Adult granulosa cell tumor (GCT) of the ovary is oftentimes a hormonally active, stromal cell neoplasm that is distinguished by its ability to secrete sex steroids such as estrogen. Patients may present with vaginal bleeding caused by endometrial hyperplasia or uterine cancer as a result of prolonged exposure to tumor-derived estrogen. In addition, GCT is a vascular tumor that may occasionally rupture and result in abdominal pain, hemoperitoneum, and hypotension, mimicking an ectopic pregnancy in younger patients. GCT is usually associated with a mass on pelvic examination that is subsequently confirmed on ultrasonography. Surgery is required for definitive tissue diagnosis, staging, and tumor debulking. In older women, a total abdominal hysterectomy and bilateral salpingooophorectomy are typically performed. In women of childbearing age, a more conservative unilateral salpingo-oophorectomy may be performed, assuming that careful staging reveals that the disease has not extended outside of the involved ovary and that a concomitant uterine cancer has been excluded. Survival of patients with GCT is generally excellent because most patients present with early-stage disease, although certain high-risk patient groups may be identified. Stage is the most important prognostic factor, with a higher risk of relapse being associated with stages II through IV disease. In addition, patients with stage I disease associated with features such as large tumor size, high mitotic index, or tumor rupture may also be at higher risk in some series. The value of postoperative adjuvant therapy for high-risk patients has not been investigated by prospective randomized trials, which are difficult to perform because of the rarity of this tumor. Nonetheless, the use of adjuvant chemotherapy or radiation has sometimes been associated with prolonged disease-free survival in patients with high-risk features. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance with serial physical examination and serum tumor markers such as estradiol and inhibin is reasonable.

Granulosa Cell Tumor of the Ovary

Purpose To describe the clinical management of ovarian stromal cell tumors, which are a heterogeneous group of neoplasms that develop from the sex cords and the ovarian stroma. Design We reviewed the current evidence on the clinical management of these relatively rare ovarian malignancies, which are typically detected at an early stage and may recur as late as 30 years following the initial treatment. The overall prognosis is favorable with a long-term survival ranging from 75% to 90% for all stages. Adult granulosa cell tumor (GCT) is the most common malignancy among these tumors. Results Surgery is the cornerstone of initial treatment. In women of childbearing age and with disease limited to one ovary, a fertility-sparing surgery can be a reasonable approach. Tumor stage represents the most important clinical parameter of prognostic relevance. The value of postoperative adjuvant therapy for high-risk patients has not been proven by prospective randomized studies. Platinum-based chemotherapy is used curr...

Management of Ovarian Stromal Cell Tumors

Granulosa cell tumor of the ovary

Granulosa cell tumors of the ovary (GCT) comprise a distinct subset of ovarian cancers that account for approximately 5% of all ovarian malignancies. They are thought to arise from normal proliferating granulosa cells of the late preovulatory follicle and exhibit many morphological and biochemical features of these cells. GCT are distinct from other ovarian carcinomas in their hormonal activity; their ability to secrete estrogen, inhibin, and Mullerian inhibiting substance accounts for some of the clinical manifestations of the disease and also provides useful tumor markers for disease surveillance. Although considered to be of low malignant potential, GCT are commonly associated with slow, indolent disease progression, and frequent yet long delays to tumor recurrence are characteristic of this disease. Unlike the more intensely investigated epithelial ovarian tumors, relatively little is known about the molecular and genetic changes that give rise to GCT. To date, many investigations have centered around...

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Molecular Pathogenesis of Granulosa Cell Tumors of the Ovary

Background: Granulosa cell tumor of the ovary is an uncommon neoplasm. The majority of patients are diagnosed in early stages of disease and overall prognosis is favorable. The stage at time of diagnosis is the only prognostic factor that is unequivocally related to survival. Other prognostic factors have not been well defined and are discussed in the literature controversially. Materials and Methods: In a multi-institutional retrospective study we analyzed all relevant clinical data of patients with histologically proven granulosa cell tumor of the ovary. We applied the Kaplan-Meier method in order to estimate overall survival rates and evaluate prognostic factors. Results: The median follow-up was 75 months (range, 6-315 months). Overall survival was 87% and 76% after 5 and 10 years, respectively. Eighty percent of granulosa cell tumors were diagnosed stage I (FIGO). The survival rate after recurrence was 56.8% after 10 years. Mitotic rate (p=0.003), tumor stage (p<0.001) and residual tumor disease (p<0.001) were associated with a poor prognosis (p<0.001). Age and rupture of the tumor could not be confirmed to be of prognostic value. Conclusion: The results of our study showed that the mitotic index may be a valuable prognostic factor. Complete tumor resection should always be attempted, since residual tumor disease is associated with a poor prognosis. Prospective studies are needed in order to confirm our findings. Rokitansky (1859) was the first to describe granulosa cell tumor of the ovary as a single tumor entity (1). The estimated incidence is between 0.5-1.5/100,000 per year (2-4). Granulosa cell tumors can occur at any age, but are more common after menopause and comprise 2-3% of all ovarian malignancies. Hormonal secretion is characteristic of these tumors and can lead to meno- and metrorrhagia, postmenopausal bleeding or amenorrhea. A continuous growth of the tumor can cause in other cases non-specific, abdominal symptoms (5).

Kurt P. Fine

Papers

Recurrent Granulosa Cell Tumor of the Ovary 37 Years after Initial Diagnosis: A Case Report and Review of the Literature