Gonadoblastoma

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List of terms related to Gonadoblastoma

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

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

• There are no specific laboratory findings associated with [disease name].
• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

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].