title: Sertoli - Leydig cell tumor with retiform areas and overgrowth of rhabdomyosarcomatous elements: case report and literature review.
abstract: BACKGROUND
  Sertoli - Leydig cell tumors (SLCTs) are sex-cord stromal tumors that account less than 0.5 % of primary ovarian neoplasms. They are mostly benign and occur in reproductive age women. Variants with heterologous mesenchymal elements are exceptionaly rare. The usual presentation of SLCTs is with signs of androgen excess as majority of them produce androgens.
  
  
  METHODS
  We present a case of a SLCT occurring in a 70 year old woman. Her presenting complaint was abdominal distension and pain. She had no signs of androgen or estrogen excess. Transvaginal ultrasound (TVUS) and CT scan showed a multilocular adnexal tumor and level of CA 125 was raised. A complete cytoreduction was achieved with surgical procedure. Histopathological examination revealed moderately differentiated SLCT with retiform areas and owergrowth of heterologous component in form of embrional rhabdomyosarcoma (RMS). She returned 7 months after the surgery with a large abdominal mass, ascites, right- sided hydronephrosis and massive pulmonary embolism. Due to the widespread disease and her poor general condition, she received only palliative care. She died 15 days after the admission. No autopsy was performed.
  
  
  CONCLUSIONS
  Due to the rarity of SLCTs, especially those with retiform areas and heterologous elements, their management remains challenging. There is no firm evidence that adjuvant chemotherapy is effective in improving survival in SLCTs with malignant heterologous elements. Further studies with a higher number of cases and a longer follow-up are needed to better predicting the prognosis and determine the role of chemotherapy in such cases.
authors.affiliation: Division of Obstetrics and Gynecology, University Medical Centre Ljubljana, Slajmerjeva 3, 1000, Ljubljana, Slovenia.|Department of Obstetrics and Gynecology, University Medical Centre Ljubljana, Slajmerjeva 3, 1000, Ljubljana, Slovenia.
authors.forename: T
authors.initials: T
authors.lastname: Burnik Papler
authors.affiliation: Department of Gynecological Pathology, Division of Obstetrics and Gynecology, University Medical Center Ljubljana, Ljubljana, Slovenia.|Department of Gynecological Pathology, University Medical Centre Ljubljana, Slajmerjeva 2, 1000, Ljubljana, Slovenia.
authors.forename: S
authors.initials: S
authors.lastname: Frković Grazio
authors.affiliation: Division of Obstetrics and Gynecology, University Medical Centre Ljubljana, Slajmerjeva 3, 1000, Ljubljana, Slovenia. borut.kobal1@siol.net.
authors.forename: B
authors.initials: B
authors.lastname: Kobal
mesh_terms: D000368:Aged; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D011379:Prognosis; D018233:Rhabdomyosarcoma, Embryonal / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D018310:Sertoli-Leydig Cell Tumor / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery
keywords: Postmenopausal woman; Retiform; Rhabdomyosarcoma; Sertoli - Leydig cell tumor