Clinical perspectives of rare ovarian tumors: clear cell ovarian cancer

Ovarian clear cell carcinoma (OCCC) is a rare and distinct histological type of epithelial ovarian carcinoma in terms of its histopathological, clinical and genetic features. Patients with OCCC are younger and diagnosed at earlier stages than those with the most common histological type-high-grade serous carcinoma. Endometriosis is considered a direct precursor of OCCC. Based on preclinical data, the most frequent gene alternations in OCCC are mutations of AT-rich interaction domain 1A and phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha. The prognosis of patients with early-stage OCCC is favorable, whereas patients at an advanced stage or who have the recurrent disease have a dismal prognosis due to OCCC's resistance to standard platinum-based chemotherapy. Despite a lower rate of response due to its resistance to standard platinum-based chemotherapy, the treatment strategy for OCCC resembles that of high-grade serous carcinoma, which includes aggressive cytoreductive surgery and adjuvant platinum-based chemotherapy. Alternative treatment strategies, including biological agents based on molecular characteristics specific to OCCC, are urgently needed. Furthermore, due to its rarity, well-designed collaborative international clinical trials are needed to improve oncologic outcomes and the quality of life in patients with OCCC. Ovarian clear cell carcinoma (OCCC) is a rare and poor prognosis histologic type of epithelial ovarian cancer. This review summarizes its characteristics and concludes with an overview of new therapeutic strategies for patients with OCCC.

Automated summary

Ovarian clear cell carcinoma (OCCC) is a rare and distinct type of epithelial ovarian cancer with unique histopathological, clinical, and genetic features. It is associated with younger age at diagnosis and earlier stages compared to the most common type of ovarian cancer. OCCC is considered to have endometriosis as a direct precursor, and the most frequent gene mutations in OCCC are AT-rich interaction domain 1A and phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha. Patients with early-stage OCCC have a favorable prognosis, while those at advanced stages or with recurrent disease have a poor prognosis due to the resistance of OCCC to standard platinum-based chemotherapy. Aggressive cytoreductive surgery and adjuvant platinum-based chemotherapy are the recommended treatment strategies, but alternative therapies based on OCCC's specific molecular characteristics are urgently needed. Well-designed international clinical trials are necessary to improve outcomes and quality of life for patients with OCCC.