Benignan and malignant tumours in patients with neurofibromatosis type 1

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.