46, XX 핵형을 가진 여자에서 발생한 생식샘모세포종에 동반된 미분화세포종 -2예 보고-
Gonadoblastoma Overgrown by Dysgerminoma in Women with 46, XX Karyotype: A Report of Two Cases
김미정, 안희정, 김지영, 김규래,
소속 상세정보
김미정 ( Kim Mi-Jung )
울산대학교 의과대학 서울아산병원 병리과
안희정 ( Ahn Hee-Jeong )
포천중문의과대학교 분당차병원 병리과
김지영 ( Kim Ji-Young )
포천중문의과대학교 분당차병원 병리과
김규래 ( Kim Kyu-Rae )
울산대학교 의과대학 서울아산병원 병리과
KMID : 0357920030370010066
Abstract
Gonadoblastoma is a neoplasm containing an intimate mixture of germ cells and elements resembling immature granulosa or Sertoli cells. It has been considered as in situ germ cell malignancy that can be overgrown by more malignant germ cell neoplasms. The tumor has been reported to almost exclusively develop in various types of gonadal maldevelopment syndromes containing the Y chromosome, such as in pure or mixed gonadal dysgenesis and, less commonly, in male hermaphroditism. However, occurrences in phenotypically and chromosomally normal, menstruating women are exceptionally rare. We report two cases of gonadoblastoma overgrown by dysgerminoma occurring in the ovaries of phenotypically and cytogenetically normal menstruating women. One of the two cases showed an area composed of granulosa cell tumor-like elements. This type of combination has been very rarely described, and exemplified that gonadoblastoma may progress to sex cord-stromal tumors as well as to the malignant germ cell tumors.
키워드
Gonadoblastoma;Dysgerminoma;Sex Cord;Stromal Tumor;Gonadal Dysgenesis;46;XX
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