Malignant Genital Tumors in Childhood – Yesterday, Today and Tomorrow

Overview

Genital tumors in children and adolescents represent 1.5 to 2.0 % of al malignancies in these age groups. Organincidence differs from that in adult women. In children and in young adolescents non-epithelial gynaecologicaltumors predominate, while carcinomas are rare and their incidence rises with the age of girls. Malignant tumors ofthe external genital are very rare (sarcomas of the soft tissues). Malignancies of vagina are represented by theembryogenic rabdomyosarcoma, yolk sack tumor and tumor of pale cells or vaginal adenocarcinoma.All these tumorsare highly malignant. Cytostatics are used as the basic therapy and only later the less radical surgery is recommended.Radiotherapy is used in chemoresistant tumors. Vaginal bleeding of the premenarcheal girl is an early symptom andrequires immediate examination, including vaginoscopy. Tumors of uterus in childhood do not occur and they arerare in postmenarcheal girls. Ovarian tumors represent about 1.5% of all tumors in childhood and adolescence andabout 95% of all gynaecologic tumors. They differ in types from those of adults: Epithelial tumors (carcinomas) donot occur in childhood, germinal and gonadal stromal tumors are typical in this age. Mature differentiated teratomasare usually benign and the less differentiated they are, the worse biological effect they have (not mature or mixedteratomas). It seems that nowadays the proportion of immature and mixed teratomas has been rising. Dysgerminomoccurs more frequently in Y-chromosome karyotypes. It has malignant progression with early propagation alonglymph vessels into the lymph nodes. Beside ovarectomy, also lymphadenectomy at the affected side is performedand the treatment proceeds with chemotherapy. For the prognostic reasons, immunological examinations, DNAploidity identification and other tests are recommended. Gonadal stromal tumors are always unilateral, malignant,and frequently hormonally active, but they usually have a good prognosis. In order to preserve fertility of the younggirl with ovarian tumors, uterus should be spared as well as the macroscopically healthy contra-lateral ovarium. Toprotect gonad from the adverse effects of oncological treatment, pharmacologically induced regression to premenarchealstadium has been tested. Present development of paediatric and adolescent gynaecology is aimed to preservefor patients with tumors all prospects of future pregnancy, either in the natural way or using methods of assistedreproduction with a donated oocyte. Complex treatment of gonadalmalignancies in childhood in future will be aimednot only at the lifesaving but also at the preservation of the highest possible quality of life, including the motherhood.

Key words:
genital tumors, paediatric and adolescent gynaecology, ovarian tumors.