BACKGROUND: Germ cell tumor is a rare malignancy accounting for 3% of all pediatric tumors. They are highly curable malignancies. The aim of this study was to evaluate the clinical presentation, management, and outcome in this subset. METHODOLOGY: The study population included 40 patients with age less than 18 years at diagnosis. They were treated in Cancer Institute Chennai, India from 1990 to 2002. They were analyzed for the various clinical, pathologic presentations, and survival outcomes. Actuarial method was used to estimate the overall survival and relapse-free survival. RESULTS: The mean age of the study group was 14+2.7 years with most of the patients being postpubertal. The common histologies being mixed germ cell tumor (32%) and dysgerminoma (27%). Ten percent of patients presented with ovarian torsion. Sixty-two percent of patients presented in advanced stage. Fertility preservation surgery was possible in 70% of the patients. Relapses were seen in 25% of the patients. The median duration of follow-up was 7.5 years with a 5 years disease-free survival of 72.8% and overall survival of 94.9%. Most of the patients achieved a good quality of life with normal menstrual cycles. CONCLUSIONS: This study confirms an excellent outcome for girls with ovarian germ cell tumor, although majority of the patients presented in advanced stage. Patients with initial histology of Teratoma and Mixed germ cell tumor relapsed frequently. The mainstay of treatment being fertility preservation and cisplatin-based chemotherapy.
BACKGROUND: Germ cell tumor is a rare malignancy accounting for 3% of all pediatric tumors. They are highly curable malignancies. The aim of this study was to evaluate the clinical presentation, management, and outcome in this subset. METHODOLOGY: The study population included 40 patients with age less than 18 years at diagnosis. They were treated in Cancer Institute Chennai, India from 1990 to 2002. They were analyzed for the various clinical, pathologic presentations, and survival outcomes. Actuarial method was used to estimate the overall survival and relapse-free survival. RESULTS: The mean age of the study group was 14+2.7 years with most of the patients being postpubertal. The common histologies being mixed germ cell tumor (32%) and dysgerminoma (27%). Ten percent of patients presented with ovarian torsion. Sixty-two percent of patients presented in advanced stage. Fertility preservation surgery was possible in 70% of the patients. Relapses were seen in 25% of the patients. The median duration of follow-up was 7.5 years with a 5 years disease-free survival of 72.8% and overall survival of 94.9%. Most of the patients achieved a good quality of life with normal menstrual cycles. CONCLUSIONS: This study confirms an excellent outcome for girls with ovarian germ cell tumor, although majority of the patients presented in advanced stage. Patients with initial histology of Teratoma and Mixed germ cell tumor relapsed frequently. The mainstay of treatment being fertility preservation and cisplatin-based chemotherapy.