Biswas Ahitagni, Julka Pramod Kumar, Bakhshi Sameer, Singh Manmohan, Rath Goura Kishor
Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India.
Pediatr Neurosurg. 2017;52(4):240-249. doi: 10.1159/000474946. Epub 2017 May 25.
Primary intracranial germ cell tumour is a rare entity and constitutes 2-3% of all paediatric brain tumours in Western countries. We herein intend to report the clinical features and treatment outcome of patients with primary central nervous system germ cell tumour treated at our institute.
Clinical data were collected by retrospective chart review from 2006 to 2012. Histopathology slides were reviewed and relevant immunohistochemistry stains were done. Overall survival (OS) and progression-free survival (PFS) were analysed by the Kaplan-Meier product-limit method.
Twenty patients met the study criterion (male:female = 7:3). Median age at presentation was 13 years. Tumour location was pineal in 10 patients, suprasellar in 6, thalamic in 2, basal ganglion in 1, and spinal in 1. Leptomeningeal spread was noted in 1 patient at presentation. Surgical resection was gross-total in 7 patients (35%), near-total in 2 (10%), subtotal in 4 (20%), and limited to biopsy in 6 (30%). The tumours were germinomatous, non-germinomatous, and of mixed germ cell subtype in 17 patients (85%), 2 patients (10%), and 1 patient (5%), respectively. Systemic chemotherapy (median of 4 cycles) was given to 19 patients (95%). The common regimens used were a combination of bleomycin, etoposide and cisplatin (BEP) in 14 patients (70%) and etoposide and cisplatin (EP) in 5 patients (25%). Radiation therapy (40-50 Gy in conventional fractionation; median of 42 Gy) was delivered to 17 patients (85%): local radiation in 6 and whole ventricular, whole brain, and craniospinal irradiation followed by a boost in 5, 3, and 3 patients, respectively. After a median follow-up of 44.52 months, 17 patients (85%) were in complete response and 3 (15%) had progressive disease. Death and disease recurrence were noted in 6 patients (30%) and 1 patient, respectively. Median OS and PFS were not reached. The actuarial rates of OS at 3 and 5 years were 75.8 and 68.9%, respectively. The actuarial rates of PFS at both 3 and 5 years were 81.6%.
Multimodality treatment consisting of limited resection followed by platinum-based systemic chemotherapy and radiotherapy (40-50 Gy) is a reasonable treatment strategy in patients of primary central nervous system germ cell tumour in a developing nation.
原发性颅内生殖细胞肿瘤是一种罕见疾病，在西方国家占所有儿童脑肿瘤的2% - 3%。我们在此报告在我院接受治疗的原发性中枢神经系统生殖细胞肿瘤患者的临床特征及治疗结果。
通过回顾性病历审查收集2006年至2012年的临床数据。复查组织病理学切片并进行相关免疫组化染色。采用Kaplan-Meier乘积限界法分析总生存期（OS）和无进展生存期（PFS）。
20例患者符合研究标准（男∶女 = 7∶3）。就诊时的中位年龄为13岁。肿瘤位于松果体区10例，鞍上区6例，丘脑2例，基底节1例，脊髓1例。1例患者就诊时发现软脑膜播散。7例患者（35%）手术切除为全切，2例（10%）近全切，4例（20%）次全切，6例（30%）仅行活检。肿瘤为生殖细胞瘤、非生殖细胞瘤和混合生殖细胞亚型的分别有17例（85%）、2例（10%）和1例（5%）。19例患者（95%）接受了全身化疗（中位4个周期）。常用方案为博来霉素、依托泊苷和顺铂联合（BEP），共14例（70%）；依托泊苷和顺铂联合（EP），共5例（25%）。17例患者（85%）接受了放射治疗（常规分割剂量40 - 50 Gy；中位剂量42 Gy）：局部放疗6例，全脑室、全脑和全脊髓放疗后加量分别为5例、3例和3例。中位随访44.52个月后，17例患者（85%）完全缓解，3例（15%）疾病进展。分别有6例（30%）患者死亡和1例患者疾病复发。未达到中位OS和PFS。3年和5年的OS精算率分别为75.8%和68.9%。3年和5年的PFS精算率均为81.6%。
对于发展中国家原发性中枢神经系统生殖细胞肿瘤患者，采用有限切除后行铂类全身化疗和放疗（40 - 50 Gy）的多模式治疗是一种合理的治疗策略。
