Granulosa cell tumors
Granulosa cell tumors are a type of slow-growing ovarian tumor that generally have a low risk of malignancy. They predominantly occur in postmenopausal women, with most diagnoses made between the ages of fifty and fifty-five, although a small percentage can arise in younger women. These tumors often secrete estrogen, leading to symptoms such as irregular menstrual cycles, uterine bleeding, and, in children, early signs of puberty. Diagnosis typically involves physical examination, imaging, and blood tests, with the majority of cases identified in early stages when the tumor is confined to one ovary.
Treatment usually involves surgical removal of the affected ovary, which can impact fertility; however, there are options for fertility-sparing surgeries. Chemotherapy and hormone therapies may be utilized for more advanced cases. The prognosis for stage I tumors is highly favorable, with ten-year survival rates ranging from 90 to 96 percent, but these rates decrease significantly for tumors diagnosed at later stages. Understanding granulosa cell tumors is essential for early detection and effective treatment, making awareness of symptoms and risk factors important for affected individuals.
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Granulosa cell tumors
RELATED CONDITIONS: Endometrial hyperplasia, endometrial adenocarcinoma
DEFINITION: Granulosa cell tumors are slow-growing tumors almost always found in the ovaries. They have a low potential for becoming malignant. These tumors usually secrete large amounts of estrogen, although occasionally, they secrete androgens (male hormones).
Risk factors: Granulosa cell tumors are evenly distributed among women of all races and ethnicities. Most tumors occur in postmenopausal women and are diagnosed between the ages of fifty and fifty-five. Only between 5 and 12 percent are found in women under the age of thirty.
Etiology and the disease process: Researchers are unclear about how these malignancies arise. One theory is that they are related to chromosomal abnormalities, possibly related to a mutation of the FOXL2 gene. Another possible cause is a defect in hormonal regulation. These tumors grow slowly. Other causes may include: the expression of Sma and Mad-related proteins, transforming growth factor-β, telomerase mutation of reverse transcriptase promoter, and serine/threonine kinase gene activation.
Incidence: Between 1,500 and 2,000 cases are diagnosed each year in the United States. Granulosa cell tumors account for between 2 and 5percent of all tumors of the ovaries.
Symptoms: Granulosa cell tumors usually secrete estrogen. In children, this tends to cause abnormally early signs of puberty, including precocious thelarche. In reproductive-age women, it disrupts the menstrual cycle, and in postmenopausal women, the most common symptom is uterine bleeding. Other symptoms include an increase in the size of the abdomen and abdominal or pelvic pain.
Screening and diagnosis: Most women seek medical attention because of the side effects of increased estrogen. On physical examination, the physician can feel the tumor in more than 90 percent of women. The average diameter of the tumor at diagnosis is 4.7 inches (12 centimeters). They can range from 2 to 50 centimeters. Physical findings are confirmed with blood tests, imaging tests, and biopsy.
Some 90 percent of tumors are diagnosed in Stage I with malignancy confined to only one ovary. In Stage II, the tumor has begun to extend into the tissues near the ovary. In Stage III, it has metastasized to the abdomen, and in Stage IV to distant sites.
Treatment and therapy: Treatment often involves surgical removal of the affected ovary, although this can affect fertility. For stage I tumors, unilateral salpingo-oophorectomy may be performed. Postmenopausal women may also have a hysterectomy. However, fertility-sparing surgical methods have been developed. Chemotherapy and radiation treatment are used in women whose cancer has advanced beyond stage I. Hormone therapies, such as aromatase inhibitors, may also be effective.
Prognosis, prevention, and outcomes: Prognosis is excellent for tumors in stage I, with ten-year survival rates of 90 to 96 percent. The ten-year survival rate for more advanced cancers is about half this rate. Tumors diagnosed in women under age thirty tend to recur within three years, but in postmenopausal women, years or even decades may pass before recurrence.
Bibliography
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