Clinicopathological features and treatment of undifferentiated/dedifferentiated endometrial carcinoma

doi:10.3877/cma.j.issn.1673-5250.2022.06.007

Objective

To explore the clinicopathological features, diagnosis, treatment and outcomes of undifferentiated/dedifferentiated endometrial carcinoma (UEC/DEC) in order to provide a basis for clinical decision-making.

Methods

Eleven patients diagnosed as UEC/DEC from January 2018 to March 2022 in the Department of Gynecology of the First Affiliated Hospital of Nanjing Medical University were enrolled in the study. The data including clinical, pathological, molecular features were retrospectively analyzed to investigate the diagnosis, treatment and outcomes of UEC/DEC. This study was approved by the Medical Ethics Committee of the First Affiliated Hospital of Nanjing Medical University (Approval No. QYFYWZll26841). Informed consents were obtained from all those patients.

Results

①Among 11 cases with pathologically confirmed UEC/DEC were identified, including 3 cases of UEC and 8 cases of DEC. The main clinical manifestation is abnormal vaginal bleeding. ②Morphologically, undifferentiated components (UC) of UEC/DEC were composed of solid sheet-like ovoid or polygonal de-cohesive cells with uniform vesicular nuclei, abundant chromatin, lack of glandular differentiation and presenting sharp boundaries from low-grade cancer components. Immunohistochemistry (IHC) stain showed predominantly focal or complete loss expression of cytokeratin (CK), epithelial membrane antigen (EMA), estrogen receptor(ER), paired box protein (Pax8), vimentin and E-cadherin in the UC components. ③Mismatch repair (MMR) protein status of the tumor were performed on 8 cases. Two cases showed MMR deficiency, while the remaining 6 cases showed normal MMR status. ④Six cases presented with FIGO stage Ⅰ-Ⅱ, whereas 5 cases were FIGO stage Ⅲ-Ⅳ. ⑤All patients underwent surgery, and the operation process and postoperative recovery all were good. There were no complications during and after the operation. Only 1 patient with FIGO stage Ⅰ_A received postoperative adjuvant therapy. ⑥These 11 patients were followed up to March 31, 2022. On the follow-up, the longest overall survival (OS) time was 34 months, and median OS time was 10.5 months. Two patients died of the disease with FIGO stage Ⅲ_B and Ⅲ_C2, respectively, and 9 patients were disease-free.

Conclusions

UEC/DEC presents highly aggressive behavior and poor prognosis. It is crucial for clinicians and pathologists to fully understand its clinicopathological and molecular features, to establish accurate diagnosis and appropriate treatment.

Key words: Carcinoma, endometrioid, Undifferentiated endometrial carcinoma, Dedifferentiated endometrial carcinoma, Pathology, clinical, Diagnosis, Chemoradiotherapy, Prognosis, Women

图1 1例DEC患者(No.8，43岁)的子宫影像学检查图像及子宫剖视图[图1A：超声声像图提示宫腔内低回声占位(黄色箭头所示)。图1B~IG：子宫MRI提示宫腔肿瘤病灶(图1B：T2加权矢状位图；图1C：弥散加权成像；图1D：T2加权冠状位图；图1E：T1加权矢状位图；图1F：表观弥散系数图；图1G：增强后T1加权冠状位图。黄色箭头所示为宫腔肿瘤病灶)。图1H：手术切除子宫剖视图(黄色箭头所示为子宫切除后剖视见宫腔内占位，侵犯子宫右前壁肌层)]注：DEC为去分化子宫内膜癌

图2 1例DEC患者(No.8，43岁)髋臼转移MRI检查图像(图2A：T1加权横断面图；图2B：T2加权横断面图；图2C：T2加权冠状位图；图2D：增强后T1加权横断面图；图2E：弥散加权成像；图2F：增强后T1加权冠状位图。黄色箭头所示为右侧髋臼转移病灶)注：DEC为去分化子宫内膜癌

图3 1例DEC患者(No.2，43岁)组织病理学切片HE染色及IHC染色检测图[图3A：DEC可见UC(黄色箭头所示)及低级别成分(黑色箭头所示)，二者间界限清晰，UC细胞多形性明显，细胞核显著(HE染色，高倍)；图3B：未分化区域可见肿瘤细胞黏附性差，呈弥漫片状分布，多形性明显(HE染色，高倍)；图3C：DEC伴腹主动脉旁淋巴结转移，可见转移的肿瘤细胞呈UC细胞的特征(HE染色，低倍)；图3D: DEC未分化区域CK-pan IHC染色可见灶性区呈阳性(高倍)；图5E：DEC未分化区域Pax8 IHC染色可见灶性区呈阳性(高倍)；图3F：DEC未分化区域ER IHC染色可见肿瘤细胞不表达，子宫平滑肌组织表达呈阳性(高倍)]注：DEC为去分化子宫内膜癌，HE为苏木精-伊红，IHC为免疫组织化学，UC为未分化组分，CK为细胞角蛋白，Pax8为配对盒8基因，ER为雌激素受体，MMR为错配修复

图4 11例UEC/DEC患者不同部位肿瘤的分子免疫组化表达热图注：PALN为腹主动脉旁淋巴结，LGC为低级别组分，UC为未分化组分，UEC为未分化子宫内膜癌，DEC为去分化子宫内膜癌，LGEC为低级别子宫内膜样癌，HGEC为高级别子宫内膜样癌，HGSC为高级别子宫内膜浆液性癌

图5 1例DEC患者(No.9，50岁)接受肿瘤细胞减灭术1个月后的CT检查结果[图5A：阴阜及阴道壁可见转移灶(黄色箭头所示)；图5B：肝可见转移灶弥漫性分布(黄色箭头所示)；图5C：肺可见多发转移灶(黄色箭头所示)]注：DEC为去分化子宫内膜癌

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