Abstract

Objectives: High-grade serous carcinoma (HGSC) generally presents at an advanced stage with poor overall survival. However, the clinical features of long-term (LT; >10 years) survivors have not been well characterized. Methods: A multicenter research consortium was established among 5 participating academic centers in the United States. Patient selection criteria included a diagnosis of stage III/IV high-grade serous ovarian, fallopian tube, or primary peritoneal carcinoma with at least 10 years of follow-up from the date of initial diagnosis. Nonserous, borderline tumors and low-grade serous subtypes were excluded. Results: A total of 203 LT survivors with HGSC were identified. Median age at diagnosis was 57 years (range 37–84 years). Most patients had stage IIIC (72.4%) disease at presentation. Of those who underwent primary cytoreductive surgery, optimal cytoreduction (residual disease of no more than 1 cm in maximal diameter) was achieved in 143 (85.6%) patients, and 46.6% of all patients had a complete gross resection. After a median follow-up of 144 months, 88 (46.8%) patients did not develop recurrent disease. Twenty-one patients (11.2%) had 1 recurrence during the follow-up interval, 19 patients (10.1%) had 2 recurrences, and 60 patients (31.9%) had more than 2 recurrences. Of the 79 patients tested for BRCA1 and BRCA2 germline mutations, 43 (54.4%) carried a deleterious mutation. Of note, 24 (14%) patients had suboptimal cytoreductive surgery, 16 (11%) had a platinum-free interval of less than 12 months, and 100 (53%) with recurrent disease were surviving 10 or more years after diagnosis. Conclusions: Long-term survivors of advanced HGSC generally have clinical features including optimal surgical cytoreduction and platinum-sensitive disease. The majority of these patients will develop recurrent disease with many being treated with multiple therapeutic regimens. Future work will compare the clinical features of this LT survivor cohort of “exceptional” responders with the characteristics of HGSC patients with less favorable outcomes.

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