Granulosa cell tumor

Descripción general

Granulosa cell tumor is a rare type of ovarian cancer that starts in the granulosa cells that surround eggs in the ovary. The cancer can spread to other parts of the body, which is why it's called a malignant tumor. However, most granulosa cell tumors are found before they spread outside the ovary, and many grow slowly.

Granulosa cell tumors can be serious and, in some cases, life-threatening. However, most granulosa cell tumors are found at an early stage, when outcomes are much better.

Granulosa cell tumors also can form in testicles, also called testes. This is extremely rare. When they form in the testes, granulosa cell tumors usually are not cancerous.

Granulosa cell tumors are a type of sex cord-stromal tumor. This means they start in ovary or testicle tissues that make hormones or that support the ovary or testicles.

Types

There are two main types of granulosa cell tumor — adult-type and juvenile-type. The names do not reflect the age of the person with the tumor. Instead, the names describe how the tumor cells look under a microscope and how they behave.

• Adult-type granulosa cell tumor. This most common type makes up about 95% of granulosa cell tumors. It most often happens around or after menopause.
• Juvenile-type granulosa cell tumor. This much less common type most often happens in children, teenagers and young adults. But it also can happen in older adults.

Granulosa cell tumors are different from Sertoli-Leydig cell tumors. Both are sex cord-stromal tumors. Both can make hormones, including estrogen and androgens such as testosterone. But granulosa cell tumors more often make estrogen. Sertoli-Leydig cell tumors more often make androgens.

Síntomas

Granulosa cell tumor symptoms can be caused by the tumor itself. Symptoms also can be caused by hormones the tumor makes.

Granulosa cell tumor symptoms caused by the tumor itself may include:

• Pain in the belly.
• Swelling or a feeling of fullness in the belly.

Granulosa cell tumor symptoms caused by hormones made by the tumor may include:

• Breast tenderness.
• Changes in menstrual bleeding, such as missed periods, heavy periods or bleeding after menopause.
• Early puberty, such as breast growth or vaginal bleeding.
• Increased hair on the face or body, acne, or a deeper voice, though these symptoms are rare.

Causas

The exact cause of granulosa cell tumors is not known. A change in a gene called FOXL2 may play a role in many adult-type granulosa cell tumors. This gene change, also called a FOXL2 mutation, is found in the tumor cells. It seems to play an important part in how adult-type granulosa cell tumors grow. It is not an inherited gene change.

Juvenile-type granulosa cell tumors usually do not have the FOXL2 gene change. Juvenile-type granulosa cell tumors can have other gene changes. But no single inherited gene change is known to cause either type of granulosa cell tumor.

Factores de riesgo

There are no definite risk factors for most granulosa cell tumors. Some research shows that adult-type granulosa cell tumors may be more common in people with a family history of breast or ovarian cancer.

Some juvenile-type granulosa cell tumors happen in children with Ollier disease and Maffucci syndrome.

However, more research is needed to understand these possible links.

Complicaciones

Granulosa cell tumors can sometimes cause complications.

Possible complications from the granulosa cell tumor, itself, include:

• Fluid buildup in the belly. This is called ascites.
• Twisting of the ovary. This is called torsion.
• Bleeding inside the tumor.
• Bleeding inside the belly. The tumor can break open and cause bleeding in the belly.

Adult-type granulosa cell tumors can make extra estrogen. Possible complications from the extra estrogen made by granulosa cell tumors include:

• The lining of the uterus may grow too much. This is called endometrial hyperplasia, which can increase the risk of endometrial cancer.
• Cancer may form in the lining of the uterus. This is called endometrial cancer.