16例伴有睾丸侵犯的伯基特淋巴瘤患儿的临床特征及疗效分析

The clinical characteristics and outcomes of 16 Burkitt＇ s lymphoma with testicular involvement

目的总结伴有睾丸侵犯的儿童伯基特淋巴瘤的临床特征，以及应用大剂量甲氨蝶呤（HD-MDX，5-8g／m2）等联合化疗取代睾丸放疔的临床疗效及预后。方法收集16例伴有睾丸侵犯的伯基特淋巴瘤患儿资料，分析其临床特征，按前瞻性方案设计及分层。全部患儿进入高危组分层治疗，并定期追踪随访。方案采用北京儿童医院淋巴瘤治疗方案中的NHL-BCH-2009．成熟B细胞淋巴瘤方案（来自改良的LMB-89方案联合利妥昔单抗治疗）。结果137例伯基特淋巴瘤患儿中伴睾丸侵犯的患儿16例（11．7％）。16例患儿均为Ⅳ期，发病中位年龄为6．5（2．25～13．5）岁。16例患儿中8例存在骨髓侵犯（其中5例已达白血病期），9例存在中枢神经系统受累（其中5例为中枢神经系统白血病，4例为颅内或脊髓占位）；5例为双侧睾丸侵犯，11例为单侧睾丸侵犯；1例病初存在近期EBV感染（EBV-IgM＋）。中位随访时间为31．8（0．5～79．0）个月，16例患儿中死亡2例（1例因早期化疗合并症放弃治疗死亡，1例于早期停药后复发死亡）；1例停药2年8个月后出现膀胱部位肿瘤复发，经治疗目前处于完全缓解；13例无事件生存，无睾丸复发。3年总生存率为87．5％，3年无事件生存率为72．9％。追踪其中〉10岁患儿（3例）的睾酮水平，均在正常范围。结论对于伴有睾丸侵犯的伯基特淋巴瘤患儿，为减少放疗的远期不良反应、保留睾丸功能，应用HD-MTX等联合化疗取代睾丸放疗，疗效好，不增加睾丸复发率，性腺功能正常，但远期不良反应及睾丸功能仍有待观察。

Objective To analyze the clinical features and outcomes of Burkitt lymphoma with testicular involvement and study the efficiency of high dose methotrexate （HD-MTX： 5-8g/m2） in those patients without radiation therapy. Method Retrospective analysis was conducted in 16 Burkitfs lymphoma cases with testicular lymphoma involvement between Jan 2009 and Dec 2014. We followed the BCH-NHL-2009 protocol, modified from FAB LMB 89 combined with rituximab. All patients were enrolled in high-risk group （treated by Group C protocol）. Results Of 137 Burkitt lymphoma, 16 （11.67%） had testicular involvement. All the patients were in stage IV, the median age was 6.65 years （ranges： 2.25 to 13.5 years）. 8 cases had bone marrow involvement, 9 with central nervous system involvement, 5 with bi-testicular involvement, 1 with EB virus infection （EBV-IgM＋）. The median follow- up was 31,8 months （ranges： 0.5 to 79 months）. During the study period, 2 cases died, 1 due to the disease relapse, the other one due to chemo-related dead. 1 had disease relapse 32 months after off treatment; the other 13 cases were all event free survival. 3-year OS was 87.5%, 3-year EFS was 72.9%. We also found the level of testosterone in ten adolescent were normal before and after chemotherapy. Conclusion For the Burkitt lymphoma with testicular involvement, we abandon radiotherapy, and administer HD-MTX to lower the toxicity. The short-term survival is better, long-term survival still needs to be clarified.