Broekman Marike L D, Risselada Roelof, Engelen-Lee Jooyeon, Spliet Wim G M, Verweij Bon H
Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, 3508 GA Utrecht, The Netherlands.
Case Rep Med. 2009;2009:757898. doi: 10.1155/2009/757898. Epub 2009 Dec 16.
Patients with Neurofibromatosis type 1 (NF1) have an increased risk of developing neoplasms. The most common brain tumors, found in 15%-20% of NF1 patients, are hypothalamic-optic gliomas, followed by brainstem and cerebellar pilocytic astrocytomas. These tumors generally have a benign nature. NF1 patients are predisposed to a 5-fold increased incidence of high-grade astrocytomas, which are usually located in supratentorial regions of the brain. We present an NF1 patient who developed a high-grade astrocytoma in the posterior fossa and discuss possible pathophysiological mechanisms.
1型神经纤维瘤病(NF1)患者发生肿瘤的风险增加。在15%-20%的NF1患者中发现的最常见脑肿瘤是下丘脑-视神经胶质瘤,其次是脑干和小脑毛细胞型星形细胞瘤。这些肿瘤通常具有良性性质。NF1患者发生高级别星形细胞瘤的几率增加5倍,这些肿瘤通常位于脑幕上区域。我们报告1例在颅后窝发生高级别星形细胞瘤的NF1患者,并讨论可能的病理生理机制。