Ovarian Clear Cell Carcinoma (OCCC) is distinct histopathologically and clinically from the other EOC subtypes. This tumor has a high incidence at stage I and usually present as a large pelvic mass, rarely exist bilaterally, associated with endometriosis, hypercalcemia as well as thromboembolic vascular complications. While the incidence of OCCC is not high and accounts 3.7-12.1% of all EOC, patients with OCCC have a poorer prognosis and higher recurrences than patients with other EOC subtypes. However, the survival rates of OCCC patients are much lower than other advanced EOC such as endometrial subtype. The poor response of OCCC to platinum-based regimens may be due to the intrinsic chemo-resistance. Therefore, novel treatment approaches such as molecular-targeted therapies plus more effective combinations of new chemotherapeutic agents should be investigated in a prospective clinical trial in OCCC.
Last date updated on September, 2014