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 |
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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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