title: Treatment issues in clear cell carcinoma of the ovary: a different entity?
abstract: BACKGROUND
  Ovarian clear cell carcinoma (OCCC) is a distinct histopathologic subtype of epithelial ovarian cancer (EOC) with an incidence of <5% of all ovarian malignancies. Our goal was to review the clinical features and management of patients with OCCC.
  
  
  RESULTS
  We performed a PubMed search using the phrase "clear cell ovarian cancer." We reviewed 54 articles referring to OCCC. OCCC patients have a high incidence of stage I disease and frequently present with a large pelvic mass. Recurrences are more frequent with this entity than with other types of EOC. The clinical management of advanced EOC includes maximal cytoreduction and platinum plus paclitaxel-based chemotherapy. The survival rates of patients with advanced OCCC are lower than those of patients with advanced serous EOC (serous subtype). The poor response rate to platinum-based regimens may be related to the intrinsic chemoresistance of these tumors. Despite their aggressive clinical course, OCCCs are still treated similarly to the other EOCs at the present time, because the rarity of these tumors prevents the conduction of randomized studies.
  
  
  CONCLUSIONS
  Novel treatment approaches should be adopted in OCCC. Molecular-targeted therapies and effective new agents without cross-resistance to platinum compounds should be evaluated in a prospective clinical trial in OCCC.
authors.affiliation: Second Department of Internal Medicine, Oncology Section, University of Athens, Greece. pectasid@otenet.gr
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Pectasides
authors.forename: Eirini
authors.initials: E
authors.lastname: Pectasides
authors.forename: Amanda
authors.initials: A
authors.lastname: Psyrri
authors.forename: Theofanis
authors.initials: T
authors.lastname: Economopoulos
mesh_terms: D018262:Adenocarcinoma, Clear Cell / Q000188:drug therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D006801:Humans; D009367:Neoplasm Staging; D010051:Ovarian Neoplasms / Q000188:drug therapy*; D011446:Prospective Studies; D016032:Randomized Controlled Trials as Topic; D012008:Recurrence; D015996:Survival Rate