Smíšené germinální nádory ovaria u dětía dospívajícíchRetrospektivní klinicko-patologická studie

Mixed Germ Cell Tumours of the Ovary in Childhood and Adolescence

Mixed germ cell tumours of the ovary are rare malignant neoplasms containing combinations oftwo or more types of germ cell elements.The aim of the study was to review biopsy examinations, medical records, treatment strategy,follow-up and outcome of all girls treated for mixed germ cell tumour of the ovary at the Departmentof Pediatric Oncology, University Hospital Motol during the period 1979–2002.Archival slides of all tumours were reviewed and tumours were classified according to the WHOsystem. The clinical data on surgical treatment, chemotherapy and radiotherapy used and followupinformation were obtained in all girls. The staging was reviewed retrospectively on the basisof surgical and pathological findings and results of imaging investigations, and it was outlinedaccording to the most recent FIGO criteria and TNM classification.Sixteen girls with mixed germ cell tumour of the ovary, age range 3 years 11 months to 17 years 8months (median 12 years) were treated. All girls presented with unilateral tumour of the ovaryand all underwent surgery as an initial treatment. The most common presenting symptom wasabdominal pain, occurring in ten patients. The original diagnosis of mixed histology was confirmedin all cases; in five cases the tumour contained three histologic components, in eleven casesthe tumour consisted of two germ cell types. All tumours contained elements of yolk sac tumour,followed by immature teratoma, embryonal carcinoma, dysgerminoma and mature teratoma. Atthe time of diagnosis three patients had stage I disease, four patients stage II, seven stage III andtwo stage IV disease. All patients were treated with chemotherapy after surgery, predominantlywith platinum-based regimens (PVB, BEP). Three patients treated initially with MAC (metothrexate,dactinomycin, cyclophosphamide) were diagnosed in the early eighties. In seven girls withadvanced disease treated in the early years, radiotherapy was administered to the pelvis or wholeabdomen. Overall survival and event-free survival were 80% and 81.3% respectively (median follow-up time 7.6 years). Three patients have died from the disease, two progressed on treatment(MAC), one girl relapsed three months after finishing therapy, no further therapy was administered.One girl underwent resection of tumour of her remaining ovary 24 months after original diagnosis. Histology showed mixed serous and mucinous cystadenoma. The latest examinationsrevealed that all other patients were in good health.Microscopic examination should be extensive and careful to find out all types of malignant germcell elements. Platinum based chemotherapy is effective in the management of children andadolescents with mixed germ cell tumors of the ovary. Chemosensitivity of these tumours a most girls to have conservative surgery with possible preservation of reproductive function.

Key words:
mixed germ cell tumour – ovary – childhood – adolescence – treatment – chemotherapy