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婴幼儿期和儿童期畸胎瘤的恶性潜能:MAKEI在非睾丸畸胎瘤方面的经验及对新方案的启示

The malignant potential of teratomas in infancy and childhood: the MAKEI experiences in non-testicular teratoma and implications for a new protocol.

作者信息

Göbel U, Calaminus G, Schneider D T, Koch S, Teske C, Harms D

机构信息

Department of Pediatric Oncology, Hematology and Immunology, Children's-Hospital, UKD, Heinrich-Heine-University, Düsseldorf.

出版信息

Klin Padiatr. 2006 Nov-Dec;218(6):309-14. doi: 10.1055/s-2006-942275.

Abstract

UNLABELLED

Since 1982, mature and immature teratomas have been recruited into the MAHO and MAKEI protocols of the German Society for Pediatric Oncology and Hematology (GPOH) for testicular and non-testicular germ cell tumors in order to study the epidemiology and clinical behaviour of teratomas. Patients were registered in the epidemiologic German Childrens Cancer Registry and the GPOH Childrens Tumor Registry for pathological review. Patients with immaturity grade 2 and 3 according to Gonzales-Crussi were eligible for adjuvant chemotherapy. The consecutive protocols MAKEI 83/86/89 have been published previously in detail (Klin Paediatr 1997; 209: 228-234, Med Pediat Oncol 1998; 31: 8-15) and will be compared to the data of MAKEI 96. For this comparison, 274 patients from MAKEI 83/86/89 and 261 patients from MAKEI 96 are evaluable.

RESULTS

  1. EFS after complete tumor resection has been estimated to 0.96 +/- 0.01 in both observation periods. 2) Incomplete tumor resection remains the main risk factor for relapse (EFS 0.55 +/- 0.09). 3) The relapse rate declined from 13.9 % in MAKEI 83/86/89 to 9.5 % in MAKEI 96. 4) In MAKEI 83/86/89 four newborns with teratoma died due to perioperative complications and nine children as a result of tumor progression, whereas in MAKEI 96 no newborn died, only one child died from tumor progression, and another child died during long time observation for another reason (meningitis). 5) In accordance to the experience of the MAKEI 83/86/89 studies, no child of the MAKEI 96 study presented with yolk sac tumor at recurrence if adjuvant chemotherapy was administered during first-line treatment because of immaturity. In contrast, more than half of the children with tumor recurrence after watch and wait strategy had yolk sac tumor in addition to teratoma.
摘要

未标注

自1982年以来,成熟和不成熟畸胎瘤被纳入德国儿科肿瘤学和血液学协会(GPOH)针对睾丸和非睾丸生殖细胞肿瘤的MAHO和MAKEI方案,以研究畸胎瘤的流行病学和临床行为。患者在德国儿童癌症流行病学登记处和GPOH儿童肿瘤登记处登记,以便进行病理复查。根据Gonzales-Crussi分级为2级和3级不成熟的患者符合辅助化疗条件。连续的MAKEI 83/86/89方案此前已详细发表(《临床儿科》1997年;209:228 - 234,《医学儿科肿瘤学》1998年;31:8 - 15),并将与MAKEI 96的数据进行比较。为进行此次比较,MAKEI 83/86/89的274例患者和MAKEI 96的261例患者可进行评估。

结果

1)在两个观察期内,肿瘤完全切除后的无事件生存率估计为0.96±0.01。2)肿瘤切除不完全仍然是复发的主要危险因素(无事件生存率0.55±0.09)。3)复发率从MAKEI 83/86/89的13.9%降至MAKEI 96的9.5%。4)在MAKEI 83/86/89中,4例患有畸胎瘤的新生儿死于围手术期并发症,9例儿童死于肿瘤进展,而在MAKEI 96中,无新生儿死亡,仅1例儿童死于肿瘤进展,另1例儿童在长时间观察期间因其他原因(脑膜炎)死亡。5)根据MAKEI 83/86/89研究的经验,如果因不成熟在一线治疗期间给予辅助化疗,MAKEI 96研究中的儿童复发时无卵黄囊瘤。相比之下,在观察等待策略后肿瘤复发的儿童中,超过一半除畸胎瘤外还患有卵黄囊瘤。

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