Chou Yung-Chih, Wu Yao-Yu, Hung Sheng-Ping, Tsang Ngan-Ming, Pai Ping-Ching, Jaing Tang-Her, Lin Chien-Yu, Hsieh Cheng-En, Fan Kang-Hsing, Kao Wei-Heng, Tseng Chen-Kan
Department of Radiation Oncology, Proton and Radiation Therapy Center, Linkou Chang Gung Memorial Hospital.
Division of Hematology and Oncology, Chang Gung Children's Hospital; Taoyuan, Taiwan.
J Pediatr Hematol Oncol. 2021 Oct 1;43(7):e907-e912. doi: 10.1097/MPH.0000000000002006.
To investigate the clinical utility of short-course induction chemotherapy followed by low-dose radiotherapy without a tumor bed boost in patients with primary central nervous system (CNS) germinomas.
We retrospectively reviewed the clinical records of patients with primary CNS germinomas who received short-course induction chemotherapy (2 cycles of cisplatin 20 mg/m2 plus etoposide 40 or 100 mg/m2 for 5 days) followed by low-dose radiotherapy (dose: 2340 cGy) without a tumor bed boost. Disease-free survival and overall survival served as the main outcome measures.
Between February 2002 and June 2018, 24 patients (20 males and 4 females; median age: 14.1 y; age range: 7.9 to 21.2 y) with pathology-proven CNS germinomas were included. The median follow-up time was 106 months (range: 17 to 169 mo). Isolated and multifocal lesions were identified in 13 and 11 patients, respectively. Tumor location was as follows: pineal gland (n=17), suprasellar region (n=13), periventricular region (n=7), and basal ganglia (n=2). Five patients had increased levels (>5 mIU/mL) of beta-human chorionic gonadotropin (β-hCG), whereas alpha-fetoprotein concentrations were within the reference range in all participants. A total of 16 patients achieved remission after induction chemotherapy. The complete response rates of patients with increased and normal β-hCG levels were 40.0% and 72.2%, respectively (P=0.208). Low-dose radiotherapy without a tumor bed boost was subsequently delivered to either the whole ventricle (n=16) or the whole brain (n=8), resulting in complete remission in all participants. Compared with patients without increased β-hCG levels, those with β-hCG-secreting germinomas had less favorable 5-year disease-free survival rates (100% vs. 60%, respectively, P=0.000115).
Some children with primary CNS germinoma may benefit from short-course induction chemotherapy followed by low-dose radiotherapy to the whole ventricle without a tumor bed boost. The validity of our findings needs to be confirmed in a randomized phase II study for children with β-hCG levels <5 mIU/mL.
探讨短程诱导化疗后低剂量放疗且不进行瘤床加量照射在原发性中枢神经系统（CNS）生殖细胞瘤患者中的临床应用价值。
我们回顾性分析了接受短程诱导化疗（顺铂20mg/m²加依托泊苷40或100mg/m²，共5天，2个周期）后进行低剂量放疗（剂量：2340cGy）且不进行瘤床加量照射的原发性CNS生殖细胞瘤患者的临床记录。无病生存期和总生存期作为主要观察指标。
2002年2月至2018年6月期间，纳入了24例经病理证实的CNS生殖细胞瘤患者（20例男性，4例女性；中位年龄：14.1岁；年龄范围：7.9至21.2岁）。中位随访时间为106个月（范围：17至169个月）。分别在13例和11例患者中发现孤立性和多灶性病变。肿瘤位置如下：松果体区（n = 17）、鞍上区（n = 13）、脑室周围区（n = 7）和基底节区（n = 2）。5例患者β-人绒毛膜促性腺激素（β-hCG）水平升高（>5mIU/mL），而所有参与者的甲胎蛋白浓度均在参考范围内。共有16例患者在诱导化疗后达到缓解。β-hCG水平升高和正常的患者的完全缓解率分别为40.0%和72.2%（P = 0.208）。随后对16例患者进行了全脑室低剂量放疗，对8例患者进行了全脑低剂量放疗，所有参与者均完全缓解。与β-hCG水平未升高的患者相比，分泌β-hCG的生殖细胞瘤患者的5年无病生存率较低（分别为100%和60%，P = 0.000115）。
一些原发性CNS生殖细胞瘤患儿可能从短程诱导化疗后全脑室低剂量放疗且不进行瘤床加量照射中获益。我们的研究结果的有效性需要在一项针对β-hCG水平<5mIU/mL的儿童的随机II期研究中得到证实。
