Adult granulosa cell tumors of the ovary in two first-degree relatives

Background. Known inherited mutations are responsible for approximately 10% of all epithelial ovarian cancers; however, prior to this report, sex cord-stromal ovarian tumors had not been documented in related females. Case. We report here on a mother and daughter, each diagnosed with rare, adult-type granulosa cell tumors of the ovary. Conclusion. This novel report of granulosa cell tumors occurring in two first-degree relatives must be examined with reserve. No familial tendency has previously been documented, and our finding may be coincidental. However, if additional cases are reported and future research is undertaken, biological markers and inherited mutations for certain sex cord-stromal ovarian tumors may be identified. These advances could lead to targeted therapy and specific surveillance protocols with appropriate surgical interventions for high-risk patients.