title: [Ovarian tumors with endocrine function].
abstract: OBJECTIVE
  To analyze the clinicopathological features of ovarian tumors with endocrine function.
  
  
  METHODS
  Twenty-four cases of ovarian tumor with endocrine manifestation were collected from the hospital. Their clinical presentation and histopathologic features were reviewed, along with a panel immunohistochemistry stainings (EnVision method). The antibodies were AE1/AE3, epithelial membrane antibody (EMA), alpha-inhibin, calretini and smooth myoglobin (SMA).
  
  
  RESULTS
  The main clinical endocrinological manifestations were related to an excess production of sex steroids. Histologically, the principle histological subtype of these tumors was ovarian sex cord-stroma tumors, including 13 cases ovarian type (8 granulosa cell tumors, 2 thecofibromas, 3 sclerosing stromal tumors), 7 cases testicular type (1 sertoli cell tumors, 5 sertoli-Leydig cell tumors, 1 Leydig cell tumor, and 2 cases of steroid cell tumor (NOS). Another 2 cases were ovarian epithelial tumors. Grossly, 50% (11/22) ovarian sex cord-stromal tumors were less than 5 cm in diameter. However, 4 tumors were quite larger, up to 18 cm in diameter. Most of these tumors were solid or solid-cystic and their cut surfaces were brown, pink, yellow or grey in color. The 2 primary ovarian epithelial tumors were larger, being 12 cm and 14 cm in diameter, respectively. Immunohistochemically, ovarian sex cord-stromal tumors showed positive staining for alpha-inhibin in all cases (22/22) and for calretinin in majority cases (18/22), and that the intensity of reactivity correlated with the degree of tumor differentiation. The non-neoplastic, luteinized stromal cells in 2 ovarian primary epithelial tumors also showed positive staining. Five cases of fibrothecomas and sclerosing stroma tumors were all positive for SMA. Although 6 of the 22 ovarian sex cord-stromal tumors were AE1/AE3 positive, all were EMA negative.
  
  
  CONCLUSIONS
  Most endocrinological syndromes in ovarian neoplasia reflect an overproduction of sex steroids, but the clinical manifestations do not correlate with the tumor histological subtypes. Most functional ovarian tumors are sex cord-stromal tumors and are usually of small to medium in size, but few are larger or giant. The size of the tumor does not correlate with the duration and the degree of clinical manifestations. Ovarian tumors of non-sex cord-stromal type may also be clinically functional. The immunohistochemical results suggests that alpha-inhibin and/or calretinin expression are useful markers in support of a diagnosis of sex cord-stromal tumor of the ovary. Although some of these tumors are AE1/AE3 positive, EMA negativity may be useful for the differential diagnosis with epithelial ovarian tumors.
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China. guoln@pumch.ac.cn
authors.forename: Li-Na
authors.initials: LN
authors.lastname: Guo
authors.forename: Tong-Hua
authors.initials: TH
authors.lastname: Liu
authors.forename: Ai-Jun
authors.initials: AJ
authors.lastname: Sun
authors.forename: Quan
authors.initials: Q
authors.lastname: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D064032:Calbindin 2; D005260:Female; D012739:Gonadal Steroid Hormones / Q000378:metabolism*; D006106:Granulosa Cell Tumor / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007265:Inhibins / Q000032:analysis; D008875:Middle Aged; D010051:Ovarian Neoplasms / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology; D064030:S100 Calcium Binding Protein G / Q000032:analysis; D018310:Sertoli-Leydig Cell Tumor / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D018312:Sex Cord-Gonadal Stromal Tumors / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology
chemical_list: D014408:Biomarkers, Tumor; C576764:CALB2 protein, human; D064032:Calbindin 2; D012739:Gonadal Steroid Hormones; D064030:S100 Calcium Binding Protein G; C094377:inhibin-alpha subunit; D007265:Inhibins