Billmire D, Vinocur C, Rescorla F, Colombani P, Cushing B, Hawkins E, Davis M, London W B, Lauer S, Giller R
Section of Pediatric Surgery, J.W. Riley Hospital for Children, Indianapolis, Indiana 46202, USA.
J Pediatr Surg. 2003 Mar;38(3):315-8; discussion 315-8. doi: 10.1053/jpsu.2003.50100.
BACKGROUND/PURPOSE: This randomized study examined survival (S) and event-free survival (EFS) rates using high-or standard-dose cisplatin-based combination chemotherapy and surgical resection for this subset of germ cell tumors.
Twenty-six of 317 patients enrolled on the POG 9049/COG 8882 intergroup study for malignant germ cell tumors had abdomen or retroperitoneum as the primary site. Twenty-five of 26 were eligible for inclusion (n = 25). Patients had biopsy or resection at diagnosis and randomization to chemotherapy including etoposide, bleomycin, and either standard-dose (PEB) or high-dose cisplatin (HDPEB). In patients with initial biopsy, delayed resection was planned.
Median age was 26 months. There were 14 girls and 11 boys. There were 3 stage I to II, 5 stage III, and 17 stage IV patients. Surgical management included primary resection in 5, resection after chemotherapy in 13, and biopsy or partial resection in 7 patients. Overall 6-year EFS rate was 82.8% +/- 10.9%, and 6-year survival rate was 87.6% +/- 9.3%. By group, 6-year survival rate was 90.0% +/- 11.6% for PEB and 85.7 +/- 14.5% for HDPEB. Deaths include one from sepsis, one from malignant tumor progression, and one from bulky disease caused by benign components despite response of the malignant elements to chemotherapy.
Malignant germ cell tumors arising in the abdomen and retroperitoneum have an excellent prognosis despite advanced stage in most children. Aggressive resection need not be undertaken at diagnosis, but a concerted attempt at complete surgical removal after chemotherapy is important to distinguish viable tumor from necrotic tumor or benign elements that will not benefit from further chemotherapy.
背景/目的：本随机研究使用基于顺铂的高剂量或标准剂量联合化疗及手术切除，对该亚组生殖细胞肿瘤的生存率（S）和无事件生存率（EFS）进行了研究。
在POG 9049/COG 8882恶性生殖细胞肿瘤组间研究中登记的317例患者中，26例以腹部或腹膜后为主要部位。26例中的25例符合纳入标准（n = 25）。患者在诊断时进行活检或切除，并随机接受包括依托泊苷、博来霉素以及标准剂量顺铂（PEB）或高剂量顺铂（HDPEB）的化疗。对于初始活检的患者，计划进行延迟切除。
中位年龄为26个月。有14名女孩和11名男孩。有3例I至II期、5例III期和17例IV期患者。手术处理包括5例初次切除、13例化疗后切除以及7例患者的活检或部分切除。总体6年EFS率为82.8%±10.9%，6年生存率为87.6%±9.3%。按组计算，PEB组的6年生存率为90.0%±11.6%，HDPEB组为85.7±14.5%。死亡包括1例死于败血症、1例死于恶性肿瘤进展以及1例尽管恶性成分对化疗有反应但因良性成分导致的巨大肿块疾病死亡。
尽管大多数儿童处于晚期，但腹部和腹膜后发生的恶性生殖细胞肿瘤预后良好。诊断时无需进行积极切除，但化疗后协同努力完整切除肿瘤对于区分存活肿瘤与坏死肿瘤或不会从进一步化疗中获益的良性成分很重要。
