Division of Pediatric Hematology-Oncology, İstanbul University Oncology Institute, İstanbul, Turkey.
Division of Pediatric Hematology- Oncology, MD Anderson Cancer Center, Houston, TX, USA.
Turk J Pediatr. 2021;63(6):955-961. doi: 10.24953/turkjped.2021.06.002.
Pineoblastomas (PB) are rare tumors of the central nervous system and are more common in children. There is no consensus about standard of care. The objective of this study is to analyze the outcome of children with PB.
Six patients with PB who were diagnosed between 1990-2012 were evaluated retrospectively. Demographics, age of diagnosis, first complaint, tumor region, diagnosis type, seeding metastasis to the spinal axis or cerebrospinal fluid (CSF), treatment and survival of these patients were recorded.
Three patients had subtotal resection and all patients received chemotherapy and craniospinal irradiation (CSI) after diagnosis. Median follow-up after treatment was 5.5 (range:1-19) years. Two patients are alive with no evidence of disease for 7.5 and 10 years, one of whom was diagnosed with papillary thyroid carcinoma 9.5 years after treatment. One of the patients who died had lived for 19 years after diagnosis.
Pineoblastomas are rare but very aggressive tumors; more effective treatment strategies are needed. Survivors should be followed up for late effects such as second malignancies and endocrine deficiencies.
成神经管细胞瘤（PB）是一种罕见的中枢神经系统肿瘤，在儿童中更为常见。目前尚无关于标准治疗的共识。本研究旨在分析儿童成神经管细胞瘤的预后。
回顾性分析了 1990 年至 2012 年间诊断的 6 例 PB 患儿的临床资料。记录患儿的一般资料、诊断年龄、首发症状、肿瘤部位、诊断类型、是否有脊髓轴播散或脑脊液转移、治疗方法和生存情况。
3 例患儿行肿瘤大部切除术，所有患儿均在诊断后接受化疗和全脑全脊髓放疗。治疗后中位随访时间为 5.5 年（范围：1-19 年）。2 例患儿无病生存 7.5 年和 10 年，其中 1 例在治疗后 9.5 年诊断为甲状腺乳头状癌。1 例死亡患儿的生存时间为诊断后 19 年。
成神经管细胞瘤是一种罕见但侵袭性很强的肿瘤，需要更有效的治疗策略。幸存者应随访晚期效应，如第二恶性肿瘤和内分泌缺陷。
