Fulkerson Daniel H, Luerssen Thomas G, Hattab Eyas M, Kim Daniel L, Smith Jodi L
Department of Neurosurgery, Division of Pediatric Neurosurgery, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
Pediatr Neurosurg. 2008;44(6):480-5. doi: 10.1159/000180303. Epub 2008 Dec 10.
Juvenile xanthogranuloma is a benign, non-Langerhans-cell histiocytic infiltrate that typically presents as a solitary cutaneous lesion in childhood. There are reports of extracutanous involvement, including tumors in the central nervous system. A solitary, intraparenchymal tumor without skin manifestations is a rare event, with only 3 prior cases reported in the literature. Cerebral lesions have been associated with multifocal or systemic forms of the disease, with an occasionally fulminate clinical course. Considering the rarity of this tumor, it is unclear whether patients need adjuvant therapy after excision of a solitary intraparenchymal tumor. Previous reports suggested that complete excision of the lesion was curative; however long-term follow-up was not provided. This report illustrates a case of surgical excision of a solitary juvenile xanthogranuloma in an 8-year-old male with a 3-year follow-up period.
幼年性黄色肉芽肿是一种良性的、非朗格汉斯细胞组织细胞浸润性疾病，通常表现为儿童期的孤立性皮肤病变。有关于皮肤外受累的报道，包括中枢神经系统肿瘤。无皮肤表现的孤立性脑实质内肿瘤是一种罕见情况，文献中仅报道过3例先前病例。脑病变与该疾病的多灶性或系统性形式相关，临床病程偶尔呈暴发性。鉴于这种肿瘤的罕见性，尚不清楚孤立性脑实质内肿瘤切除术后患者是否需要辅助治疗。先前的报道表明病变完全切除可治愈；然而未提供长期随访情况。本报告阐述了一例8岁男性孤立性幼年性黄色肉芽肿手术切除并随访3年的病例。
