소아 고환 배세포종양의 치료와 예후
Clinical Experience of Testicular Germ Cell Tumor in Infants and Children
소속 상세정보
윤상진/Sang Jin Yoon
김용태/이안기/오승준/김광명/최황/Yong-Tae Kim/Ahnkie Lee/Seung-June Oh/Kwang Myung Kim/Hwang Choi
KMID : 0358319980390040361
Abstract
서론
소아연령에 있어서 고환종양은 소아 10만명당 0.5에서 2명 정도의 발병률을 보이고 전체
소아 교형 악성종양의 1%내외를 차지한다고 알려져 있다. 이와 같은 희귀성으로 인하여 소
아 고환종양 발생률과 유병율, 병리적인 분포나 예후 등과 같은 임상경과에 대하여 정확히
알려져 있지 않다.
미국의 경우 Section of Urology of the American Academy of Pediatrics에 의하여 1980
년에야 처음으로 소아 고환암 환자들에 대한 등록 프로그램(Prepubertal Testicular Tumor
Registry. 이하 PTTR)이 시작되어 1953년에 327례에 대한 임상소견이 보고되었으나, 한국
의 경우 고환암에 대해서는 보고가 있긴 하지만 이는 증례 발표 수준이거나 소수 환자만을
수집하여 보고된 것이기 때문에 성인의 그것과는 매우 다른 임상양상을 보이는 소아에서의
고환암에 대해서는 그 자료가 극히 부족한 실정이다. 이미 알려진 바와 같이 인종에 따라
발병률이 다르다는 보고를 고려할 때 한국에서의 소아고환종양에 대한 전반적인 파악이 절
실하다 이에 서울대학교병원에서 지난 35년간 경험하였던 원발성 소아 고환 배세포종양
(germ cell tumor) 74례에 대한 임상 경험을 보고하고자 한다.
본 보고에서는 소아 고환 배세포종양의 병기와 나이에 따른 분포 및 치료, 예후에 관하여
조사하였다 특히 국소 난황낭 종양(yolk sac tumor, 이하 YST)의 재발된 사례에서 병리학
적 위험요소에 대한 고찰을 가장 비중 있게 다루었다. 아울러 사춘기 이전 고환 기형종
(teratoma)의 치료에 있어서 고환보존술식의 적용 가능성을 고려하여 보았다.
#초록#
Purpose : The relative rarity of prepubertal testis tumors has resulted in poor
understanding about incidence, histologic distribution and prognosis of germ cell tumors
in children. We attempted to elucidate overall clinical features of testicular germ cell
tumors in infants and children, to analyze risk factors for relapse in stage A yolk sac
tumor(YST) and to find out possibility of testis-sparing surgery in prepubertal testicular
teratoma.
Materials and Methods : Retrospectively, we reviewed 74 cases of primary testicular
germ cell tumors of infants and children(under 15 years old) experienced in Seoul
National University Hospital from January 1970 to November 1995.
Results : There were 38 YSTs(stage A, 34 cases, stage B; 3, stage C; 1), 32
teratomas(all stage A) and 4 teratocarcinomas(stage A, 3, stage C; 1). Median age of
presentation was 2.0(range 0.4-15.0) years. Preoperative serum alpha-fetoprotein (α-FP)
elevation was observed in 37 patients(100%) with YST, 2(7%) with teratoma, 3(100%)
with teratocarcinoma and elevated serum beta human chorionic gonadotropin(β-HCG)
was observed in one patient(33%) with teratocarcinoma. Orchiectomy including 4 partial
orchiectomy(all with teratoma) was done in all patients, chemotherapy in all patients
with teratocarcinoma and stage B, C YST. Two-year survival rate was 92%(22 out of
24 patients who had been followed up) and 100%(19/19) with teratoma. Out of 4 patients
with teratocarcinoma, 2 patients with stage A disease showed no evidence of disease,
one patient progressed to death and follow-up was lost in the other one patient with
stage C.
In 9 patients with YST who had ultimately presented recurrence, initial symptoms and
signs for recurrence were as follows; marker elevation(4), abdominal mass(3), inguinal
mass(1) and scrotal mass(1). Their mean duration of relapse after surgery was 7.0
months. On pathologic review, 7 patients among total 34 patients with YST had at least
one risk factors such as epididymal involvement(2), microangioinvasion(3) and focal
embryonal component(2). Out of them(7), 5 patients proved to have relapse. There was
statistically significant relationship between having risk factors and relapse(Fisher's
exact test, p=0.0086). Complete remission rate after chemotherapy In all relapsed patients
was 83%(5/6). Until now, there is no evidence of recurrence in 4 patients with teratoma
who underwent partial orchiectomy
Conclusions : YST is characterized as early presentation, high relapse rate and short
relapse interval and had high response rate to chemotherapy and high survival rate.
Therefore, careful surveillance is needed at least for 2 year in stage A patients with
high risk group on pathology(microangioinvasion etc). Testis-sparing surgery may be
considered as one of treatment options in prepubertal teratoma.
키워드
Testis tumor; Children; Yolk sac tumor; Risk factor; Partial orchiectomy;
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