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The Korean Journal of Pathology 2003;37(1): 66-70.
Gonadoblastoma Overgrown by Dysgerminoma in Women with 46,XX Karyotype: A Report of Two Cases.
Mi Jung Kim, Hee Jeong Ahn, Ji Young Kim, Kyu Rae Kim
1Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. krkim@www.amc.seoul.kr
2Department of Pathology, Bundang Hospital, College of Medicine Pochon Cha University, Seongnam, Korea
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.
Key Words: Gonadoblastoma; Dysgerminoma; Sex Cord; Stromal Tumor; Gonadal Dysgenesis,46,XX