title: Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report.
abstract: 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 premenarcheal patient with a SCTAT.
  
  
  METHODS
  Case report.
  
  
  METHODS
  The patient was encountered during routine patient care process.
  
  
  METHODS
  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.
  
  
  METHODS
  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.
  
  
  METHODS
  The patient did well postoperatively. She is being observed with serial examinations and serum inhibin measurements.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, UCLA Medical Center, University of California, Los Angeles, California; Department of Reproductive Medicine and Surgery, Moscow State University for Medicine and Dentistry, Moscow, Russia.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Nosov
authors.affiliation: Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, UCLA Medical Center, University of California, Los Angeles, California.
authors.forename: Susan
authors.initials: S
authors.lastname: Park
authors.affiliation: Department of Pathology, UCLA Medical Center, University of California, Los Angeles, California.
authors.forename: Jianyu
authors.initials: J
authors.lastname: Rao
authors.affiliation: Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, UCLA Medical Center, University of California, Los Angeles, California. Electronic address: smemarzadeh@mednet.ucla.edu.
authors.forename: Sanaz
authors.initials: S
authors.lastname: Memarzadeh
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D010052:Ovariectomy / Q000379:methods; D010580:Peutz-Jeghers Syndrome / Q000150:complications; D011629:Puberty, Precocious / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000601:surgery; D012489:Salpingostomy; D018312:Sex Cord-Gonadal Stromal Tumors / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery