| Background:Mesonephric-like adenocarcinoma(MLA)is a malignant tumor of female reproductive tract,which is morphologically and immunophenotypically similar to Mesonephric adenocarcinoma(MA).It usually occurs in the locations that are not typically of mesonephric duct remnants.But due to the uncertainty of its origin,no mesonephric duct hyperplasia or remnants were seen in or around the tumor,so it was defined as MLA.In the early stage,the definition of MLA was not clear,so the majority of cases reported on MLA were defined as MA.After the introduction of this subtype in the WHO classification of tumors of the female reproductive system in2020,the number of reported cases of MLA has slightly increased,but because the number of reported cases related to MLA is still low and cases located at fundus of uterus are even rarer,the microscopic pattern of MLA can have multiple growth patterns and therefore can be easily misdiagnosed as other types of tumors.Summarizing the existing case reports and sorting out the clinicopathological features is helpful to clarify the diagnosis,treatment and to predict the prognosis of this disease.Purpose:The clinical manifestations,histopathological features,treatment methods and prognosis of MLA were studied and discussed in depth,so as to improve our cognition of MLA,reduce misdiagnosis and missed diagnosis,and explore the factors that may affect its prognosis.Method:This study includes case analysis and literature review:1.Case analysis:(1)Correct treatment of tissue samples according to the operating norms of the pathology department.(2)Standardized description of the fundus of uterus MLA of this example was carried out according to the standards of clinical and pathological reports.2.Literature review:(1)The case reports of MLA of uterus in domestic and foreign literatures(CNKI,VIP,Wanfang,Pubmed,etc.)were searched.(2)Cases satisfying the pathological diagnostic standard of MLA were mainly studied in this study,and cases with duplicate,unreadable and unclear clinicopathological data were excluded.(3)The clinicopathological data in the relevant literature were analyzed,and the data on the epidemiology,clinical manifestations,site of onset,pathological histological features,treatment modalities,and prognostic follow-up of MLA patients were summarized and summarized,and statistical analysis was performed to compare the clinicopathological features and prognosis of MLA at each site.Results:All literature on MLA published(CNKI,Vipers,Wanfang,Pubmed,etc.)was searched,and a total of 141 cases were retrieved according to the inclusion and exclusion criteria,including 1 case at our hospital,total of 142 cases are included in this study.To summarize their main characteristics:(1)age: MLA patients had a wide age span ranging from 18 to 91 years,with a mean age of 60 years and a median age of 61 years;(2)site of onset: 47 cases located in the ovaries,95 cases located in the uterus,62 of which were located in the body of the uterus,only 3 cases were located in the fundus of the uterus,the present case being one of them,and another 31 cases of uterine MLA where the exact location was unknown;(3)Clinical features and laboratory data: common symptoms include irregular vaginal bleeding,postmenopausal bleeding and abdominal pain,elevated CA125 levels in 17 patients,elevated CA199 levels in 7 patients and elevated CEA levels in 3 patients;(4)Clinicopathological features: tumors span a wide range in sizes,with maximum diameters ranging from 1.5 cm to 19 cm,with an average diameter of 6.9 cm;they are mostly yellowish brown to grayish brown,with poorly defined perimeters,and multiple structures of different shapes exist simultaneously under the microscope.The presence of many different morphological structures at the same time,including tubular and glandular structures,as well as papillary,solid,reticular,spindle-like structures or spindle cell areas and other components,in most cases,will show a mixture of two or more morphological structures,eosinophilic secretion in the lumen of the duct is the characteristic manifestation,cellular nucleus is vacuolated,nuclear overlap is visible,and some of the nuclear grooves are visible;immunophenotype:tumor cells usually PAX-8,CK7 Immunophenotype: tumor cells are usually positive for PAX-8,CK7,GATA3,TTF-1,positive for CD10 luminal margin,some tumor cells are positive for Calretinin,positive for P16 mottled,negative for ER,PR,WT-1,P53 are wild type;(5)MLA should be differentiated from MA,endometrioid adenocarcinoma,plasmacytoma,clear cell carcinoma,carcinosarcoma,etc.;(6)Surgery is the main treatment.For MLA patients with a primary focus in the uterus,total hysterectomy,bilateral salpingo-oophorectomy,pelvic lymph node dissection and,if necessary,para-aortic lymph node dissection and/or greater omentum dissection are usually performed,whereas for MLA patients with a primary focus in the ovaries,total hysterectomy,bilateral salpingo-oophorectomy,pelvic lymph node dissection,greater omentum dissection and,if necessary,para-aortic lymph node dissection are usually performed;(7)Follow-up time: 1-100 months.Conclusion:MLA cases occurring in fundus of uterus are very rare,most cases of uterine MLA occur in the uterine body,and there is no significant difference in histological characteristics,immunophenotype and prognosis between MLA cases occurring in fundus of uterus and MLA in other sites.Multiple morphological structures can be seen in MLA under the microscope,and immunophenotype is crucial for the diagnosis of MLA,because PAX-8 has low specificity.The immune indicators that play a decisive role in diagnosis are GATA3,TTF-1 and CD10,and whether there is necrosis affects the disease progression of MLA patients after treatment. |