Prevalência de neurofibromas plexiformes em crianças e adolescentes com neurofibromatose tipo 1

Abstract

Objective: To assess prevalence of plexiform neurofibroma in children and adolescents with type I neurofibromatosis and its malignant potential. Results: Over that period, 104 patients aged between 1-17 years were admitted with clinical diagnosis of type I neurofibromatosis. Of these, 53 were male and 51 were female, and 28 patients (15 male and 13 female) had plexiform neurofibroma (26.9%). Division by age group resulted in 21.42% (six) between 1-5 years; 35.71% (10) between 6-12 years and 42.85% (12) between 13-17 years. Of the 104 patients, two developed a malignant peripheral nerve sheath tumor (1.92%). Methods Conclusions:Plexiform neurofibromas are relatively common manifestations in patients with type I neurofibromatosis and may be a cause of significant increase in morbidity and mortality among patients. In this study, we conclude that frequency of plexiform neurofibroma and its malignant potential in the population studied is in agreement with data from the international literature.J Pediatr (Rio J). 2007;83(6):571-573: Type I neurofibromatosis, plexiform neurofibroma, prevalence.

Pigmented (melanotic) diffuse neurofibroma of the back in neurofibromatosis type 1

“…While the typical cutaneous neurofibroma, which often occur in large numbers, characteristically is observed after puberty, plexiform neurofibroma (PNF) are thought to develop during the embryonic or perinatal period. According to recent findings, the frequency of PNF in NF1 is about 30% [ 5 ].…”

Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports

“…They represent one of the most frequent non-rhabdomyosarcomatous soft tissue tumors in pediatric age and usually occur in young adults from a previously anticipated plexiform neurofibroma in the context of NF1, with a noted change in size and pain [4]. At present there are only limited data based on anecdotal reports regarding the occurrence of MPNST in NF1 in children and adults [4,5]. Herein we report two cases of adult MPNST in NF1 and emphasize the need of a multidisciplinary approach in the treatment of these tumors.…”

Moyamoya syndrome associated with neurofibromatosis type 1 in a pediatric patient

“…However, when they occur isolatedly and idiopathically, the term adopted is moyamoya disease. 1 , 4 Other authors consider that the characteristic arteriographic changes are bilateral in patients with moyamoya disease, while they are unilateral in moyamoya syndrome. The term "moyamoya", when used alone, refers only to arteriographic changes, regardless of the cause.…”