Benesch Martin, Eder Hans-Georg, Sovinz Petra, Raith Johann, Lackner Herwig, Moser Andrea, Urban Christian
Division of Pediatric Hematology and Oncology, Department of Pediatrics, Medical University of Graz, Graz, Austria.
Pediatr Neurosurg. 2006;42(3):159-64. doi: 10.1159/000091859.
The aim of this study was to report on children with cerebellar low-grade glioma (LGG), who were found to have progressive or nonprogresssive residual tumors or tumor recurrence after tumor resection.
Medical records and magnetic resonance imaging (MRI) studies of children (<16 years) with cerebellar LGG were retrospectively analyzed.
Of 289 patients with CNS tumors referred between 1983 and 2003, 28 (9.7%) (15 male, 13 female; median age at diagnosis: 71 months) had cerebellar LGG (pilocytic astrocytoma grade I: n = 21; fibrillary astrocytoma grade II: n = 5; mixed hamartoma/pilocytic astrocytoma: n = 1; radiographic diagnosis: n = 1). Total resection was initially performed in 16 patients (57.1%), near total resection in 4 (14.3%), and partial resection in 6 patients (21.4%). One patient underwent biopsy. At a median follow-up of 112 months, 25 patients (89.3%) were alive, 18 of them being in complete remission. Three patients died, 2 due to symptoms related to brain stem compression/infiltration and 1 patient due to postoperative cerebral edema. Presently 5 patients have nonprogressive residual tumors and 2 patients developed nonprogressive recurrences 10 years and 20 months after initial total resection, respectively. None of them required second surgery and none received additional nonsurgical therapies. Only 1 additional patient had to undergo second surgery due to disease progression.
A 'wait and see' strategy is justified in patients with nonprogressive recurrent or residual cerebellar LGG after primary tumor resection. However, long-term follow-up with repeated MRI is mandatory in these patients to detect disease progression. Second surgery is indicated only in patients with unequivocal disease progression, as documented by MRI.
本研究旨在报告小脑低级别胶质瘤(LGG)患儿的情况,这些患儿在肿瘤切除后被发现存在肿瘤进展、非进展性残留肿瘤或肿瘤复发。
对年龄小于16岁的小脑LGG患儿的病历和磁共振成像(MRI)研究进行回顾性分析。
在1983年至2003年间转诊的289例中枢神经系统肿瘤患者中,28例(9.7%)(男性15例,女性13例;诊断时的中位年龄:71个月)患有小脑LGG(毛细胞型星形细胞瘤I级:n = 21;纤维型星形细胞瘤II级:n = 5;混合性错构瘤/毛细胞型星形细胞瘤:n = 1;影像学诊断:n = 1)。16例患者(57.1%)最初进行了全切,4例(14.3%)进行了近全切,6例患者(21.4%)进行了部分切除。1例患者接受了活检。中位随访112个月时,25例患者(89.3%)存活,其中18例完全缓解。3例患者死亡,2例死于与脑干压迫/浸润相关的症状,1例死于术后脑水肿。目前,5例患者有非进展性残留肿瘤,2例患者分别在初次全切后10年和20个月出现非进展性复发。他们均无需二次手术,也未接受额外的非手术治疗。仅1例额外患者因疾病进展不得不接受二次手术。
对于原发性肿瘤切除后出现非进展性复发或残留小脑LGG的患者,“观察等待”策略是合理的。然而,这些患者必须进行长期的MRI随访以检测疾病进展。仅在MRI证实有明确疾病进展的患者中才考虑二次手术。