Oral neurofibrosarcoma associated with neurofibromatosis type I.
One of the most feared complications of neurofibromatosis type I (NF-I) is development of cancer, which is estimated to occur in about 5% of cases. The most common associated malignancy is the neurofibrosarcoma (NFS). HOwever, oral NFS in association with NF-I has rarely been reported. We report two cases of oral NFS arising in patients with NF-I. Both patients died of their tumors. Oral NFS arising in association with NF-I appears to have an extremely poor prognosis, as do these tumors at other sites of the body.
Neville
B W
BW
Division of Oral Pathology, College of Dental Medicine, Medical University of South Carolina, Charleston.
Hann
J
J
Narang
R
R
Garen
P
P
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008339:Mandibular Neoplasms / Q000209:etiology* / Q000473:pathology; D008441:Maxillary Neoplasms / Q000209:etiology* / Q000473:pathology; D009455:Neurofibroma / Q000209:etiology* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications*; D011379:Prognosis; D016896:Treatment Outcome