Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.Keywords: neurofibromatosis, neurofibromatosis type 1, neurofibromatosis type 2, schwannomatosis, Legius syndrome. resumo A primeira parte desta diretriz abordou o diagnóstico diferencial das neurofibromatoses (NF): neurofibromatose do tipo 1 (NF1), neurofibromatose do tipo 2 (NF2) e schwannomatose (SCH). As NF compartilham algumas características, como a origem neural dos tumores e sinais cutâneos, e afetam cerca de 80 mil brasileiros. O aumento do conhecimento científico sobre as NF tem permitido melhor manejo clínico e redução da morbidade das complicações, resultando em melhor qualidade de vida para os pacientes com NF. A maioria dos médicos é capaz de realizar o diagnóstico das NF, mas a variedade de manifestações clínicas e a dificuldade de se prever o surgimento e a gravidade de complicações, torna o manejo da NF um desafio para o clínico e envolve diferentes especialistas para o tratamento adequado e aconselhamento genético, especialmente a NF2 e a SCH. O presente texto sugere algumas orientações para o acompanhamento dos portadores de NF, com ênfase na NF1.Palavras-chave: neurofibromatoses, neurofibromatose 1, neurofibromatose 2, schwannomatose, síndrome de Legius. 532Arq Neuropsiquiatr 2015;73(6): [531][532][533][534][535][536][537][538][539][540][541][542][543] Part 1 of this guideline addressed differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH) 1 . This second part aims to offer some practical suggestions on the clinical management of NF.NF shares some features, such as the genetic origin of the neural tumors, cutaneous manifestations, heterogeneous phenotype and unpredictable and usually progressive natural course. However, they differ in age of onset, progression of the symptoms and prognosis. Moreover, NF clinical presentation and severity vary among patients and even between twins carrying the same NF gene mutation 2 . The wide range of NF clinical manifestations and the difficulties to predict the onset or the severity of new features, consequences, o...

-Lesions located in the bilateral posterior incisural space are difficult to treat due to limited e x p o s u re. The classical approaches to this area are limited for lesions located bilaterally and especially when the lesion extends also below the tentorium as it may occur with meningiomas. Kawashima et al. re p o rted, in anatomic studies, a new occipital transtentorial approach: the occipital bi-transtentorial/falcine approach, to treat such lesions. We present a patient with a large falcotentorial meningioma, located bilaterally in the posterior incisural space. The occipital bi-transtentorial/falcine approach allowed an excellent surgical exposure and complete tumor removal with an excellent patient outcome.KEY WORDS: occipital bi-transtentorial/falcine approach, posterior incisural space, falcotentorial meningioma.Acesso occipital bitranstentorial-falcino para abordagem de meningioma falco-tentorial: relato de caso RESUMO -Grandes lesões que ocupam bilateralmente o espaço incisural posterior são de difícil abord a g e m c i r ú rgica pelos acessos clássicos. Recentemente, Kawashima et al. descreveram, em peças anatômicas, uma modificação do acesso occipital transtentorial, o acesso occipital bitranstentorial-falcino, para abord a g e m de grandes lesões que ocupam bilateralmente o espaço incisural posterior. Retata-se um caso de grande meningioma falco-tentorial que ocupava o espaço incisural posterior bilateralmente. O acesso occipital bitranstentorial-falcino permitiu exérese completa da lesão sem déficit no pós-operatório. PALAVRAS-CHAVE: acesso occipital bitranstentorial-falcino, espaço incisural posterior, meningioma falcotentorial.The posterior incisural space is located posterior to the midbrain, in the pineal body region 1 . Lesions located in this region include pineal tumors, meningiomas, gliomas, medial occipital lobe and superior c e rebellar arteriovenous malformations. The infratentorial supracerebellar approach and the occipital t r a n stentorial appro a c h 2 -5 a re the main approaches used to reach lesions located in this area. However, these a p p roaches do not allow a good exposure of lesions located bilaterally in the posterior incisural space 1 . Using anatomical specimens, Kawashima et al. 1 describe the occipital bi-transtentorial/falcine approach that provides better exposure of the bilateral posterior incisural space. It is a modification of the occipital transtentorial approach with the division of the falx and the tentorium bilaterally.We re p o rt a case of a large falcotentorial meningioma located bilaterally in the posterior incisural space, in which it was used the occipital bi-transtentorial/falcine approach. CASEOn November 2002, a 58 year-old woman was admitted to our neuro s u rgical unit with headache, torpor, and papilledema. The CT scan revealed a large mass compre s sing the midbrain and causing hydrocephalus. A ventriculoperitoneal shunt was placed on an emergency basis.The patient re c o v e red fully after the shunt, and an MRI (Fig 1) showed a large h...

Primary malignant melanoma of the meninges is an exceedingly rare neoplasm. Usually its symptoms include raised intracraneal pressure resulting from hydrocephalus seconday to tumoral obliteration of cisternal basal cisterns, but the passage of time from initial symptomalogy to diagnosis is frequently delayed. A 12-year-old male with primary letomeningeal melanoma is reported. At the beginning, he presented with vomiting, headache, complex seizures, fever four months before the admission in the hospital where progressive loss of consciouness after admission. Lumbar puncture reveled high protein level, normal glucose level and 50 leucocytes/mm3 with 86% polymorphic cells. Magnetic ressonance imaging of the brain was referred and in T1-weighted images revealed a diffuse enhancement of the leptomeninges on right frontal cortical. An open brain biopsy trough was performed, after exclusion of the infectious nervous system disease. Histological examination revealed massive infiltration of meninges with brown tumor cells. These cells stained positive for HMB-45, S-100 protein and vimentin. The patient received post operative radiation therapy, but died after three months of the diagnosis with septic shock and epileptical status

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