Gonadoblastoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Your Name
Synonyms and keywords: Synonym 1; Synonym 2; Synonym 3
Overview
Historical Perspective
- Gonadoblastoma was first discovered by Scully, in 1953.
Pathophysiology
- A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements, such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells.
- Abnormal chromosomal karyotype, gonadal dysgenesis, and the presence of a Y chromosome has been associated with the development of gonadoblastoma.
Causes
- Gonadoblastoma may be caused by either complete androgen insensitivity, pure gonadal dysgenesis or Swyer syndrome, mixed gonadal dysgenesis, Frasier syndrome, Denys-Drash syndrome, and 9p Partial monosomy.
Differentiating Gonadoblastoma from other Diseases
- Gonadoblastoma must be differentiated from other diseases that cause ovarian tumors, such as:
- Germinoma
- Cystadenoma
- Cystadenocarcinoma
- Endometrioid tumor
- Brenner tumor
- Leydig cell tumor
- Granulosa cell tumor
- 17-Hydroxylase Deficiency Syndrome
- 5-Alpha-Reductase Deficiency
- Amenorrhea
- Congenital Adrenal Hyperplasia
- Genital Anomalies
- Hypospadias
- Pediatric Seminoma
- Turner Syndrome
- Meigs syndrome
Other Diagnostic Studies
- Gonadoblastoma may also be diagnosed using chromosomal analysis.
Treatment
Surgery
- Surgery is the mainstay of therapy for gonadoblastoma.
- Surgical exploration via laparotomy and bilateral salpingo-oophorectomy (BSO) is the most common approach to the treatment of gonadoblastoma.