Ovarian granulosa cell tumor

A granulosa-stromal cell tumor that arises from the ovary. it is characterized by the presence of granulosa cells that comprise at least ten percent of the cellular population. the granulosa cells are often found in a background that contains theca and fibrous cells. there are two major subtypes recognized, adult and juvenile granulosa cell tumor. clinically, patients may present with an abdominal mass. symptoms depend on the patient's age. the most important indicator of prognosis is tumor stage. age over forty years at the time of the initial diagnosis, large tumor size, bilaterality, cellular atypia, and increased mitotic activity are factors indicating a potentially aggressive clinical course and relative poor prognosis.

Other names

GCT of the ovary; Granulosa theca cell tumor; GTCT; Adult granulosa cell tumor of the ovary; Granulosa theca cell tumor of the ovary

Pathophysiology

Granulosa   of the ovary is a rare type of ovarian  that accounts for approximately 2% of all ovarian tumors. This type of tumor is known as a sex cord-stromal tumor and usually occurs in adults. Granulosa cell tumors of the ovary cause higher than normal levels of  in a woman's body.  The symptoms of this excess estrogen depend on the woman's menstrual status; the most common symptoms include early puberty for affected young girls, an increase in abdomen size or irregularities of menstrual cycles in premenopausal women, and abnormal uterine bleeding in postmenopausal women. The exact cause of granulosa cell tumors is unknown.  Treatment consists of surgery to remove the tumor, and additional treatments may also be used depending on the extent of the tumor.

Treatment

Surgery is the first step of treating a granulosa of the ovary and aims to remove as much of the tumor as possible.  Additional treatments - such as , , or  - may follow surgery depending on the severity and extent of the original tumor, or if the tumor regrows after surgery (a recurrence).  These treatments may improve survival or increase the length of disease-free time before a recurrence.  After treatments are complete, individuals should continue to have regular visits with their physicians to check for recurrences.  These visits should include updating the medical history, a pelvic examination, and possibly blood testing to look for chemicals produced by  cells ().  If there are any suspicious findings during these visits, an  test such as a  may be done

Prognosis

The  is dependent on the severity and extent (stage) of the  at the time of diagnosis. The majority of cases of granulosa  tumor of the ovary are stage I at diagnosis ( is still contained within the ovary and has not spread). Stage I granulosa cell tumors of the ovary have been found to have a good prognosis with a 5 year survival rate ranging from 90-100% and a 10 year survival rate ranging from 84-95%.

Juvenile granulosa cell tumors

Incidence

The most common histologic subtype in girls younger than 18 years is juvenile granulosa cell tumors (median age, 7.6 years; range, birth to 17.5 years). Juvenile granulosa cell tumors represent about 5% of ovarian tumors in children and adolescents and are distinct from the granulosa cell tumors seen in adults.

Risk Factors

Juvenile granulosa cell tumors have been reported in children with Ollier disease and Maffucci syndrome.

Clinical Presentation

Patients with juvenile granulosa cell tumors present with the following symptoms:

• Precocious puberty (most common; caused by estrogen secretion).
• Abdominal pain.
• Abdominal mass.
• Ascites.

Treatment of Childhood Juvenile Granulosa Cell Tumors

Treatment options for childhood juvenile granulosa cell tumors include the following:

1. Surgery. As many as 90% of children with juvenile granulosa cell tumors will have low-stage disease (stage I) by International Federation of Gynecology and Obstetrics (FIGO) criteria and are usually curable with unilateral salpingo-oophorectomy alone.
2. Chemotherapy. Patients with spontaneous tumor rupture or malignant ascites (FIGO stage IC2, IC3), advanced disease (FIGO stages II–IV), and those with high mitotic activity tumors have a poorer prognosis and require chemotherapy. Use of a cisplatin-based chemotherapy regimen has been reported in both the adjuvant and recurrent disease settings with some success.

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Classification	ICD-10: C56 MeSH: D006106
External resources	eMedicine: med/928