Guo Ailing, Suresh Vigneyshwar, Liu Xianzhi, Guo Fuyou
Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, ZhengZhou, 450052, HeNan Province, People's Republic of China.
Key Laboratory of Neurosurgical Diseases, The First Affiliated Hospital of Zhengzhou University, Jianshe East Road No 1, ZhengZhou, HeNan Province, 450052, People's Republic of China.
Childs Nerv Syst. 2017 Mar;33(3):447-455. doi: 10.1007/s00381-017-3341-1. Epub 2017 Feb 8.
Giant pediatric intracranial tumor (GPIT) remains to be a challenging disease with high morbidity and mortality.
The clinical data of 60 patients under 18 years of age operated on with GPIT (≥5 cm in diameter) were retrospectively analyzed.
Gross total resection was achieved in 46 cases (77%) and subtotal resection was obtained in 14 cases (23%). Ninety percent (47/52) of the cases with obstructive hydrocephalus were resolved remarkably and only 10% (5/52) of the patients needed a ventriculoperitoneal shunt after tumor resection. Postoperative histopathological type revealed malignant brain tumors in 51 cases (85%). The most common neoplasm was medulloblastoma; other types include the following: ependymoma, pilocytic astrocytoma, mixed glioma, primitive neuroectoderm tumor (PNET), subependymal giant cell astrocytoma, gangliocytoma, gliosarcoma, choroid plexus carcinoma, and atypical teratoid rhabdoid tumor (AT/RT). Benign histopathological categories include the following: craniopharyngioma, choroid plexus papilloma, and meningioma. No death or serious complications occurred after the operation. However, transient subcutaneous effusion occurred in 14 patients postoperatively, nerve injury including the posterior cranial nerve in two cases and abduction nerve in one case, and mutism and pulmonary infection were observed in two cases, respectively. The follow-up period ranged from 1 to 72 months, with an average of 32 months. Poor prognosis occurred significantly in the high-grade malignant tumors and PNET, AT/RT, and gliosarcoma were implicated in the death of patients within 1 year.
To the best of our knowledge, the present study is the first description of clinicopathological features and the largest case analysis for GPIT. Optimal outcomes for GPIT were achieved by strict evaluation and perioperative management as well as microsurgical skills.
巨大儿童颅内肿瘤(GPIT)仍然是一种具有高发病率和死亡率的挑战性疾病。
回顾性分析60例18岁以下接受GPIT(直径≥5 cm)手术的患者的临床资料。
46例(77%)实现了全切除,14例(23%)实现了次全切除。90%(47/52)的梗阻性脑积水病例得到明显缓解,肿瘤切除后仅10%(5/52)的患者需要行脑室腹腔分流术。术后组织病理学类型显示51例(85%)为恶性脑肿瘤。最常见的肿瘤是髓母细胞瘤;其他类型包括:室管膜瘤、毛细胞型星形细胞瘤、混合性胶质瘤、原始神经外胚层肿瘤(PNET)、室管膜下巨细胞星形细胞瘤、神经节细胞瘤、胶质肉瘤、脉络丛癌和非典型畸胎样横纹肌样肿瘤(AT/RT)。良性组织病理学类别包括:颅咽管瘤、脉络丛乳头状瘤和脑膜瘤。术后未发生死亡或严重并发症。然而,14例患者术后出现短暂皮下积液,2例出现包括后颅神经在内的神经损伤,1例出现展神经损伤,2例分别出现缄默症和肺部感染。随访时间为1至72个月,平均32个月。高级别恶性肿瘤的预后明显较差,PNET、AT/RT和胶质肉瘤与1年内患者死亡有关。
据我们所知,本研究是对GPIT临床病理特征的首次描述以及最大规模的病例分析。通过严格评估、围手术期管理以及显微外科技术,实现了GPIT的最佳治疗效果。