The Association of Malignancies with The Clinical Profile of Children with Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF-1) is a significant autosomal dominant disorder with wide spectrum of clinical findings. These signs (Café au lait spots, bone dysplasia, Lisch nodules) usually start to emerge after the first months life and most are benign in nature. On other hand, neoplasms (optic glioma, neurofibroma, malignant peripheral nerve sheath tumor, soft tissue sarcoma, leukemia, breast cancer) major cause morbidity mortality NF-1 patients. Cancer risk during lifetime an patient almost 10 times more than person without NF-1, but what drives these patients into cancer still unknown.
 This study aims analyze possible association findings malignancies children NF-1.
 Medical records 55 who were followed up tertiary care pediatric oncology clinic between January 2005 December 2014 analyzed. We assessed demographic characteristics patients, as well diagnostic criteria, related complications, malignancies. The malignancy classified Group while 2. Logistic regression analysis was used determine factors mean age 7.68 ± 4.65 years. Female sex both groups. Café spots present all Axillary-inguinal freckling observed 76.4% by neurofibromas 30.9%, nodules 29.1%, dysplasia 14.5%, optic gliomas 23.6%, history degree relative 63.6%. Central nervous system (CNS) tumors 40%. Tumors CNS acute myeloid leukemia schwannoma. None criteria factor for predisposing itself. Having >3 found be (OR:5.891, CI 95%: 1.676-20.705, p=0.006).
 There no clearly defined predicting occurance at present. However, we higher meet 3 NF-1.