Ovarian Sertoli-Leydig Cell Tumor

Ovarian Sertoli-Leydig Cell Tumor

Article
Women's Health
Diseases & Conditions
+1
Contributed byKrish Tangella MD, MBADec 14, 2018

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

  • Androma
  • Arrhenoblastoma
  • Sertoli-Leydig Cell Tumor of Ovary

What is Ovarian Sertoli-Leydig Cell Tumor? (Definition/Background Information)

  • Sertoli-Leydig Cell Tumor of Ovary is a rare, ovarian tumor arising from the sex cord cells (hormone-producing cells) present in the ovary. These tumors produce the male sex hormone (androgen), and rarely, the female sex hormone (estrogen)
  • Ovarian Sertoli-Leydig Cell Tumor commonly affects young adult females. The exact cause of the condition is not well known. The tumor produces the male sex hormone (such as testosterone) resulting in symptoms of masculinization (having qualities of a male) and cessation of menstruation
  • A diagnosis of the tumor is made by imaging studies, such as ultrasound, CT and MRI scan of the pelvis, along with histological examination of the tumor under a microscope, after its surgical removal
  • Surgical excision of the tumor is the treatment of choice. Most of the Ovarian Sertoli-Leydig Cell Tumors are benign with good prognosis
  • A few such tumors may become malignant with the potential to spread to other body parts, and in these cases, the outcome may be guarded. Malignant tumors also require additional treatment by radiation and chemotherapy

Who gets Ovarian Sertoli-Leydig Cell Tumor? (Age and Sex Distribution)

  • Ovarian Sertoli-Leydig Cell Tumors accounts for less than 0.5-1% of all ovarian tumors
  • The condition typically affects females between the ages of 20-30 years age; though it can occur at any age, including in toddlers and postmenopausal women
  • All racial and ethnic groups are affected; the condition is observed worldwide

What are the Risk Factors for Ovarian Sertoli-Leydig Cell Tumor? (Predisposing Factors)

The risk factors for Ovarian Sertoli-Leydig Cell Tumor may include:

  • Young adult women are more at risk
  • Family history of the condition
  • A history of certain cancers, such as kidney tumors, cervical cancer, and certain lung cancer types
  • Goiter affecting the thyroid gland

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 Ovarian Sertoli-Leydig Cell Tumor? (Etiology) 

  • The exact cause of Ovarian Sertoli-Leydig Cell Tumor is not known
  • Research studies seem to indicate that certain genetic mutations (in the DICER1 gene) may play a role in many cases

What are the Signs and Symptoms of Ovarian Sertoli-Leydig Cell Tumor?

Ovarian Sertoli-Leydig Cell Tumor produces the male sex hormone, such as testosterone and androgen, and it produces the following signs and symptoms:

  • Masculinization (development of male physical characteristics) that include deepening of the voice and increased facial hair (hirsutism), which may be progressive in nature
  • Irregularities of menstrual cycles or complete cessation of cycle
  • Acne
  • Enlarged clitoris
  • Abdominal mass or pain
  • Increased muscle tone
  • Hairline recession
  • Loss of appetite with weight loss
  • Fatigue
  • Frequent urination, difficulty while urinating
  • Frequent pain during sex

How is Ovarian Sertoli-Leydig Cell Tumor Diagnosed?

The diagnosis of Ovarian Sertoli-Leydig Cell Tumor may involve:

  • Complete evaluation of medical history and a thorough physical exam
  • A pelvic ultrasound is the best imaging modality for diagnosis of the condition
  • Magnetic resonance imaging (MRI) scan of the pelvis
  • Computerized tomography scan (CT or CAT scan) of the pelvis
  • Blood test for hormone levels: Testosterone, dehydroepiandrosterone (DHEA) and progesterone
  • Serum tumor markers (substances whose level increases in blood, when this type of tumor is growing in the body), such as serum inhibin and alpha fetoprotein (AFP)
  • Exploratory laparoscopy: It is a procedure wherein the abdomen is examined using a minimally invasive technique. During this procedure, a tissue biopsy and tissue for culture is taken. A minimally-invasive approach helps decrease complications and also the length of stay at hospital. A diagnostic laparoscopy is also helpful in establishing the stage of the tumor
  • Colonoscopy: A colonoscopy may be performed to assess the extent of tumor spread
  • A tissue biopsy of the tumor: A tissue biopsy 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. Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing and very rarely electron microscopic studies. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive 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 Ovarian Sertoli-Leydig Cell Tumor?

Complications of Ovarian Sertoli-Leydig Cell Tumor may include:

  • Precocious puberty (unusually early onset of puberty) will result in short stature (height) and early menarche (onset of menstruation)
  • Deep-seated tumors may create problems for adjoining tissues and organs by compressing them
  • Metastasis of tumor to different parts of the body such as to the bones, lungs, and liver
  • Side effects from chemotherapy (toxicity), radiation therapy

How is Ovarian Sertoli-Leydig Cell Tumor Treated?

Treatment of Ovarian Sertoli-Leydig Cell Tumor may include:

  • If the tumor is unilateral (present on one side) and benign, then unilateral salpingo-oophorectomy (removal of the fallopian tube and ovary on one side) is performed
  • After removal of the tumor, the levels of serum tumor markers are regularly checked to detect re-growth of the tumor
  • Malignant tumors (tumors with potential to spread to other parts of the body) need additional treatment by radiation and chemotherapy

How can Ovarian Sertoli-Leydig Cell Tumor be Prevented?

Currently, there are no effective measures to prevent the occurrence of Sertoli-Leydig Cell Tumor of Ovary. However, the following factors may be considered:

  • Early diagnosis with close monitoring, and treatment of the tumor is important
  • Prompt treatment and early tumor recognition will help in having an optimal outcome
  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child

What is the Prognosis of Ovarian Sertoli-Leydig Cell Tumor? (Outcomes/Resolutions)

  • Most of the Ovarian Sertoli-Leydig Cell Tumor are benign with good prognosis
  • Few Sertoli-Leydig cell tumors are malignant with potential to spread to other body parts; in such cases, the outcome may not be predictable and depends upon various factors including stage of the tumor

Additional and Relevant Useful Information for Ovarian Sertoli-Leydig Cell Tumor:

  • Ovarian dysgerminoma is a rare malignant ovarian tumor, which accounts for 2% of all ovarian tumors. It is a rapidly growing tumor that occurs mostly as a single solid mass

The following article link will help you understand ovarian dysgerminoma:

http://www.dovemed.com/diseases-conditions/ovarian-dysgerminoma/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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