The Journal of Pediatric Academy

Yazarlar: ["Nihal Sahin", "Ugur Demirsoy", "Funda Corapcioglu"]

Konular:-

DOI:10.51271/jpea-2022-170

Anahtar Kelimeler:Children,Low grade glial tumors,Neurocutaneous syndromes,Neurofibromatosis

Özet: Neurofibromatosis type 1 (NF-1) is a significant autosomal dominant disorder with a wide spectrum of clinical findings. These signs (Café au lait spots, bone dysplasia, Lisch nodules) usually start to emerge after the first months of life and most are benign in nature. On the other hand, neoplasms (optic glioma, neurofibroma, malignant peripheral nerve sheath tumor, soft tissue sarcoma, leukemia, breast cancer) are a major cause of morbidity and mortality in NF-1 patients. Cancer risk during the lifetime of an NF-1 patient is almost 10 times more than a person without NF-1, but what drives these patients into cancer is still unknown.

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