Riffaud Laurent, Vinchon Matthieu, Ragragui Omar, Delestret Isabelle, Ruchoux Marie-Madeleine, Dhellemmes Patrick
Department of Pediatric Neurosurgery, Salengro Hospital, 59037 Lille cedex, France.
Childs Nerv Syst. 2002 Feb;18(1-2):43-7. doi: 10.1007/s00381-001-0534-3. Epub 2001 Dec 21.
Neurofibromatosis type 1, or peripheral neurofibromatosis, is the most form of common phakomatosis. In some instances, it can be associated with tumors of the central nervous system.
We describe four cases of hemispheric cerebral glioma in children with NF1. Two of these tumors were pilocytic astrocytomas and two, ependymomas. We reviewed the literature on hemispheric cerebral gliomas in NF1 patients in an attempt to find characteristic features of and some explanations for these lesions.
We conclude that surgical resection of these tumors should be proposed whenever possible, with due consideration for their progressive nature and the uncertainties about malignancy. We advocate yearly clinical and neuroradiological follow-up over a long period in NF1 children, since they may develop additional CNS tumors during their lifetime.
1型神经纤维瘤病，或周围神经纤维瘤病，是最常见的错构瘤病形式。在某些情况下，它可能与中枢神经系统肿瘤有关。
我们描述了4例患有1型神经纤维瘤病儿童的大脑半球胶质瘤病例。其中2例肿瘤为毛细胞型星形细胞瘤，2例为室管膜瘤。我们回顾了关于1型神经纤维瘤病患者大脑半球胶质瘤的文献，试图找出这些病变的特征及一些解释。
我们得出结论，只要有可能，就应考虑对这些肿瘤进行手术切除，同时适当考虑其进展性和恶性程度的不确定性。我们主张对1型神经纤维瘤病儿童进行长期的年度临床和神经放射学随访，因为他们在一生中可能会发生其他中枢神经系统肿瘤。
