Is it time to abandon staging surgery and prolonged follow-up in patients with primary adult-type granulosa cell tumor?

BACKGROUND: As current literature does not provide sufficient data to support clear guidelines in patients with a rare adult-type granulosa cell tumor, we aim to investigate: (1) whether additional staging surgery following primary surgical treatment is necessary; (2) how long standard follow-up should be and (3) risk factors for disease recurrence.

METHODS: A national multicenter prospective study was initiated in April 2018. Patients with suspected or confirmed adult-type granulosa cell tumor were eligible. Data on staging, follow-up and risk factors were both retrospectively and prospectively collected from medical records, and patients were followed until April 2024 or until death. Descriptive statistical analysis and survival analysis were performed using Cox regression methods and Kaplan-Meier analyses.

RESULTS: In total, 208 patients with histopathologically confirmed adult-type granulosa cell tumor were included, with a median follow-up of 5.5 years (IQR: 2.2-12.3 years). Vaginal bleeding and abdominal pain were the most common symptoms at diagnosis. Median time until first recurrence was 4.2 years (range 2 months- 32 years). Additional staging surgery did not reduce the risk of recurrence. During follow-up, most patients had no symptoms at the time of detection of recurrence. No difference in overall survival was found between patients who were diagnosed with a recurrence during follow-up, and those who were no longer in follow-up and presented with symptoms.

CONCLUSIONS: Staging surgery does not improve recurrence free survival in patients with adult-type granulosa cell tumor. Our results suggest that adult-type granulosa cell tumor patients can be discharged from follow-up of adult-type granulosa cell tumor after five years.