Resumo

ORRACA CASTILLO, Miladys  e  LICOURT OTERO, Deysi. Characteristics of plexiform neurofibroma in patients suffering from Type-1 Neurofibromatosis. Pinar del Rio. Rev Ciencias Médicas [online]. 2012, vol.16, n.4, pp. 25-35. ISSN 1561-3194.

Plexiform neurofibroma is a complex tumor involving several tissues, this tumor provokes a massive distortion of the region where it is located leading to esthetical and medical problems. This work was aimed at knowing the frequency of plexiform neurofibromas in patients suffering from Type-1 neurofibromatosis, location, prevalence of sex and other clinical-genetic characteristics to design a protocol of diagnosis and treatment of plexiform neurofibromas in Pinar del Rio province. A descriptive, cross-sectional study was carried out in patients suffering from plexiform neurofibroma. Out of the total of patients, 34 presented plexiform neurofibroma, female sex prevailed. The majority of plexiform neurofibromas were located in limbs, torso and skull-face. The hyperpigmentation and the hypertrichosis were the most frequent clinical signs. A protocol was designed to accomplish the diagnosis and treatment of the patients affected.

Palavras-chave : Plexiform neurofibroma [diagnosis]; Plexiform neurofibroma [genetics]; Plexiform neurofibroma [complications]; Neurofibromatoses [genetics]; Neurofibromatoses [classification]; Neurofibromatoses [diagnosis].

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