Ova Sex Cord-Stromal Tumors

Ovarian Sex Cord–Stromal Tumors in Children and Adolescents

D.T. Schneider, G. Calaminus, R. Wessalowksi, R. Pathmanathan, B. Selle, W. Sternschulte, D. Harms, U. Göbel

From the Department of Pediatric Hematology and Oncology, Children’s Hospital, Heinrich-Heine-University Medical Center, Düsseldorf; Department of Pediatric Hematology and Oncology, Children’s Hospital, University of Heidelberg; Children’s Hospital Köln, Köln; and Institute for Pediatric Pathology, Christian-Albrechts-University, Kiel, Germany.

Address reprint requests to D.T. Schneider, MD, Clinic of Pediatric Oncology, Hematology, and Immunology, Children’s Hospital, University Hospital Düsseldorf, Heinrich-Heine-University, Moorenstr 5, D-40225 Düsseldorf, Germany; email: dominik.schneider@uni-duesseldorf.de.

Purpose: To develop diagnostic standards and a risk-adapted therapeutic strategy for ovarian sex cord–stromal tumors (OSCST).

Patients and Methods: Fifty-four patients were prospectively enrolled as follow-up patients onto the German Maligne Keimzelltumoren protocols. Surgical protocols and histopathology were reviewed centrally (53 patients with complete data).

Surgery included ovariectomy in 18 patients, salpingo-ovariectomy in 34 patients, and hysterectomy in one patient. Patients with stage IA tumors were followed-up at regular intervals, whereas nine patients with stage IC and six patients with stage II to III tumors were treated with cisplatin-based chemotherapy.

Results: International Federation of Gynecology and Obstetrics stage was IA in 27 patients, IC in 21 patients, II in three patients, and III in three patients. After a median follow-up of 59 months (range, 6 to 193 months), event-free survival ± SD was 0.86 ± 0.05 (47 of 54 patients) and overall survival was 0.89 ± 0.05 (49 of 54 patients).

Prognosis correlated with stage (event-free survival ± SD: IA, 1.0 [27 of 27 patients]; IC, 0.76 ± 0.09 [16 of 21 patients]; and II/III, 0.67 ± 0.19 [four of six patients]; P = .02). Ten of 15 patients treated with chemotherapy, including four of six stage II to III patients, are alive after a median follow-up of 33 months.

Conclusion: On the basis of a standardized clinical and histopathologic assessment, risk-adapted therapeutic strategies for OSCST can be evaluated.

Considering our experience, we would recommend that stage IA tumors be followed up at regular intervals, whereas we would recommend cisplatin-based chemotherapy in stage IC tumors with preoperative rupture or malignant ascites, especially those with high mitotic activity.

Finally, cisplatin-based chemotherapy also seems to be effective in advanced-stage tumors.

Supported by the Deutsche Krebshilfe, Bonn, Germany.

Journal of Clinical Oncology, Vol 21, Issue 12 (June), 2003: 2357-2363

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