Pure-type clear cell carcinoma of the ovary as a distinct histological type and improved survival in patients treated with paclitaxel-platinum-based chemotherapy in pure-type advanced disease

Author(s):  
C HO
2020 ◽  
pp. ijgc-2020-001656
Author(s):  
Yasushi Iida ◽  
Aikou Okamoto ◽  
Robert L Hollis ◽  
Charlie Gourley ◽  
C Simon Herrington

Clear cell carcinoma of the ovary has distinct biology and clinical behavior. There are significant geographical and racial differences in the incidence of clear cell carcinoma compared with other epithelial ovarian tumors. Patients with clear cell carcinoma are younger, tend to present at an early stage, and their tumors are commonly associated with endometriosis, which is widely accepted as a direct precursor of clear cell carcinoma and has been identified pathologically in approximately 50% of clear cell carcinoma cases. The most frequent and important specific gene alterations in clear cell carcinoma are mutations of AT-rich interaction domain 1A (ARID1A) (~50% of cases) and phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA) (~50% cases). More broadly, subgroups of clear cell carcinoma have been identified based on C-APOBEC (apolipoprotein B mRNA editing enzyme, catalytic polypeptide-like) and C-AGE (age-related) mutational signatures. Gene expression profiling shows upregulation of hepatocyte nuclear factor 1-beta (HNF1β) and oxidative stress-related genes, and has identified epithelial-like and mesenchymal-like tumor subgroups. Although the benefit of platinum-based chemotherapy is not clearly defined it remains the mainstay of first-line therapy. Patients with early-stage disease have a favorable clinical outcome but the prognosis of patients with advanced-stage or recurrent disease is poor. Alternative treatment strategies are required to improve patient outcome and the development of targeted therapies based on molecular characteristics is a promising approach. Improved specificity of the histological definition of this tumor type is helping these efforts but, due to the rarity of clear cell carcinoma, international collaboration will be essential to design appropriately powered, large-scale clinical trials.


1996 ◽  
Vol 60 (3) ◽  
pp. 412-417 ◽  
Author(s):  
Barbara A. Goff ◽  
Ricardo Sainz de la Cuesta ◽  
Howard G. Muntz ◽  
Deborah Fleischhacker ◽  
Marit Ek ◽  
...  

2021 ◽  
Vol 4 (4) ◽  
pp. 01-08
Author(s):  
Chul Kim

Background: It is well known that clear cell carcinoma of the ovary (CCC) demonstrates different clinical behaviors from other epithelial ovarian cancer and has strong association with endometriosis, thromboembolic complication, hypercalcemia, and large pelvic mass. The introduction of cisplatin-based chemotherapy significantly changed the postoperative management of ovarian cancer patients. Different studies showed a better response rate of CCC to chemotherapy with paclitaxel plus carboplatin regime than with the conventional platinum-based regimens. Aim: The purpose of this study was to evaluate the patients’ clinical characteristics and treatment results for clear cell carcinoma (CCC) of the ovary treated in paclitaxel-platinum chemotherapy in comparison with those treated in conventional platinum-based chemotherapy after primary surgery Methods: We retrospectively reviewed the medical records of 40 patients with CCC who received treatment in the department of obstetrics and gynecology, Samsung Medical Center from March, 1996 to April 2006. The clinical characteristics, treatment results and follow-up data were collected from medical records and/or telephone surveys. Results: Mean age was 47 years (range 30–72 years). Patients with age less than 50years were 62.5%. Tumors were 15% (6/40) stage IA, 2.5% (1/40) stage IB, 37.5% (15/40) stage IC, 5% (2/40) stage II, 32.5% (13/40) stage III, and 7.5% (3/40) stage IV. Patients with CCC were more likely to have FIGO stage I & II disease than FIGO stage III & IV (60% vs. 40%). Five-year progression-free survival and overall survival were 91% and 80% in stage I & II, 36% and 55% in stage III & IV, respectively (5-yr PFS; P<0.01, 5-yr OS; P=0.03). With a median follow-up of 45 months (2-112 months), 75% (18/24) of stage I/II patients are alive, while 19% (3/16) of stage III/IV patients are alive. 37.5% (15/40) of the patients presented with endometriosis. Except for one patient who was referred by a local clinic, all patients underwent cytoreductive surgery. The rate of optimal debulking (≤ 1cm residual tumor diameter) was 90% (36/40). Overall, for women treated with platinum-based chemotherapy, 75% (27/36) had clinically complete responses to adjuvant chemotherapy. But there was no survival benefit according to chemotherapeutic differences in the patients who received cytoreductive surgery followed between conventional platinum-based chemotherapy (CAP or CP) and by paclitaxel and platinum-based chemotherapy (P=0.40). Univariate analysis showed that stage was the only favorable prognostic factor for women with clear cell carcinoma of the ovary (P=0.04). Conclusions: Our results suggest that CCC has a distinct clinical behavior, similar to previous studies, that frequently presents at early- stages and is associated with endometriosis. In addition, there was a close correlation between the level of CA-125 and survival, and there was no survival benefit according to chemotherapeutic differences. 〔CAP (CP) VS TP(TC)〕


Author(s):  
Angiolo Gadducci ◽  
Francesco Multinu ◽  
Stefania Cosio ◽  
Silvestro Carinelli ◽  
Mariacristina Ghioni ◽  
...  

2016 ◽  
Vol 16 ◽  
pp. 1-4
Author(s):  
Zhen Ni Zhou ◽  
Christina Tierney ◽  
William H. Rodgers ◽  
Long Nguyen ◽  
David Fishman

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