关键词: MMR deficiency dedifferentiated endometrioid carcinoma large cell neuroendocrine carcinoma

来  源:   DOI:10.2147/CMAR.S279888   PDF(Sci-hub)   PDF(Pubmed)

Abstract:
UNASSIGNED: To investigate the relationship between dedifferentiated endometrioid carcinomas with neuroendocrine differentiation and mismatch repair deficiency.
UNASSIGNED: The clinicopathological records and samples of three patients were retrieved from the Pathology Department of Zhejiang University\'s School of Medicine Women\'s Hospital.
UNASSIGNED: The tumors comprised one dominant poorly differentiated component (60-90% of the neoplasm volume) and one well-differentiated glandular component. The poorly differentiated component showed solid sheets with organoid growth patterns and insular, trabecular and rosette/pseudorosette patterns. Large polygonal cells, vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm were observed in the poorly differentiated area. All three cases were diffusely positive for p16 and for at least two of three neuroendocrine markers (chromogranin, synaptophysin, neural cell adhesion molecule (CD56)) in >10% of cancer cells. Loss of MMR protein expression was found in two patients: MLH1 and PSM2 in patient 2 and MSH2 and MSH 6 in patient 3. Abnormal P53 and SMARCB1 (INI1) expression was noted in patient 3. All three patients underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy, and two received postoperative chemotherapy and/or radiation therapy. The patients survived disease-free for 60, 26 and 15 months, respectively.
UNASSIGNED: Dedifferentiated endometrioid carcinomas with neuroendocrine differentiation may be associated with mismatch repair deficiency and have an improved prognosis.
摘要:
探讨去分化子宫内膜样癌与神经内分泌分化和错配修复缺陷的关系。
从浙江大学医学院附属妇女医院病理科检索3例患者的临床病理记录和标本。
肿瘤包含一种显性低分化成分(肿瘤体积的60-90%)和一种高分化腺体成分。分化差的成分显示出具有类器官生长模式和岛状的固体薄片,骨小梁和玫瑰花结/假玫瑰花结模式。大型多边形细胞,囊泡核,突出的核仁,在低分化区域观察到丰富的嗜酸性细胞浆。所有3例p16和至少两种三种神经内分泌标志物(嗜铬粒蛋白,突触素,神经细胞粘附分子(CD56))在>10%的癌细胞中。在两名患者中发现了MMR蛋白表达的缺失:患者2中的MLH1和PSM2以及患者3中的MSH2和MSH6。在患者3中发现P53和SMARCB1(INI1)表达异常。所有3例患者均接受了全腹子宫切除术和双侧附件卵巢切除术,两名患者接受了术后化疗和/或放疗。患者无病存活了60、26和15个月,分别。
去分化子宫内膜样癌伴神经内分泌分化可能与错配修复缺陷相关,预后改善。
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