Mokhtech Meriem, Morris Christopher G, Indelicato Daniel J, Rutenberg Michael S, Amdur Robert J
Department of Radiation Oncology, University of Florida College of Medicine, Gainesville.
Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, FL.
Am J Clin Oncol. 2018 Oct;41(10):1015-1018. doi: 10.1097/COC.0000000000000407.
The objective of this study is to evaluate long-term outcomes, specifically patterns of tumor recurrence, in patients with adult medulloblastoma treated with radiotherapy.
We recorded outcomes of 28 (median age, 25 y) patients 18 years old or above with M0 to M3 medulloblastoma treated between 1971 and 2012. Among them, 61% had standard-risk disease. All received craniospinal irradiation with a posterior fossa boost. Median dose to the craniospinal axis was 36 Gy. Median total dose to the primary site was 55.9 Gy and 46% received chemotherapy.
Median follow-up among survivors was 14.2 years. At 5 and 10 years, local control was 80% and 73%, overall survival was 71% and 59%, cause-specific survival was 71% and 63%, and freedom from progression was 68% and 59%. Tumors recurred in 11 patients (39%); median time to recurrence was 2.4 years, and 82% of recurrences developed in patients with standard-risk disease. Of the recurrences, 55% involved the primary site; 36% were bone metastases without neural axis recurrence. There were no isolated recurrences in the spinal canal or ventricular system outside of the posterior fossa.
The primary site is the main site of medulloblastoma recurrence, with isolated bone metastases more common in adults than children. Our results prompt us to consider 4 modifications to our treatment approach with adults who present with medulloblastoma: (1) initial staging to include PET or bone scan; (2) radiotherapy dose to the primary site of 59.4 Gy; (3) chemotherapy during and/or following radiotherapy in medically fit patients; (4) follow-up to include PET or bone scan twice a year for 3 years.
本研究旨在评估接受放射治疗的成年髓母细胞瘤患者的长期预后，尤其是肿瘤复发模式。
我们记录了1971年至2012年间接受治疗的28例（中位年龄25岁）18岁及以上M0至M3级髓母细胞瘤患者的预后情况。其中，61%患有标准风险疾病。所有患者均接受了全脑全脊髓照射及后颅窝推量照射。全脑全脊髓轴的中位剂量为36Gy。原发部位的中位总剂量为55.9Gy，46%的患者接受了化疗。
幸存者的中位随访时间为14.2年。5年和10年时，局部控制率分别为80%和73%，总生存率分别为71%和59%，病因特异性生存率分别为71%和63%，无进展生存率分别为68%和59%。11例患者（39%）出现肿瘤复发；复发的中位时间为2.4年，82%的复发发生在标准风险疾病患者中。在复发患者中，55%累及原发部位；36%为骨转移且无神经轴复发。在后颅窝以外的椎管或脑室系统中未出现孤立性复发。
原发部位是髓母细胞瘤复发的主要部位，孤立性骨转移在成人中比儿童更常见。我们的研究结果促使我们考虑对成年髓母细胞瘤患者的治疗方法进行4项改进：（1）初始分期包括PET或骨扫描；（2）原发部位的放射治疗剂量为59.4Gy；（3）身体状况适宜的患者在放疗期间和/或放疗后进行化疗；（4）随访3年，每年进行两次PET或骨扫描。
