Granulosa cell tumors (GCTs) are a rare type of ovarian cancer. They arise from specialized cells within the ovary that produce hormones. While generally having a favorable outlook, understanding their nature and management is important.
What Granulosa Cell Tumors Are
Granulosa cell tumors originate from granulosa cells, which are part of the ovarian follicle and produce hormones. They are rare, accounting for 2% to 5% of all ovarian cancers. They are also the most common type of sex cord-stromal tumor, a category of ovarian tumors that arise from the connective tissues of the ovary.
GCTs typically exhibit a slow-growing pattern, which often leads to their diagnosis at an early stage. A key characteristic is their ability to produce hormones, particularly estrogen. This hormonal activity can lead to symptoms that prompt earlier medical attention, distinguishing them from other ovarian cancers that often present with non-specific symptoms at later stages.
Factors Influencing Outcomes
While generally having a good prognosis, granulosa cell tumors can be life-threatening. Their outcome is influenced by several factors. The most important factor impacting survival is the tumor’s stage at diagnosis. Stage I tumors, confined to the ovary, have a better outlook. Approximately 90% of GCTs are diagnosed at Stage I.
For adult GCTs, the 10-year survival rate for Stage I tumors is high, ranging from 90% to 96%. However, for more advanced stages, the 5-year and 10-year survival rates decrease to about 33% to 44%. Tumor size also plays a role, with larger tumors (e.g., greater than 10-15 cm) linked to a poorer prognosis. The patient’s age at diagnosis can also influence outcomes; adult GCTs are more common in women around age 50, and juvenile GCTs occur in younger individuals.
There are two main histological subtypes: adult granulosa cell tumors (AGCTs) and juvenile granulosa cell tumors (JGCTs). AGCTs account for about 95% of cases and tend to be slow-growing. JGCTs, though rarer, can sometimes be more aggressive and have a higher likelihood of recurrence within a few years.
Approaches to Treatment
The primary treatment for granulosa cell tumors is surgical removal. The extent of surgery depends on the tumor’s stage and the patient’s age. For early-stage disease, complete surgical resection is often curative.
Chemotherapy and radiation therapy are considered for more advanced cases, recurrent disease, or when surgery alone is not sufficient. Chemotherapy often involves platinum-based drugs, such as bleomycin, etoposide, and cisplatin (BEP). However, the role of adjuvant chemotherapy (given after surgery) in early-stage GCTs is not fully established, and some studies suggest it may not significantly improve disease-free survival. Radiation therapy, such as whole abdominal radiation, may be used for chemo-refractory tumors or pelvic recurrence, and can induce clinical responses. Hormonal therapies, including aromatase inhibitors, are also being explored for managing recurrent disease.
Long-Term Surveillance
Long-term surveillance is important for individuals treated for granulosa cell tumors due to the potential for late recurrence. GCTs can recur many years after initial treatment, sometimes decades later. This requires consistent and prolonged follow-up care.
Common surveillance methods include regular physical examinations and imaging tests like CT scans to detect any signs of recurrence. Blood tests for tumor markers are particularly important. Inhibin, a hormone produced by granulosa cells, is a highly sensitive and specific tumor marker for GCTs, and elevated levels can indicate recurrence months before it is clinically apparent. Anti-Müllerian hormone (AMH) is another marker that can be used for monitoring. Consistent monitoring allows for early detection of recurrence, which can lead to more effective secondary treatment and improved long-term survival outcomes.