Göbel U, Calaminus G, Engert J, Kaatsch P, Gadner H, Bökkerink J P, Hass R J, Waag K, Blohm M E, Dippert S, Teske C, Harms D
Department of Pediatric Hematology and Oncology, Heinrich Heine University, Düsseldorf, Germany.
Med Pediatr Oncol. 1998 Jul;31(1):8-15. doi: 10.1002/(sici)1096-911x(199807)31:1<8::aid-mpo2>3.0.co;2-h.
Outcomes in children with teratomas collected between October 1982 and December 1995 in cooperative protocols of the German Society of Pediatric Oncology and Hematology (GPOH) were analyzed. Teratomas were diagnosed in 329 (42%) of 780 registered patients with germ cell tumors. The annual incidence was 0.24/100,000. Main primary sites were coccygeal (n = 132, 2.2:1 female predominance), ovary (n = 81), testis (n = 40) and brain (n = 15, 2.8:1 male predominance).
Two hundred seventy cases of extracranial non-testicular teratoma were evaluated: In mature teratomas (n = 154) the observed relapse rate was 10%. Incomplete resection was the main risk factor for relapse. After complete resection, the relapse-free survival (RFS, Kaplan-Meier-estimation) was 0.96 +/- 0.01 (n = 126, observation time 18-155 months) in comparison to an RFS of 0.56 +/- 0.09 in incompletely resected teratomas grade 0 (n = 28, observation time 28-94 months) (P < 0.01). Im-mature teratomas were treated by surgery alone in 76 cases and by surgery and adjuvant chemotherapy in 40 cases. The observed relapse risk was 18%. Main risk factors for relapse were incomplete tumor resection (n = 38) as well as immaturity in incompletely resected teratomas. Fifteen of 29 relapsing patients presented with malignant tissue in the recurrent tumor (mainly yolk sac tumor); in contrast, seven of 40 patients with immature teratoma relapsed despite adjuvant chemotherapy without showing malignant components (P = 0.014). Nine of 36 (25%) relapsing patients died of disease. Eleven of the 27 (41%) surviving children suffered from mutilation after repeated surgery.
It is suggested that an international randomized trial for patients with incompletely resected high risk teratoma be initiated to evaluate the effect of adjuvant chemotherapy on specific end-points: 1) influence on relapse rate in general; 2) reduction of the proportion of malignant relapses; 3) avoidance of mutilating surgery.
分析了1982年10月至1995年12月期间德国儿科肿瘤学和血液学学会（GPOH）合作方案中收集的畸胎瘤患儿的治疗结果。在780例登记的生殖细胞肿瘤患者中，329例（42%）被诊断为畸胎瘤。年发病率为0.24/10万。主要原发部位为尾骨（n = 132，女性优势比为2.2:1）、卵巢（n = 81）、睾丸（n = 40）和脑（n = 15，男性优势比为2.8:1）。
对270例颅外非睾丸畸胎瘤进行了评估：在成熟畸胎瘤（n = 154）中，观察到的复发率为10%。切除不完全是复发的主要危险因素。完全切除后，无复发生存率（RFS，Kaplan-Meier估计）为0.96±0.01（n = 126，观察时间18 - 155个月），相比之下，不完全切除的0级畸胎瘤（n = 28，观察时间28 - 94个月）的RFS为0.56±0.09（P < 0.01）。76例未成熟畸胎瘤仅接受手术治疗，40例接受手术及辅助化疗。观察到的复发风险为18%。复发的主要危险因素是肿瘤切除不完全（n = 38）以及不完全切除的畸胎瘤不成熟。29例复发患者中有15例复发肿瘤中出现恶性组织（主要为卵黄囊瘤）；相比之下，40例未成熟畸胎瘤患者中有7例尽管接受了辅助化疗仍复发，且未显示恶性成分（P = 0.014）。36例复发患者中有9例（25%）死于疾病。27例存活儿童中有11例（41%）在多次手术后出现肢体残缺。
建议针对不完全切除的高危畸胎瘤患者开展一项国际随机试验，以评估辅助化疗对特定终点的影响：1）总体上对复发率的影响；2）降低恶性复发的比例；3）避免致残性手术。
