2012, Number 4
Characteristics of plexiform neurofibroma in patients suffering from Type-1 Neurofibromatosis. Pinar del Rio
Language: Spanish
References: 11
Page: 25-35
PDF size: 271.44 Kb.
ABSTRACT
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.REFERENCES
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Fisher MJ, Basu S, Dombi E, Yu JQ, Widemann BC, Pollock AN, et al. The role of [18F]-fluorodeoxyglucose positron emission tomography in predicting plexiform neurofibroma progression. J Neurooncol [Internet]. 2008 [citado 8 Jun 2012];87(2):[aprox. 7 p.]. Disponible en: http://dx.doi.org/10.1007/s11060-007-9501-5
Ferner RE, Golding JF, Smith M, Calonje E, Jan W, Sanjayanathan V, et al. [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol [Internet]. Feb 2008 [citado 8 Jun 2012];19(2):[aprox. 5 p.]. Disponible en: http://annonc.oxfordjournals.org/content/19/2/390.full.pdf+html
Lammert M, Friedman JM, Roth HJ, Friedrich RE, Kluwe L, Atkins D, et al. Vitamin D deficiency associated with number of neurofibromas in neurofibromatosis 1. J Med Genet [Internet]. Sep 2003 [citado 8 Jun 2012]; 43(10): [aprox. 5 p.]. Disponible en: http://jmg.highwire.org/content/43/10/810.full.pdf+html