Arrhenoblastoma of Ovary

Arrhenoblastoma of Ovary

Article
Women's Health
Diseases & Conditions
+1
Contributed byMaulik P. Purohit MD MPHMay 02, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Androblastoma of Ovary
  • Gonadal Stromal Tumor of Ovary (Arrhenoblastoma)
  • Ovarian Androblastoma

What is Arrhenoblastoma of Ovary? (Definition/Background Information)

  • Arrhenoblastoma of Ovary is a rare tumor of the ovary (the egg-producing organ in females) that mainly affects young adult women
  • This tumor causes an excessive secretion of the male sex hormone ‘testosterone’ in females, leading to various signs and symptoms, which may include, excess body hair (on the chin, chest, etc.), stoppage of menses, reduction in size of breast, acne, broad shoulders, etc.
  • The exact cause of Arrhenoblastoma of Ovary is unknown and the condition cannot be prevented
  • A complete surgical removal is the treatment of choice. The prognosis depends upon the presence of metastatic tumors and it is generally guarded

Who gets Arrhenoblastoma of Ovary? (Age and Sex Distribution)

  • Arrhenoblastoma of Ovary can occur in women of any age; but, it is more common in younger women (in the 20-30 year age group)
  • The tumor is very rare and only about 1 in 200 ovarian tumors are arrhenoblastomas
  • It is found in women of all racial and ethnic groups across the world

What are the Risk Factors for Arrhenoblastoma of Ovary? (Predisposing Factors)

The following are the predisposing factors for Arrhenoblastoma of Ovary:

  • Women, who have never had children
  • Women, who have not breastfed their children
  • Having a family member with ovarian or breast cancer
  • The tumor may also arise due to complications of pregnancy (in rare cases)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Arrhenoblastoma of Ovary? (Etiology)

The exact cause of Arrhenoblastoma of Ovary remains unknown.

  • But, it is thought to occur due to some mutation in the genes (genetic change)
  • The amount of masculinization in Arrhenoblastoma of Ovary depends on the activity of the leydig cells, which produces the male sex hormone ‘testosterone’

What are the Signs and Symptoms of Arrhenoblastoma of Ovary?

The signs and symptoms of Arrhenoblastoma of Ovary depend on the size of the tumor and may include:

  • These tumors are usually slow-growing and form a single mass
  • Development of male type of hair distribution, such as on the chin and upper lip, chest, and extremities (hirsutism)
  • Enlargement of the clitoris (a part of the female genitalia)
  • Deepening of voice, due to enlargement of the voice box
  • Cessation of menses
  • Decrease in size of breasts
  • Redistribution of fat
  • Broadening of the shoulders
  • Acne of the face and chest
  • Loss of sexual desire
  • Increased musculature
  • Abdominal pain may occur in some cases

Small tumors usually do not cause any symptoms; but, occasionally may become painful, if they compress the surrounding structure.

How is Arrhenoblastoma of Ovary Diagnosed?

Arrhenoblastoma of Ovary is diagnosed by examination of the genital area, a thorough medical history, and also by the following tests:

  • Blood testosterone and androstenodione (male sex hormone) levels: Generally, these are elevated with Ovarian Arrhenoblastoma
  • Transvaginal ultrasound: An ultrasound is inserted into the vagina and the mass is examined using sound waves
  • Plain x-ray of the abdomen
  • CT or CAT scan with contrast of the abdomen usually shows a well-defined mass, which may have calcifications. This radiological procedure creates detailed 3-dimensional images of structures inside the abdomen
  • MRI scans of the abdomen: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor
  • Ultrasound scan of abdomen
  • Arrhenoblastoma of Ovary can additionally be evaluated using angiography of the abdomen region

Although the above modalities can be used to make the initial diagnosis, a tissue biopsy of the tumor is required to make a definitive diagnosis to begin treatment.

  • The tissue for diagnosis can be procured in multiple different ways, and they include:
    • Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred 
    • Core biopsy of the tumor
    • Open biopsy of the tumor
  • A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely electron microscopic studies to assist in the diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Arrhenoblastoma of Ovary?

The complications of Arrhenoblastoma of Ovary may include:

  • If not treated properly and in a timely manner, the tumor may spread to other parts of the body, such as to the abdomen, chest, etc.
  • Masculinising symptoms can have an emotional impact, especially on younger women
  • Damage of vital nerves, blood vessels, and surrounding structures, during surgery

How is Arrhenoblastoma of Ovary Treated?

There is no standard treatment protocol established for Arrhenoblastoma of Ovary. However, in majority of cases, a complete surgical excision is the preferred mode of treatment. The tumors are surgically removed with wide or clear margins, which may result in a cure, if the tumors are benign.

  • Long-term follow-up is required, because recurrence at the tumor at site of surgery or metastasis in distant sites have been reported many years after surgery even with benign appearing tumors
  • Radiotherapy can be used as primary therapy in situations where the tumor cannot be removed completely, or when the tumor reappears (recurrent Arrhenoblastoma of Ovary) after surgery.
  • Radiotherapy for Arrhenoblastoma of Ovary can also be used as additional therapy after surgery if there is a possibility of tumor recurrence after surgery, or if there are inadequate margins (possibility of tumor being left behind) following surgery. In some cases, due to the location of the tumor, complete surgical removal is difficult.
  • Chemotherapy can be used for treating Arrhenoblastoma of Ovary in following:
    • Tumors cannot be removed completely (incomplete surgical resection)
    • Tumors that recur after surgery (recurrent Arrhenoblastoma of Ovary)
    • Tumors that have spread to distant parts of the body (metastatic Arrhenoblastoma of Ovary)
  • Arterial embolization of the tumor is a possible treatment option. Here the blood supply to the tumor is blocked resulting in tumor death
  • Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Arrhenoblastoma of Ovary be Prevented?

  • Current medical research has not established a way of preventing Arrhenoblastoma of Ovary formation
  • Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations for those who have already endured the tumor are helpful

What is the Prognosis of Arrhenoblastoma of Ovary? (Outcomes/Resolutions)

  • The most reliable prognostic factor of Arrhenoblastoma of Ovary is dependent on whether the tumor can be completely removed through surgery with free margins (no traces of the tumor in adjoining tissue) or not
  • Individuals have an overall excellent survival rate following first complete surgical resection
  • As with any tumor, it is important to have follow-up appointments with a physician to monitor for any returning tumors
  • The prognosis of Ovarian Arrhenoblastoma also depends upon a set of several factors, which include:
    • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) 
    • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
    • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
    • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment

An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment.

Additional and Relevant Useful Information for Arrhenoblastoma of Ovary:

A tumor is an abnormal growth of tissue arising due to uncontrolled and rapid multiplication of cells that serve no function. They are also called neoplasms. Tumors can be benign or cancerous. A benign tumor may generally indicate no threat to one’s health; it also means that it is not “cancerous”.

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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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