Ovarian Dysgerminoma in Children and Adolescents

Overview

Background.
To review the treatment strategy, follow up and outcome for all patients with pure ovarian dysgerminomatreated in childhood and adolescence.Methods and Results. Twenty-one patients younger than 18 years were treated between 1979–2002 in FacultyHospital Motol for newly diagnosed pure ovarian dysgerminoma. Patients were included into the cohort on the basisof revision of archival biopsy specimens deposited in Institute of Pathology and inMolecularMedicine tumor registry.The staging was reviewed retrospectively on the basis of surgical and pathological findings and on results of imaginginvestigations and outlined according to the TNMand International Federation of Gynecology and Obstetrics (FIGO)classification. The median age at the time of diagnosiswas 12,5 years (range 6 years, 5months – 17 years, 11 months).There were ten FIGO stage IA tumors, one stage IB, two of stage IIC, one stage IIIB and seven IIIC tumors. Allpatients, except two girls with bilateral dysgerminoma, underwent unilateral adnexectomy or ovarectomy. Ten girlswere treated postoperatively with chemotherapy, eight with chemotherapy and radiotherapy (eleven with cisplatinebased chemotherapy). Three girls with dysgerminoma confined to the ovary (stage IA) have not received adjuvantchemotherapy. With a median follow up 7,1 years all girls remained continuously disease free. The 5-year overalland event free survival is 100%. Majority of patients does not have severe treatment sequelae, three pregnancieshave occurred so far.Conclusions. Most patients with dysgerminoma, including those with metastases can expect cure when treated withconservative surgery and cisplatine based chemotherapy. Reduction of treatment toxicity and preservation ofreproductive function is a main task. The girls with dysgerminoma confined to the ovary (stage IA) can be treatedwith fertility sparing surgery, other should be treated with cisplatine based chemotherapy.

Key words:
dysgerminoma, ovary, childhood, adolescence, treatment sequelae.