BACKGROUND: 'Eight drugs in a day' was one of the widely used regimen in medulloblastoma. Result of treatment of this regimen and comparison between pre-RT chemotherapy and post-RT chemotherapy were presented. METHODS: Medical records of children who were diagnosed as medulloblastoma and treated with 8 in 1 therapy in Seoul National University Children's Hospital from January 1986 to June 1997 were reviewed. RESULTS: 1) Forty nine cases(male: 30, female: 19) were analyzed. The age at diagnosis was between 3 months and 15 years 3 months and median age was 7 years 10 months. 2) The T stage by Chang classification revealed T1(2%), T2(26%), T3a(9%), T3b(56%), and T4(7%) in 43 cases. M stage revealed M0(46%), M1(15%), M3(37%), and M4(2%) in 41 cases. The surgical results revealed gross total resection(36%), near total resection(18%), subtotal resection(38%), and partial resection(9%). 3) The 5-yr disease free survival(DFS) rate of all tumors was 53%. There was no difference in DFS about sex, age, pathology, T stage, M stage, and surgical result. 4) Difference of 5-year DFS between pre-RT chemotherapy group(53%) and post-RT chemotherapy group(83%) was significant[-2Log(LR), P=0.023], excluding the patient relapsed or too young(below 18 month, who had more chemotherapy to delay radiation) to be received radiation therapy. CONCLUSIONS: DFS in post-RT chemotherapy was better than pre-RT chemotherapy. As medulloblastoma has the property to confine in CNS system, prior treatment with radiation as a intensive local therapy may be more effective than the systemic chemotherapy. Progression or relapse were the main causes of treatment failure. Result of treatment may be improved by more intensive therapy.
Child ; Classification ; Diagnosis ; Drug Therapy* ; Female ; Humans ; Medical Records ; Medulloblastoma* ; Pathology ; Recurrence ; Seoul ; Treatment Failure

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.
Age of Onset ; Biopsy, Needle ; Carcinoma, Embryonal ; Child ; Choriocarcinoma ; Classification ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Germinoma ; Gonads ; Humans ; Liver ; Mediastinum ; Medical Records ; Neoplasms, Germ Cell and Embryonal* ; Ovary ; Pathology ; Pregnancy ; Seoul ; Skull Base ; Spinal Cord ; Survival Rate ; Teratoma ; Testis ; Tongue

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.
Age of Onset ; Biopsy, Needle ; Carcinoma, Embryonal ; Child ; Choriocarcinoma ; Classification ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Germinoma ; Gonads ; Humans ; Liver ; Mediastinum ; Medical Records ; Neoplasms, Germ Cell and Embryonal* ; Ovary ; Pathology ; Pregnancy ; Seoul ; Skull Base ; Spinal Cord ; Survival Rate ; Teratoma ; Testis ; Tongue