Varan Ali, Akalan Nejat, Söylemezoğlu Figen, Zorlu Faruk, Yalçin Bilgehan, Akyüz Canan, Kutluk Tezer, Büyükpamukçu Münevver
Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey.
Pediatr Neurosurg. 2006;42(2):89-94. doi: 10.1159/000090461.
Eighty-six patients under 3 years of age with central nervous system tumors were retrospectively analyzed between 1972 and 2003. Surgical resection was done in all patients except for those with optic glioma, pons glioma and pineal tumor. Three different chemotherapy regimens were used in different time periods. In 48 patients, the tumor was located in the posterior fossa, and 29 patients had a supratentorial tumor. We had 32 (37.2%) patients with embryonic tumors (21 medulloblastoma, 4 ependymoblastoma, 5 with atypical teratoid rhabdoid and 2 with supratentorial primitive neuroectodermal tumors), 21 (24.4%) with ependymoma, 14 (16.3%) with optic glioma, 10 (11.6%) with astrocytoma, 3 (3.5%) with pons glioma and 6 (7.0%) with others. Overall (OS) and event-free survival rates were 49.5 and 40.9%. OS rates according to the tumor localizations were 40.9 and 68.1% in the posterior fossa and supratentorial localizations, respectively (p=0.001). OS rates were 33.7, 41.3 and 88.8% for the medulloblastoma+primitive neuroectodermal tumor groups, ependymoma and astrocytoma, respectively (p=0.0001). Most of the patients had primitive embryonic tumors (37.2%). The best prognostic factors were tumor localization and histology.
对1972年至2003年间86例3岁以下的中枢神经系统肿瘤患者进行了回顾性分析。除视神经胶质瘤、脑桥胶质瘤和松果体肿瘤患者外，所有患者均接受了手术切除。在不同时间段使用了三种不同的化疗方案。48例患者肿瘤位于后颅窝，29例患者肿瘤位于幕上。我们有32例（37.2%）胚胎性肿瘤患者（21例髓母细胞瘤、4例室管膜母细胞瘤、5例非典型畸胎样横纹肌样瘤和2例幕上原始神经外胚层肿瘤），21例（24.4%）室管膜瘤患者，14例（16.3%）视神经胶质瘤患者，10例（11.6%）星形细胞瘤患者，3例（3.5%）脑桥胶质瘤患者和6例（7.0%）其他肿瘤患者。总生存率（OS）和无事件生存率分别为49.5%和40.9%。根据肿瘤定位，后颅窝和幕上定位的OS率分别为40.9%和68.1%（p=0.001）。髓母细胞瘤+原始神经外胚层肿瘤组、室管膜瘤和星形细胞瘤的OS率分别为33.7%、41.3%和88.8%（p=0.0001）。大多数患者患有原始胚胎性肿瘤（37.2%）。最佳预后因素是肿瘤定位和组织学。
