Malignant peripheral nerve cell tumour

Abstract

Malignant Peripheral Nerve Sheath Tumour (MPNST) also termed as spindle cell malignancy of the peripheral nerve schwann cell or neurogenic sarcoma represents 10% of all soft tissue sarcomas. The tumour is usually found in lower extremities and only 10% to 20% of all lesions occur in head and neck region thus making it a rare entity. Central involvement, particularly in the jaw bones is quite unusual. Neurofibroma is one of the common nerve sheath tumours occurring in the soft tissues and generally appears in neurofibromatosis I (NF-I or von recklinghausen's disease). MPNST are uncommon sarcomas that almost always arise in the soft tissues. Here we report a case of intraosseous peripheral nerve sheath tumour occurring in the mandible and discuss the surgical management with adjuvant and neoadjuvant treatment plan.

Malignant peripheral nerve sheath tumour (MPNST) of mandible: solving the perplexity.

“…Approximately 50–90% of MPNSTs are positive for S-100 protein. Occasionally, they may also express glial fibrillary acidic protein, EMA and CK expression 1 2 4 5. In the present case, histopathological features of the lesion, which included fasciculated growth pattern with alternate hypodense and hyperdense areas, and spindle cells with minimal cytological atypia, were seen infiltrating into the surrounding hard and soft tissue structures.…”

“…They mainly affect the 20–50-year-old age group and have an equal gender distribution. Radiographic presentation of intraosseous MPNST includes a complete destructive pattern with bony expansion, erosion and widening of mandibular canal/mental foramen with or without irregular destruction of surrounding bone 4 5 7. In the present case, the 30-year-old patient presented a solitary intraosseous MPNST as a well-defined radiolucent lesion in the body and ramus of the mandible.…”

Tumor maligno de la vaina nerviosa periférica mandibular. Caso clínico y revisión de la literatura.

“…El TMVNP es una neoplasia muy infrecuente, con una incidencia estimada de 0,001 % en la población general [2][3][4] . Representa el 10 % de todos los sarcomas de tejidos blandos, de los cuales únicamente entre el 8 y el 16 % se localizan en la región anatómica de cabeza y cuello.…”

Tumores malignos de la vaina nerviosa periférica como origen de dolor orofacial

“…El neurinoma es inmunorreactivo a la proteína S-100 (PS-100), Leu-7, calcineurina, componentes de la lámina basal y vimentina. La PS-100 puede aparecer en tumores derivados de las vainas de Schwann (schwannoma, neurofibroma y neurinoma) y de los melanocitos (nevus y melanoma) 11 . En particular, un radio elevado de PS-100␣ a PS-100␤ favorece la malignidad sobre una etiología benigna.…”