title: Ovarian sex cord-stromal tumors in children and adolescents.
abstract: OBJECTIVE
  To develop diagnostic standards and a risk-adapted therapeutic strategy for ovarian sex cord-stromal tumors (OSCST).
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatric Hematology and Oncology, Children's Hospital, Heinrich-Heine-University Medical Center, Düsseldorf, Germany. dominik.schneider@uni-duesseldorf.de
authors.forename: D T
authors.initials: DT
authors.lastname: Schneider
authors.forename: G
authors.initials: G
authors.lastname: Calaminus
authors.forename: R
authors.initials: R
authors.lastname: Wessalowski
authors.forename: R
authors.initials: R
authors.lastname: Pathmanathan
authors.forename: B
authors.initials: B
authors.lastname: Selle
authors.forename: W
authors.initials: W
authors.lastname: Sternschulte
authors.forename: D
authors.initials: D
authors.lastname: Harms
authors.forename: U
authors.initials: U
authors.lastname: Göbel
authors.forename: R
authors.initials: R
authors.lastname: Wessalowksi
mesh_terms: D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D010052:Ovariectomy; D011446:Prospective Studies; D018312:Sex Cord-Gonadal Stromal Tumors / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D018709:Statistics, Nonparametric; D016896:Treatment Outcome