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

Presented at the 34th Annual Meeting of the American Pediatric Surgical Association, Fort Lauderdale, Florida, May 25–28, 2003.
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Abstract

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.

Section snippets

Materials and methods

Two intergroup studies for extracranial malignant germ cell tumors in children and adolescents were undertaken by the Pediatric Oncology Group (POG) and Children’s Cancer Study Group (CCG) from 1990 to 1996. Children with stage I and II ovarian tumors were entered onto POG 9048/CCG 8891 and were treated in a nonrandomized fashion with tumor resection followed by 4 cycles of chemotherapy. The regimen included cisplatin, 100 mg/m2/d×5, etoposide 100 mg/m2/d×5 and bleomycin 15 mg/m2/d×1 (PEB) for

Results

There were 131 girls with malignant germ cell tumors of the ovary entered onto the combined intergroup studies including 41 stage I, 16 stage II, 58 stage III, and 16 stage IV tumors. Age ranged from 1.4 to 20 years with mean age of 11.9 yrs.

Information on clinical presentation was available from the operative notes in 82 patients. Of these 82 girls, 9 presented with an acute abdomen with a preoperative diagnosis of appendicitis in 7 and peritonitis in 2. Three of these girls had torsion of the

Discussion

Survival rate of girls with malignant germ cell tumors of the ovary was excellent in this study with an incidence of only 3% (4 of 131) primary tumor-related mortality. Previous recommendations for extensive surgical resection of reproductive organs1 have been successfully abandoned without compromise in outcome. The high survival rate with conservative surgery can be credited to the development of effective chemotherapeutic agents. There still is significant variability in surgical procedure,

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