What is the prognosis of granulosa cell tumors (GCTs)?

Updated: Aug 30, 2018
  • Author: David C Starks, MD, MPH; Chief Editor: Warner K Huh, MD  more...
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Answer

The prognosis for granulosa-theca cell tumors generally is very favorable. GCTs are considered to be tumors of low malignant potential. Approximately 90% of GCTs are at stage I at the time of diagnosis (see Staging). 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 AGCTs or JGCTs are 90% and 95-97%, respectively. The 10-year survival rate for AGCTs is approximately 76%. In contrast, the overall 5-year survival rate for patients with epithelial ovarian cancer is only 30-50% because only one quarter of patients present with stage I disease.

Recurrences in patients with AGCTs tend to be later than in patients with JGCTs. Mom et al reported a recurrence rate of 43% in 30 patients with stage I-III GCT observed over 10 years. Mom states that while the recurrence rate might be high, it falls within the range of 9-39% cited in other studies. [30] Average recurrence for the adult type is approximately 5 years after treatment, with more than half of these occurring more than 5 years after primary treatment. These tumors tend to follow an indolent course, with a mean survival of 5 years after the recurrence is diagnosed. The 10-year overall survival after an AGCT recurrence is in the 50-60% range.

More recently, van Meurs et al developed a prognostic model for the prediction of recurrence-free survival using BMI, clinical stage, tumor diameter, and mitotic index. [31] This model may provide useful information in the surveillance of AGCTs.

JGCTs recur much sooner, with more than 90% of recurrences occurring in the first 2 years. Recurrence in these patients is rapidly fatal.

Tumor stage at the time of initial surgery is the most important prognostic variable. Besides stage, Zhang et al found early stage disease and age younger than 50 years to be statistically significant factors in predicting survival. Other features 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. The presence of bizarre nuclei and tumor rupture intraoperatively does not appear to affect prognosis. [32] A study by Bryk et al reported tumor rupture as the strongest predictive factor for AGCT recurrence. [33]

 

Approximately 10% of tumors occur during pregnancy, but this does not affect prognosis. Perform surgical treatment for an adnexal mass in pregnancy early in the second trimester in order to minimize surgical and pregnancy risks.

True thecomas have an excellent prognosis, with 5-year survival rates of nearly 100%. However, their estrogen-producing capabilities may cause increased overall morbidity due to endometrial hyperplasia, endometrial adenocarcinoma, and possibly, breast carcinoma.


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