BACKGROUND: Type 1 neurofibromatosis patients develop malignant neoplasm mo
re frecuently than general population.
PATIENTS AND METHOD: We have studied 65 patients with neurofibromatosis typ
e 1 because we intend to know the kind of neoplasms that they have presente
d.
RESULTS: 65 patients (48 men and 17 women) fulfilling type 1 neurofibromato
sis criteria have been studied. Globally 67 tumors appeared, from which 47
(70%) were benign and 20 (30%) were malignant. Benign tumors included 25 ne
urofibromas, 11 central nervous system tumors and 11 extraneurological tumo
rs. 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 neurofibromatos
is and constitutes main death and morbility cause.