Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: A case report

The majority of ovarian sex cord tumors with annular tubules (SCTAT) are be nign neoplasms that arise sporadically. in patients who have Peutz-Jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding, Malignant beh avior in SCTAT has heretofore been reported only in sporadic cases. We repo rt a case of bilateral, malignant SCTAT developing in a 47-year-old woman w ho had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytolo gy. Cervicovaginal and peritoneal fluid cytologic preparations were charact erized by pseudopapillary clusters and three-dimensional tubes of tumor cel ls with scanty cytoplasm and high nuclear: cytoplasmic ratio. Examination o f surgical resection specimens revealed bilateral, solid ovarian tumors com posed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces an d in both right and left pelvic lymph nodes. Flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, pr esenting with an atypical cervicovaginal smear.