title: Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study.
abstract: BACKGROUND
  Owing to the rarity of sex cord tumor with annular tubules (SCTAT), it is difficult to recognize SCTAT clinically and there is no standard treatment. The aim of our study was to investigate the treatment outcomes and prognosis of patients with ovarian SCTAT.
  
  
  METHODS
  A cohort of 13 patients with SCTAT diagnosed and treated in Peking Union Medical College Hospital was studied. Data on clinicopathological characteristics, treatment, and prognosis were retrospectively reviewed and analyzed.
  
  
  RESULTS
  SCTAT accounted for 1.4% of ovarian sex cord stromal tumors, with an average onset age of 22.6 years. All patients presented with menstrual disturbances or isosexual precocity at disease onset. Initial surgery was unilateral salpingo-oophorectomy in 11 cases. Recurrence rate was 46.2%, and 38.5% of patients experienced multiple recurrences. The disease free interval gradually shortened with increasing numbers of recurrences. Recurrent tumors were mostly ipsilateral to the primary tumor and located in retroperitoneum. Surgery remained the main treatment for recurrent cases. Serum estradiol and progesterone levels usually elevated at disease onset, decreased dramatically after operation, and they elevated again with the development of recurrence. The median progression-free survival (PFS) was 97.8 months, and the 1-year and 5-year PFS were 92% and 67%, respectively. Five-year overall survival (OS) was 100%.
  
  
  CONCLUSIONS
  Unilateral salpingo-oophorectomy is a feasible treatment for primary SCTAT cases with intact capsules and without PJS. Complete tumor resection is suggested for recurrent cases and long-term follow-up is strongly recommended. Despite the high risk of recurrence, SCTAT prognosis is relatively favorable.
authors.affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, P R China. qianqiuhong99@sina.com.
authors.forename: Qiuhong
authors.initials: Q
authors.lastname: Qian
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, P R China. pumchyy@sina.com.
authors.forename: Yan
authors.initials: Y
authors.lastname: You
authors.affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, P R China. yangjiaxin007@hotmail.com.
authors.forename: Jiaxin
authors.initials: J
authors.lastname: Yang
authors.affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, P R China. caodongyan@pumch.cn.
authors.forename: Dongyan
authors.initials: D
authors.lastname: Cao
authors.affiliation: Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, P R China. zhuzhh@pumch.cn.
authors.forename: Zhaohui
authors.initials: Z
authors.lastname: Zhu
authors.affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, P R China. wuming@pumch.cn.
authors.forename: Ming
authors.initials: M
authors.lastname: Wu
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, P R China. cjpumch@sina.com.
authors.forename: Jie
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, P R China. langjh@hotmail.com.
authors.forename: Jinghe
authors.initials: J
authors.lastname: Lang
authors.affiliation: Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, P R China. shenkeng@vip.sina.com.
authors.forename: Keng
authors.initials: K
authors.lastname: Shen
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery*; D010051:Ovarian Neoplasms / Q000473:pathology / Q000601:surgery*; D010052:Ovariectomy; D010580:Peutz-Jeghers Syndrome / Q000473:pathology / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D018312:Sex Cord-Gonadal Stromal Tumors / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome