A Result of Treatment of Malignant Germ Cell Tumors in Childhood

ABSTRACT

PURPOSE: Germ cell tumors have variable clinical characteristics according to the age, sex, primary site, and pathologic diagnosis. To provide the therapeutic principle, the clinical characteristics, response to the treatment, and prognostic factors were analyzed. METHODS: Medical records of 57 children who were diagnosed as malignant germ cell tumor in Seoul National University Children's Hospital from January 1986 till June 1996 were reviewed. RESULTS: 1) Fifty seven cases (male 22, female 43, true hermaphrodite 1) were analyzed. The age of diagnosis was between 2 months and 15 years and median age was 4 year 7 month. 2) The primary sites were sacrococcygeal area (28%), intracranial area (25%), ovary (18%), testis (11%), retroperitoneum (7%), and mediastinum (4%) in order of frequency. Another cases were in liver, spinal cord, skull base, tongue, and ovary & testis in hermaphrodite. 3) Pathologic diagnosis was possible in 53 cases. Mixed germ cell tumor (37%), endodermal sinus tumor (23%), germinoma (13%), immature teratoma (8%), embryonal carcinoma (4%), and choriocarcinoma (2%) were included in order of frequency. 4) The stage by CCG/POG classification revealed that 6% of stage I, 19% of stage II, 43% of stage III, and 32% of stage IV in 53 cases. 5) After the initial surgery or needle biopsy, radiation therapy was done in the intracranial tumor with EP induction or modified CCG 8891, etc. and cis-VAB, EP, VAB-3, VAC, or CCG 8891, etc. was done in the extracranial tumor with/without radiation therapy. 6) The response rate revealed that 71% of complete response, 22% of partial response, and 7% of progressive disease in 45 cases. 7) The response rate was related to the primary site but not pathology and stage. All the gonadal tumors had complete response. 8) The 5 year-survival rate of all malignant germ cell tumors was 73%. Stage 4 or sacrococcygeal tumor had poor survival rate. CONCLUSIONS: Stage 4 and sacrococcygeal area were thought as the poor prognostic factors affecting survival. Survival rate of tumors at sacrococcygeal area were poor owing to the relatively younger age of onset, and poor response rate. The 5 year-survival rate of tumors treated with cis-VAB were 74% but shown complications frequently. So BEP will be preferred.