Demystifying the Rare GranulosaStromal Cell Tumor: Causes, Diagnosis, and Treatment
– Granulosa-theca cell tumors (GCTs) are tumors of low malignant potential.
– Approximately 90% of GCTs are at stage I at the time of diagnosis.
– The 10-year survival rate for stage I tumors in adults is 90-96%.
– GCTs of more advanced stages are associated with 5- and 10-year survival rates of 33-44%.
– The overall 5-year survival rates for patients with adult-type granulosa cell tumors (AGCTs) or juvenile-type granulosa cell tumors (JGCTs) are 90% and 95-97%, respectively.
– The 10-year survival rate for AGCTs is approximately 76%.
– Recurrence rate for AGCTs is 43% in stage I-III patients observed over 10 years.
– Average recurrence for AGCTs is approximately 5 years after treatment, with more than half occurring more than 5 years after primary treatment.
– Mean survival after AGCT recurrence is 5 years.
– The 10-year overall survival after an AGCT recurrence is in the 50-60% range.
– JGCTs recur much sooner, with more than 90% of recurrences occurring in the first 2 years.
– Tumor stage at the time of initial surgery is the most important prognostic variable.
– Other factors associated with a poorer prognosis include high mitotic rates, moderate-to-severe atypia, preoperative spontaneous rupture of the capsule, and tumors larger than 15 cm.
– True thecomas have a 5-year survival rate of nearly 100%.
– 10% of GCTs occur during pregnancy, but this does not affect prognosis.
– AGCTs and JGCTs have very good cure rates due to early diagnosis.
– More than 90% of AGCTs and JGCTs are diagnosed before spread outside the ovary.
– Advanced-stage granulosa-stromal cell tumors (AGCTs) have a 25-50% 5-year survival rate.
– AGCTs can recur as late as 37 years after diagnosis.
– Mean survival after recurrence is 5 years.
– Approximately 20% of patients diagnosed with GCTs die from the disease.
– Morbidity is primarily due to endocrine manifestations of the disease.
– Removal of the tumor often regresses physical changes caused by high estrogen levels.
– Some patients may present with symptoms of androgen excess, which may partially regress over time.
– Unopposed estrogen production by these tumors can cause stimulation of the endometrium.
– 30-50% of patients develop endometrial hyperplasia and 8-33% have endometrial adenocarcinoma.
– Patients may be at an increased risk for breast cancer, although a direct correlation is difficult to prove.
– 10-15% of cases may have acute abdominal symptoms due to rupture, hemorrhage, or torsion.
– Adverse effects from chemotherapy are expected but generally well tolerated.
– Granulosa cell tumors (GCTs) are a type of ovarian tumor and the most common type of sex cord-stromal tumor in people assigned female at birth.
– Most GCTs are malignant (cancerous), but they tend to grow slowly and are often diagnosed in early stages with good treatment outcomes.
– Adult GCTs are more common, with about 95% of diagnoses in mature adult women.
– Juvenile GCTs occur in individuals under 30 and have a higher likelihood of recurring within a few years and being more aggressive if diagnosed after spreading outside the ovary.