Intraosseous Nerve Sheath Tumors in the Jaws

Abstract

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 (MPNSTs) are uncommon sarcomas that almost always arise in the soft tissue. Here, we report four cases of intraosseous peripheral nerve sheath tumors occurring in the jaw bones and compare the clinical, radiologic, and pathologic findings in order to make a differential diagnosis.

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.…”

“…Prognostic variables for MPNST are the size of lesion, location, stage and grade, status of margins, necrosis and use of adjuvant radiation. Among these, the status of surgical margin and history of irradiation are independent negative prognostic factors 1 5. Overall survival rate is 40–70% 4…”

Malignant peripheral nerve cell tumour

“…Given its origin and behavior it is classified as a sarcoma [1,2]. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type I is 8% to 13% [2].…”

Solitary Intraosseous Neurofibroma of the Frontal Bone -Case Report-

“…19) Ten cases of intraosseous neurofibroma showed evidence of NF. 5,7,9,13,15,24,26) However, the present case did not satisfy the criteria for NF, and was regarded as the solitary type of neurofibroma.…”