Clinical Characteristics of Clear Cell Ovarian Cancer: A Retrospective Multicenter Experience of 308 Patients in South Korea

Abstract

The purpose of this study was to evaluate clinical characteristics and treatment pattern of ovarian clear cell carcinoma (OCCC) in Korea and the role of adjuvant chemotherapy in early stage. Materials and Methods Medical records of 308 cases of from 21 institutions were reviewed and data including age, performance status, endometriosis, thromboembolism, stage, cancer antigen 125, treatment, recurrence, and death were collected. Results Regarding stage of OCCC, it was stage I in 194 (63.6%), stage II in 34 (11.1%), stage III in 66 (21.6%), and stage IV in 11 (3.6%) patients. All patients underwent surgery. Optimal surgery (residual disease  1 cm) was achieved in 89.3%. Majority of patients (80.5%) received postoperative chemotherapy. The most common regimen was taxane-platinum combination (96%). Median relapse-free survival (RFS) was 138.5 months for stage I, 33.4 for stage II, 19.3 for stage III, and 9.7 for stage IV. Median overall survival (OS) were not reached, 112.4, 48.7, and 18.3 months for stage I, II, III, and IV, respectively. Early-stage (stage I), endometriosis, and optimal debulking were identified as favorable prognostic factors for RFS. Early-stage and optimal debulking were also favorable prognostic factors for OS. Majority of patients with early-stage received adjuvant chemotherapy. However, additional survival benefit was not found in terms of recurrence. Conclusion Majority of patients had early-stage and received postoperative chemotherapy regardless of stage. Early-stage and optimal debulking were identified as favorable prognostic factors. In stage IA or IB, adding adjuvant chemotherapy did not show difference in survival. Further study focusing on OCCC is required.

Clear cell carcinoma of the ovary: a clinical and molecular perspective

“…10 Clear cell carcinoma is often associated with endometriosis, 11 12 and the presence of endometriosis has been associated with a good prognosis. 13 Although the molecular mechanisms underlying malignant transformation have not been elucidated fully, endometriosis, particularly ovarian endometriosis, is widely accepted as a direct precursor of endometrioid and clear cell carcinomas of the ovary. 14 15 This is supported by data showing that women with histologically proven endometriosis have a significantly elevated age-adjusted incidence rate ratio of 2.29 (95% CI 1.24 to 4.20) for clear cell carcinoma 16 and by the identification of endometriosis in the final pathology report in 51% of cases of clear cell carcinoma.…”

Updates of Pathogenesis, Diagnostic and Therapeutic Perspectives for Ovarian Clear Cell Carcinoma

“…OCCC exhibits increased activity in several signaling pathways that may drive cancer: cell cycle regulation, survival, anti-apoptosis, chemoresistance, metabolism, coagulation, and angiogenesis 7 . Based on the genomic alteration characteristics, it is possible to find some potential sources of diagnostic and prognostic biomarkers for OCCC, including hepatocyte nuclear factor 1β (HNF1β), expressed in almost all cases of OCCC without specific correlation with FIGO stage and is now used as a diagnostic marker to predict ovarian histological subtypes 33 , 99 .…”

“…Patients usually present with a large, unilateral pelvic mass, and occasionally have thromboembolic vascular complications or hypercalcemia 4 , 5 . The 5-year overall survival (OS) and progression-free survival (PFS) rates are 80%~89% and 56%~88% for International Federation of Gynecology and Obstetrics (FIGO) stages I and II and decrease to 52% and 25% for stages III and IV, respectively 6 , 7 . OCCC patients at late stage tended to have poorer prognosis than those with other histological subtypes of EOC, especially in advanced EOC stages with 1.65-fold higher hazard rate for death 8 .…”

Development and Validation of Nomograms for Predicting Overall Survival and Cancer-Specific Survival in Patients with Ovarian Clear Cell Carcinoma

“…Patients with early-stage OCCC generally exhibit favorable prognosis, while those in advanced stage present worse survival outcomes than the high-grade serous group [3]. In addition to stage, other factors were proposed to exert in uences on the prognosis of OCCC, such as the present of endometriosis, surgical methods and venous thromboembolism [4,5]. Demographic characteristics, tumor size, lymph node status and treatment strategies are also important when evaluating survival outcomes.…”