[Malignant tumors of the female genitalia in childhood--yesterday, today and tomorrow].
Genital tumors in children and adolescents represent 1.5 to 2.0% of al malignancies in these age groups. Organ incidence differs from that in adult women. In children and in young adolescents non-epithelial gynaecological tumors predominate, while carcinomas are rare and their incidence rises with the age of girls. Malignant tumors of the external genital are very rare (sarcomas of the soft tissues). Malignancies of vagina are represented by the embryogenic rabdomyosarcoma, yolk sack tumor and tumor of pale cells or vaginal adenocarcinoma. All these tumors are 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 and requires immediate examination, including vaginoscopy. Tumors of uterus in childhood do not occur and they are rare in postmenarcheal girls. Ovarian tumors represent about 1.5% of all tumors in childhood and adolescence and about 95% of all gynaecologic tumors. They differ in types from those of adults: Epithelial tumors (carcinomas) do not occur in childhood, germinal and gonadal stromal tumors are typical in this age. Mature differentiated teratomas are usually benign and the less differentiated they are, the worse biological effect they have (not mature or mixed teratomas). It seems that nowadays the proportion of immature and mixed teratomas has been rising. Dysgerminom occurs more frequently in Y-chromosome karyotypes. It has malignant progression with early propagation along lymph vessels into the lymph nodes. Beside ovarectomy, also lymphadenectomy at the affected side is performed and the treatment proceeds with chemotherapy. For the prognostic reasons, immunological examinations, DNA ploidity 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 young girl with ovarian tumors, uterus should be spared as well as the macroscopically healthy contra-lateral ovarium. To protect gonad from the adverse effects of oncological treatment, pharmacologically induced regression to premenarcheal stadium has been tested. Present development of paediatric and adolescent gynaecology is aimed to preserve for patients with tumors all prospects of future pregnancy, either in the natural way or using methods of assisted reproduction with a donated oocyte. Complex treatment of gonadal malignancies in childhood in future will be aimed not only at the lifesaving but also at the preservation of the highest possible quality of life, including the motherhood.
Horejsí
J
J
Gynekologická a porodnická klinika 2. LF UK a FNM, Praha. jan.horejsi@lfmotol.cuni.cz
Rob
L
L
D000293:Adolescent; D002648:Child; D005260:Female; D005833:Genital Neoplasms, Female / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D010051:Ovarian Neoplasms / Q000175:diagnosis
Zhoubné nádory rodidel v dĕtském vĕku vcera, dnes a zítra.