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[Tumors diagnosed in 65 patients with type I neurofibromatosis].

BACKGROUND
Type 1 neurofibromatosis patients develop malignant neoplasm more frequently than general population.


PATIENTS AND METHOD
We have studied 65 patients with neurofibromatosis type 1 because we intend to know the kind of neoplasms that they have presented.


RESULTS
65 patients (48 men and 17 women) fulfilling type 1 neurofibromatosis criteria have been studied. Globally 67 tumors appeared, from which 47 (70%) were benign and 20 (30%) were malignant. Benign tumors included 25 neurofibromas, 11 central nervous system tumors and 11 extraneurological tumors. Malignant tumors accounted for 6 soft tissue sarcomas, 6 carcinomas, 3 malignant CNS tumors and 2 acute lymphoblastic leukemias.


CONCLUSION
Presentation of neoplasms is frequent in type 1 neurofibromatosis and constitutes main death and morbility cause.