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A Result of Treatment of Malignant Germ Cell Tumors in Childhood

Journal of the Korean Pediatric Society 1997;40(12):1707-1718.
Published online December 15, 1997.
A Result of Treatment of Malignant Germ Cell Tumors in Childhood
Hyoung Jin Kang1, Jun Ah Lee1, Hyo Jeong Han1, Hyoung Su Choi1, Hyeon Jin Park1, Ki Woong Sung1, Eun Sun Yoo1, Hee Young Shin1, Hyo Seop Ahn1, Kwi Won Park2, Byung Keu Cho3, Hwang Choi4, Chul Woo Kim5
1Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
2Department of Pediatric Surgery, Seoul National University, College of Medicine, Seoul, Korea
3Department of Neurosurgery, Seoul National University, College of Medicine, Seoul, Korea
4Department of Urology, Seoul National University, College of Medicine, Seoul, Korea
5Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea
소아 악성 생식세포종의 치료 성적
강형진1, 이준아1, 한효정1, 최형수1, 박현진1, 성기웅1, 유은선1, 신희영1, 안효섭1, 박귀원2, 조병규3, 최황4, 김철우5
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 소아외과학교실
3서울대학교 의과대학 신경외과학교실
4서울대학교 의과대학 비뇨기과학교실
5서울대학교 의과대학 병리학교실
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.
Key Words: Germ cell tumor, Children, Chemotherapy, Survival rate, Prognostic factor


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