Ovarian sex cord-stromal tumors in children and adolescents

J Clin Oncol. 2003 Jun 15;21(12):2357-63. doi: 10.1200/JCO.2003.05.038.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Chemotherapy, Adjuvant
  • Chi-Square Distribution
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Humans
  • Ovarian Neoplasms / drug therapy
  • Ovarian Neoplasms / pathology
  • Ovarian Neoplasms / surgery*
  • Ovariectomy
  • Prospective Studies
  • Sex Cord-Gonadal Stromal Tumors / drug therapy
  • Sex Cord-Gonadal Stromal Tumors / pathology
  • Sex Cord-Gonadal Stromal Tumors / surgery*
  • Statistics, Nonparametric
  • Treatment Outcome