Lundar Tryggve, Due-Tønnessen Bernt Johan, Egge Arild, Scheie David, Stensvold Einar, Brandal Petter
Department of Neurosurgery, Oslo University Hospital, Norway.
J Neurosurg Pediatr. 2013 Sep;12(3):241-6. doi: 10.3171/2013.6.PEDS12586. Epub 2013 Jul 19.
The object of this study was to delineate long-term results of the surgical treatment of pediatric CNS tumors classified as oligodendroglioma (OD) or oligoastrocytoma (OA) WHO Grade II or III.
A cohort of 45 consecutive patients 19 years or younger who had undergone primary resection of CNS tumors originally described as oligodendroglial during the years 1970-2009 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Gross motor function and activities of daily living were scored using the Barthel Index (BI).
Patient records for 35 consecutive children and adolescents who had undergone resection for an OA (17 patients) or OD (18 patients) were included in this study. Of the 35 patients, 12 were in the 1st decade of life at the first surgery, whereas 23 were in the 2nd decade. The male/female ratio was 1.19 (19/16). No patient was lost to follow-up. The tumor was localized to the supratentorial compartment in 33 patients, the posterior fossa in 1 patient, and the cervical medulla in 1 patient. Twenty-four tumors were considered to be WHO Grade II, and 11 were classified as WHO Grade III. Among these latter lesions were 2 tumors initially classified as WHO Grade II and later reclassified as WHO Grade III following repeat surgery. Fifty-four tumor resections were performed. Two patients underwent repeat tumor resection within 5 days of the initial procedure, after MRI confirmed residual tumor. Another 10 patients underwent a second resection because of clinical deterioration and progressive disease at time points ranging from 1 month to 10 years after the initial operation. Six patients underwent a third resection, and 1 patient underwent a fourth excision following tumor dissemination to the spinal canal. Sixteen (46%) of the 35 children received adjuvant therapy: 7, fractionated radiotherapy; 4, chemotherapy; and 5, both fractionated radiotherapy and chemotherapy. One patient with primary supratentorial disease experienced clinically malignant development with widespread intraspinal dissemination 9 years after initial treatment. Only 2 patients needed treatment for persistent hydrocephalus. In this series there was no surgical mortality, which was defined as death within 30 days of resection. However, 12 patients in the study, with follow-up times from 1 month to 33 years, died. Twenty-three patients, with follow-up times from 4 to 31 years, remained alive. Among these survivors, the BI was 100 (normal) in 22 patients and 80 in 1 patient. Nineteen patients had full- or part-time work or were in normal school programs.
Pediatric oligodendroglial tumors are mainly localized to the supratentorial compartment and more often occur in the 2nd decade of life rather than the 1st. Two-thirds of the patients remained alive after follow-ups from 4 to 31 years. Twelve children succumbed to their disease, 9 of them within 3 years of resection despite combined treatment with radio- and chemotherapy. Three of them remained alive from 9 to 33 years after primary resection. Among the 23 survivors, a stable, very long-term result was attainable in at least 20. Five-, 10-, 20-, and 30-year overall survival in patients with Grade II tumors was 92%, 92%, 92%, and 88%, respectively.
本研究的目的是描述手术治疗WHO II级或III级的儿童中枢神经系统少突胶质细胞瘤(OD)或少突星形细胞瘤(OA)的长期结果。
在这项关于手术发病率、死亡率以及学业成绩和/或工作参与情况的回顾性研究中,对1970年至2009年间在单一机构接受过最初描述为少突胶质细胞瘤的中枢神经系统肿瘤初次切除手术的45例19岁及以下连续患者进行了回顾。使用Barthel指数(BI)对粗大运动功能和日常生活活动进行评分。
本研究纳入了35例连续接受OA(17例患者)或OD(18例患者)切除手术的儿童和青少年的患者记录。在这35例患者中,12例在首次手术时处于生命的第一个十年,而23例处于第二个十年。男女比例为1.19(19/16)。没有患者失访。肿瘤位于幕上腔室的有33例患者,位于后颅窝的有1例患者,位于颈髓的有1例患者。24个肿瘤被认为是WHO II级,11个被分类为WHO III级。在这些后者的病变中,有2个肿瘤最初被分类为WHO II级,在重复手术后后来被重新分类为WHO III级。共进行了54次肿瘤切除。2例患者在初次手术后5天内,在MRI证实有残留肿瘤后接受了重复肿瘤切除。另外10例患者因初次手术后1个月至10年期间的临床恶化和疾病进展而接受了第二次切除。6例患者接受了第三次切除,1例患者在肿瘤扩散至椎管后接受了第四次切除。35例儿童中有16例(46%)接受了辅助治疗:7例接受分次放疗;4例接受化疗;5例同时接受分次放疗和化疗。1例原发性幕上疾病患者在初始治疗9年后出现临床恶性进展并伴有广泛的脊髓内扩散。只有2例患者因持续性脑积水需要治疗。在本系列中没有手术死亡,手术死亡定义为切除术后30天内死亡。然而,该研究中的12例患者,随访时间从1个月到33年,死亡。23例患者,随访时间从4年到31年,仍然存活。在这些幸存者中,22例患者的BI为100(正常),1例患者为80。19例患者有全职或兼职工作或参加正常学校课程。
儿童少突胶质细胞瘤主要位于幕上腔室,且更常发生在生命的第二个十年而非第一个十年。在4至31年的随访后,三分之二的患者仍然存活。12名儿童死于该疾病,其中9例在切除术后3年内死亡,尽管接受了放疗和化疗联合治疗。其中3例在初次切除后9至33年仍然存活。在23名幸存者中,至少20名可获得稳定的长期结果。II级肿瘤患者的5年、10年、20年和30年总生存率分别为92%、92%、92%和88%。