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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.

Purpose: This study was designed to evaluate (1) the efficacy of standard or high-dose cisplatin with etoposide and bleomycin and (2) the role of surgical resection in infants and children with malignant germ cell tumors (MGCT) of the genital region., Methods: Fourteen of 317 children enrolled in to the Pediatric Oncology Group/Children's Cancer Group intergroup study of MGCT from 1990 through 1996 had genital tumors. Thirteen were eligible for inclusion (12 vaginal, one penile). The initial procedure was biopsy in 11 and subtotal resection in 2. Patients were assigned randomly to receive 4 cycles of etoposide, bleomycin, and either standard or high-dose cisplatin., Results: Nine children underwent postchemotherapy excision of the residual site, and 2 had subsequent biopsies to confirm a complete response. Two with relapse were saved with additional therapy, and one with progressive disease died. The 4-year event-free survival rate in these patients is 76.2% +/- 13.1%, and 4-year survival rate is 91.7% +/- 8.4%., Conclusions: The author conclude that: (1) the current survival rate for genital MGCT is excellent, (2) delayed surgical resection with organ preservation is not associated with an adverse outcome, and (3) the treatment comparison of the effect of cisplatin dose was inconclusive in this small study population.

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., Methods: 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., Results: 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., Conclusions: 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., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: The aim of this study was to correlate outcomes in patients with stage I testicular germ cell tumors with compliance to surgical guidelines and to confirm previous single-institution experiences that show excellent disease-free survival rates when treated with orchiectomy alone., Methods: Sixty-three patients were entered into this intergroup study (Children's Cancer Group 8881/Pediatric Oncology Group 9048) between 1990 and 1996. Surgical guidelines for orchiectomy included an inguinal approach, early vascular control of the cord structures, and a hemiscrotectomy if a transscrotal violation occurred. Failure of tumor marker normalization or subsequent elevation suggested advanced disease requiring further surgery and chemotherapy. Survival curves were compared using the log-rank test., Results: The median age was 16 months (range, 1 month to 5.6 years). Progression of disease occurred in 11 patients. The 6-year event-free survival (EFS) was 78.5% + 7%. The overall 6-year survival rate was 100%. Adherence to all guidelines was confirmed in only 43 patients (69%). Overall, there was no statistical significance in event-free survival rates between patients who had a correct surgical approach and all patients who had surgical violations; however, 4 patients with transscrotal violations showed a significantly higher rate of disease recurrence., Conclusions: Patients with stage I germ cell tumors of the testes have excellent survival rates when treated with surgery alone. Despite numerous surgical guideline violations, event-free survival in this multiinstitutional study was similar to previous single-institution reports. All patients with relapse or progression of their disease appear to be cured with further surgical excision and chemotherapy., (Copyright 2003, Elsevier Science (USA). All rights reserved.)