source of morbidity [10], causing disfigurement, impairment of nerve function, pain, and in some cases transform to MPNST (Figure 1) [2,3].
To date, complete surgical resection with wide negative margins is the only potentially curative treatment option for localized MPNST. Radiation treatment at high doses has been recommended for tumors > 5 cm and in the presence of microscopic positive margins [17].
Imaging features of MPNST in other locations like pelvis have been well summarized by some authors (10) and include diffuse or ring enhancement of MPNST as against central enhancement of their benign counterparts.
The differential diagnosis of cardiac SS includes carcinosarcoma, malignant mesothelioma, leiomyosarcoma, hemangiopericytoma, and MPNST. Unlike these malignant tumors, SS usually has a low-grade histologic appearance, with little or no cytologic atypia and cellular pleomorphism.
MPNST is a rare sarcoma with an incidence of 0.001% of the general population (1).
The differential diagnosis of desmoplastic melanoma includes scar, cutaneous FH, MPNST, and sarcomas.
MPNST is misdiagnosed as PA in the presence of eosinophilic stromal material with spindle cells, which is mistaken for chondromyxoid stroma and myoepithelial cells.
Malignant peripheral nerve sheath tumors (MPNST) are a group of neurogenic tumors that may be sporadic or coexist with neurofibromatosis.
The most common cause of early death in NF-1 patients is malignant peripheral nerve sheath tumors (MPNST) which most often occur in preexisting PNFs.