Non-Peutz-Jeghers Syndrome Associated Ovarian Sex Cord Tumor With Annular Tubules: A Case Report

Objective: Sex cord tumors with annular tubules (SCTAT) are a rare subtype of sex cord stromal tumor of the ovary. An evidence-based management plan with follow-up evaluations is difficult to outline because of the rarity of these tumors. We describe the case of a premenarchal patient with a SCTAT.Design: Case report.Setting: The patient was encountered during routine patient care process.Patient(s): The patient presented with a pelvic mass and precocious puberty. Her condition was diagnosed as SCTAT. Her clinical presentation was consistent with an estrogen-secreting tumor, resulting in early menarche and premature breast development. Inhibin and estradiol levels were markedly elevated preoperatively and normalized 5 weeks after surgical removal of the tumor. The preoperative computed tomography scan demonstrated a 12-cm abdominopelvic mass, which appeared to be mostly cystic.Intervention(s): The patient was treated surgically. She underwent laparotomy, right salpingo-oophorectomy, ipsilateral pelvic and paraaortic lymph node sampling, and partial omentectomy. Peritoneal biopsy samples were obtained from the abdomen and pelvis.Main Outcome Measure(s): The patient did well postoperatively. She is being observed with serial examinations and serum inhibin measurements.Result(s): Normalization of serum estradiol and inhibin along with cessation of menstruation were seen 5 weeks postoperatively, with persistence of morphologic signs of precocious puberty and advanced bone age at 11 months after the diagnosis.Conclusion(s): The diagnosis of SCTAT was established on final pathology examination based on morphologic features of the tumor microscopically and the marker expression profile on immunohistochemistry. Primary management was surgical. (Fertil Steril (R) 2009; 92: 1497.e5-e8. (C) 2009 by American Society for Reproductive Medicine.)