Hanbali Fadi, Tabrizi Peyman, Lang Frederick F, DeMonte Franco
Department of Neurosurgery, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
J Neurosurg. 2004 Feb;100(2 Suppl Pediatrics):169-78. doi: 10.3171/ped.2004.100.2.0169.
Published data obtained in children with tumors of the skull base are sparse. In the majority of the available reports, the authors focus on the technical application of skull base approaches, but they contribute a paucity of information on the management of specific tumors, especially malignant skull base lesions. The purposes of this report are to increase the collective experience with the treatment of these tumors and to identify successful management paradigms.
The authors retrospectively reviewed the clinical records, pathological reports, and diagnostic images obtained in 24 children (< or = 19 years of age) with tumors arising from the cranial base in whom resection was part of their management between 1992 and 2002. Surgery-related complications and outcomes were analyzed with regard to tumor type and surgical approach. The median age of the group was 14 years. Tumors involved the anterior skull base in eight (33%), the middle skull base in 10 (42%), both the anterior and middle skull base in four (17%), and the posterior skull base in two patients (8%). Benign lesions were discovered in 11 patients (46%) and malignant neoplasms in 13 (54%). The tumors were most commonly of mesenchymal origin (21 [87.5%] of 24 tumors). Thirty surgical procedures were performed using a number of skull base approaches. A gross-total resection was achieved in 23 procedures (77%) and a subtotal resection in five (17%); a biopsy procedure was performed in one case; and the disease process could not be accessed in one case. One patient died in the perioperative period. Minor complications (Karnofsky Performance Scale score > or = 90, no prolongation of hospital stay, and no further surgery needed) occurred following 10 (33%) of the 30 surgical procedures. These affected 10 (42%) of the 24 patients and resulted in persistent or prolonged deficits in only five patients (21%). In nine patients (38%) the tumor recurred after a mean duration of 23 months.
Skull base tumors in children affect mainly the anterior and middle cranial fossa. Sarcomas account for the majority of malignant tumors. Treatment of skull base tumors in children and adolescents needs to be tailored to patient age, tumor location, and tumor type.
关于儿童颅底肿瘤的已发表数据稀少。在大多数现有报告中，作者专注于颅底手术入路的技术应用，但他们提供的关于特定肿瘤管理的信息匮乏，尤其是恶性颅底病变。本报告的目的是增加这些肿瘤治疗的集体经验并确定成功的管理模式。
作者回顾性分析了1992年至2002年间接受手术切除治疗的24例（年龄≤19岁）颅底肿瘤患儿的临床记录、病理报告和诊断影像。分析了与手术相关的并发症及结局与肿瘤类型和手术入路的关系。该组患儿的中位年龄为14岁。肿瘤累及前颅底8例（33%），中颅底10例（42%），前颅底和中颅底均累及4例（17%），后颅底2例（8%）。11例（46%）发现为良性病变，13例（54%）为恶性肿瘤。肿瘤最常见起源于间叶组织（24例肿瘤中的21例[87.5%]）。采用多种颅底手术入路进行了30次手术。23次手术（77%）实现了全切，5次手术（17%）为次全切；1例进行了活检；1例无法进行手术。1例患者在围手术期死亡。30次手术中有10次（33%）出现轻微并发症（卡氏功能状态评分≥90，住院时间未延长，无需进一步手术）。这些并发症影响了24例患者中的10例（42%），仅5例患者（21%）出现持续或长期功能缺损。9例患者（38%）肿瘤复发，平均复发时间为23个月。
儿童颅底肿瘤主要累及前颅窝和中颅窝。肉瘤占恶性肿瘤的大多数。儿童和青少年颅底肿瘤的治疗需要根据患者年龄、肿瘤位置和肿瘤类型进行个体化。
