Gonadoblastoma (patient information)

Gonadoblastoma
Overview
What are the symptoms?
What are the causes?
Who is at highest risk?
Diagnosis
When to seek urgent medical care?
Treatment options
Where to find medical care for Gonadoblastoma?
Prevention
What to expect (Outlook/Prognosis)?
Possible complications
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Overview

Gonadoblastoma is a rare tumor of the gonads. It is an admixture of two types of cells, premature germ cells, and sex-cord stromal-like cells.

What are the Symptoms of Gonadoblastoma?

Gonadoblastoma has no specific signs and symptoms. It is a tumor usually arising from a maldeveloped gonad and it can manifest with non-specific symptoms such as:

• Excess hair overgrowth
• Delayed onset of menstrual bleeding
• Abnormal shape of genitalia

It may also present as a lower abdominal mass in women or growing mass in the testicles causing discomfort in men.

What Causes gonadoblastoma?

• The exact cause of gonadoblastoma is not clear, but it usually arises in the setting of maldeveloped sex organs.
• There is also a hypothesis regarding the presence of a putative gene called TSPY gene. Although the exact mechanism of its role in the pathogenesis of the disease is not clear, it has been observed that the product of this gene increased in the gonadoblastoma.

Who is at Highest Risk?

• Individuals with sex organ maldevelopment disorders are at highest risk of developing gonadoblastoma.
• Sex organ maldevelopment disorders are referred to disorders manifesting with an abnormal shape of genitalia during infancy or sexual organ developmental disorders during puberty such as delayed onset of menstruation, excess hair overgrowth.

Diagnosis

There is no single diagnostic tool for the diagnosis of gonadoblastoma. However, any individual with sex organ maldevelopment disorder is suspected of having gonadoblastoma and should be considered for a complete workup to diagnose the underlying disorder and should also be monitored by imaging studies for the presence or this tumor.

When to Seek Urgent Medical Care?

Individuals with a known diagnosis of sexual maldevelopment disorders such as:

• Androgen insensitivity syndrome
• Swyer syndrome
• Turner syndrome
• Frasier syndrome
• Denys-Drash syndrome
• 9p partial monosomy

should be screened for the presence of gonadoblastoma.

Treatment Options

• Surgical removal of the tumor is the mainstay of the treatment.
• Surgical removal of the affected gonad must be considered in case of coexisting sex development disorders.
• Since this tumor can affect the contralateral gonad in 40% of cases, it must be removed in case of simultaneous involvement or be followed closely by imaging studies in case of preservation.

Where to find Medical Care for Gonadoblastoma?

Medical care for gonadoblastoma can be found here.

Prevention

Gonadoblastoma is not preventable.

What to Expect (Outlook/Prognosis)?

• Gonadoblastoma prognosis is excellent after the surgical removal of the tumor.
• In the case of a coexisting malignant tumor, the prognosis depends on the staging of that tumor.

Possible Complications

Gonadoblastoma can be complicated by conversion to dysgerminoma, a malignant tumor, and/or other malignant gonadal tumors.

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/000434.htm