Abstract
Patients with neurofibromatosis type 1 develop both benign and malignant tumors at an increased frequency. Most of the malignant peripheral nerve sheath tumors (MPNSTs) are considered as high-grade sarcomas originating from tissues of mesenchymal origin. It is generally accepted that MPNSTs occur in about 2% to 5% of neurofibromatosis patients. In this paper, we present a 16-year-old male patient with neurofibromatosis who developed MPNST of the retromolar area. The mass enlarged rapidly in a period of 6 weeks. The patient was treated surgically, and a tumor mass with a diameter of 7 × 6 × 4 cm was excised, but after 8 months a recurrence was observed at the same site. The sarcomatous change in a neurofibroma has an extremely poor prognosis, so patients with neurofibromatosis should be closely monitored for a possible malignancy. A rapid change in size of a preexisting neurofibroma, infiltration of the adjacent structures, intralesional hemorrhage, and pain indicate a possible malignant transformation to MPNST.
Highlights
Neurofibromatosis type 1 (NF1), an autosomal dominant neurocutaneous disorder, is commonly identified by the development of cafe-au-lait pigmentation of the skin, axillary freckling, optic nerve gliomas, Lisch nodules, distinctive bone lesions, and cutaneous or subcutaneous tumors called neurofibromas [1]
We present a 16-year-old male patient with NF1 who developed Malignant peripheral nerve sheath tumors (MPNSTs) of the retromolar area
Oral manifestations are seen in approximately 72% to 92% of cases with NF1, usually presenting with an enlargement of the tongue and submucosal neurofibroma nodules [4]
Summary
Neurofibromatosis type 1 (NF1), an autosomal dominant neurocutaneous disorder, is commonly identified by the development of cafe-au-lait pigmentation of the skin, axillary freckling, optic nerve gliomas, Lisch nodules, distinctive bone lesions, and cutaneous or subcutaneous tumors called neurofibromas [1]. Patients with NF1 develop both benign and malignant tumors at an increased frequency [2]. Neurofibromas are the most common benign tumors in NF1 [1, 2]. These tumors are composed of an admixture of Schwann cells, perineural fibroblasts, and mast cells infiltrating the peripheral nerves [1, 2]. Malignant peripheral nerve sheath tumors (MPNSTs) may develop within an associated neurofibroma [2, 3]. We present a 16-year-old male patient with NF1 who developed MPNST of the retromolar area
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