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.
Division of Oral Pathology, College of Dental Medicine, Medical University of South Carolina, Charleston.
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BW
Neville
J
J
Hann
R
R
Narang
P
P
Garen
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