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
Laboratory Findings
- A chromosome analysis is diagnostic of Gonadoblastoma.
Imaging Findings
- There are no [imaging study] findings associated with [disease name].
- [Imaging study 1] is the imaging modality of choice for [disease name].
- On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- [Disease name] may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for [disease name]; the mainstay of therapy is supportive care.
- The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
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.
Prevention
- There are no primary preventive measures available for [disease name].
- Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].