Outcome and staging evaluation in malignant germ cell tumors of the ovary in children and adolescents: an intergroup study.

PURPOSE The aim of this study was to perform an evaluation of outcome and the role of surgical staging components in malignant germ cell tumors (GCT) of the ovary in children and adolescents. METHODS From 1990 to 1996, 2 intergroup trials for malignant GCT were undertaken by Pediatric Oncology Group (POG) and Children's Cancer Study Group (CCG). Stage I-II patients were treated with surgical resection and 4 cycles of standard dose cisplatin (100 mg/m2/cycle), etoposide, and bleomycin (PEB) chemotherapy. Stage III-IV patients were treated with surgical resection and randomly assigned to chemotherapy with PEB or high-dose cisplatin (200 mg/m2/cycle) with etoposide and bleomycin (HDPEB). Patients unresectable at diagnosis had second-look operation after 4 cycles of chemotherapy if residual tumor was seen on imaging studies. IRB approval of the protocols was obtained at each participating institution. An analysis of outcome data, operative notes, and pathology reports in girls with ovarian primary site was done for this report. RESULTS There were 131 patients with ovarian primary tumors of 515 entered on these studies. Mean age was 11.9 years (range, 1.4 to 20 years). Six-year survival rate was stage, I 95.1%; stage II, 93.8%; stage III, 98.3%; stage IV, 93.3%. In only 3 of 131 patients were surgical guidelines followed completely. Surgical omissions resulting in protocol noncompliance resulted from failure to biopsy bilateral nodes (97%), no omentectomy (36%), no peritoneal cytology (21%), no contralateral ovary biopsy (59%). More aggressive procedure than recommended by guidelines included total hysterectomy and bilateral salpingo-oophorectomy in 6 patients and retroperitoneal node dissection in 10 patients. Correlation of gross operative findings with pathology results was carried out for ascites, lymph nodes, implants, omentum, and contralateral ovary. CONCLUSIONS Pediatric ovarian malignant GCT (stages I-IV) have excellent survival with conservative surgical resection and platinum-based chemotherapy. Survival appears to have been unaffected by deviations from surgical guidelines. New surgical guidelines are proposed based on correlation of gross findings, histology, and outcome in these intergroup trials.