Neville B W, Hann J, Narang R, Garen P
Division of Oral Pathology, College of Dental Medicine, Medical University of South Carolina, Charleston.
Oral Surg Oral Med Oral Pathol. 1991 Oct;72(4):456-61. doi: 10.1016/0030-4220(91)90560-y.
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.
