Objective:Endometrium clear cell carcinoma(ECCC)is rare and prone to misdiagnosis or missed diagnosis.This study aims to explore its clinicopathological characteristics to improve diagnostic accuracy.Methods:A retrospective analysis of the clinicopathological features of 6 ECCC cases was conducted,including immunohistochemical staining and a review of relevant literature.Results:All patients were female,aged 52-81 years,presenting with postmenopausal abnormal vaginal bleeding.Ultrasound revealed abnormal intrauterine echoes,suggesting a possible mass.Microscopically,tumor cells exhibited clear,eosinophilic,or hobnail-like morphology with papillary,tubular-cystic,and solid structures,and papillary cores showing sclerosis.Cellular atypia was mild,with necrosis observed in some areas;one case was combined with endometrioid carcinoma.Tumor invasion of the myometrium was found in 3 cases(2 cases with<1/2 myometrial involvement and 1 case with>1/2).No lymph node metastases were detected in the left and right pelvic lymph nodes.Immunophenotype:Tumor cells were positive for cytokeratin 7(CK7),Napsin A,hepatocyte nuclear factor 1β(HNF1β),P504S,and paired box gene 8(PAX8).Mismatch repair proteins,MLH1,MSH2,MSH6,and PMS2 were positive.P16 and vimentin were positive or partially positive.Four cases showed partially heterogeneous P53 positive(wild type),and 2 cases showed diffuse strong P53 positive(mutant type).Estrogen receptor(ER),progesterone receptor(PR),cluster of differentiation 10(CD10),androgen receptor(AR),and Wilms'tumor gene 1(WT-1)were negative.The Ki-67 proliferation index ranged from 30%to 80%.Four cases underwent total hysterectomy with bilateral adnexectomy(3 with pelvic lymphadenectomy),and 2 underwent curettage only.Followed-up to May 2024,1 patient had died,while the others were alive.Conclusion:ECCC is a relatively rare carcinoma,primarily occurring in postmenopausal women.It can present as pure clear cell carcinoma or mixed with other carcinoma types.Its histological patterns are diverse,and its prognosis is poor.Immunohistochemistry is essential for diagnosis and differential diagnosis.
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