Malignant peripheral nerve sheath tumour in a 12 years girl: a case report

ABSTRACT

Malignant peripheral nerve sheath tumours [MPNSTs] are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. 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 jawbones is quite unusual. There is little reported in literature on these tumors in Africa. Here we report a rare case of intraosseous MPNSTS occurring in the mandible in a 12-years old girl. The biological behavior and diagnostic challenges of this rare malignancy are discussed