Biswas Ahitagni, Mallick Supriya, Purkait Suvendu, Gandhi Ajeet, Sarkar Chitra, Singh Manmohan, Julka Pramod Kumar, Rath Goura Kishor
Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India, 110029,
Childs Nerv Syst. 2015 Aug;31(8):1291-304. doi: 10.1007/s00381-015-2751-1. Epub 2015 Jun 4.
Pinealoblastoma is a highly malignant embryonal tumour of the pineal region affecting children and young adults. We herein intend to report the clinical features and treatment outcome of patients of pinealoblastoma treated at our institute.
Clinical data was collected by retrospective chart review from 2003-2012. Histopathology slides were reviewed, and relevant immunohistochemistry stains were done. Overall survival (OS) and recurrence-free survival (RFS) were analysed by Kaplan-Meier product-limit method. Univariate and multivariate analyses of prognostic factors were done by log rank test and Cox proportional hazard regression model, respectively.
Seventeen patients met the study criterion (male:female = 11:6). Median age at presentation was 14 years (range 4-47 years). Surgical resection was gross total in 6 (35.29%), near-total in 2 (11.76%), sub-total in 2 (11.76%), and limited to biopsy in 7 (41.18 %) patients. At presentation, 4 patients had leptomeningeal dissemination. Radiation therapy was delivered in all patients-craniospinal irradiation in 15 (88.24%), whole brain irradiation in 1 (5.88%), and whole ventricular irradiation followed by boost in 1 (5.88%) patient. Systemic chemotherapy (median 6 cycles) was given in 14 (82.35%) patients. The most common regimen was a combination of carboplatin and etoposide, used in 10 (58.82%) patients. After a median follow-up of 30.3 months (mean 32.01 months), death and disease recurrences were noted in 3 (17.65%) and 7 (41.18%) patients. Amongst the patients with recurrent disease, 4 had spinal drop metastases and 3 had local recurrence along with spinal drop metastases. Median OS was not reached, and estimated median RFS was noted to be 5.49 years. The actuarial rates of OS and RFS at 2 years were 85.6 and 73.1%, respectively. On univariate analysis, age more than 8 years (P = 0.0071) and M0 stage (P = 0.0483) were significant predictors of improved RFS. Age retained significance on multivariate analysis of RFS (P = 0.02932).
Maximal safe resection followed by craniospinal irradiation and systemic chemotherapy with 6 cycles of carboplatin-etoposide regimen is a reasonable treatment strategy in patients of pinealoblastoma more than 8 years of age in a developing nation. However, the same strategy is less effective in younger children and innovative study designs of intensification of post-operative treatment must be explored in this age group.
松果体母细胞瘤是一种发生于松果体区域的高度恶性胚胎性肿瘤，主要影响儿童和青年。本文旨在报告我院治疗的松果体母细胞瘤患者的临床特征及治疗结果。
通过回顾性病历审查收集2003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）和无复发生存期（RFS）。分别采用对数秩检验和Cox比例风险回归模型对预后因素进行单因素和多因素分析。
17例患者符合研究标准（男∶女 = 11∶6）。就诊时的中位年龄为14岁（范围4 - 47岁）。6例（35.29%）患者实现了肉眼全切，2例（11.76%）近全切，2例（11.76%）次全切，7例（41.18%）患者仅行活检。就诊时，4例患者有软脑膜播散。所有患者均接受了放疗，15例（88.24%）接受全脑脊髓照射，1例（5.88%）接受全脑照射，1例（5.88%）患者接受全脑室照射后加量照射。14例（82.35%）患者接受了全身化疗（中位6个周期）。最常用的方案是卡铂和依托泊苷联合，10例（58.82%）患者使用。中位随访30.3个月（平均32.01个月）后，3例（17.65%）患者死亡，7例（41.18%）患者疾病复发。在复发患者中，4例有脊髓播散转移，3例有局部复发并伴有脊髓播散转移。未达到中位OS，估计中位RFS为5.49年。2年时的OS和RFS精算率分别为85.6%和73.1%。单因素分析显示，年龄大于8岁（P = 0.0071）和M0期（P = 0.0483）是RFS改善的显著预测因素。在RFS的多因素分析中，年龄仍具有显著性（P = 0.02932）。
对于发展中国家8岁以上的松果体母细胞瘤患者，最大安全切除后行全脑脊髓照射及6周期卡铂 - 依托泊苷方案的全身化疗是一种合理的治疗策略。然而，该策略在年幼儿童中效果较差，必须探索针对该年龄组强化术后治疗的创新性研究设计。
