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[伴有成肌母细胞分化的结直肠癌:8例临床病理分析]

[Colorectal adenocarcinoma with enteroblastic differentiation: a clinicopathological analysis of eight cases].

作者信息

Wang Q, Zhang Y, Tan C, Ni S J, Huang D, Chang B, Sheng W Q, Wang L

机构信息

Department of Pathology, Fudan University Shanghai Cancer Center/Department of Oncology, Shanghai Medical School, Fudan University, Shanghai 200032, China.

Department of Pathology, Shuguang Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai 201203, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2024 Apr 8;53(4):370-376. doi: 10.3760/cma.j.cn112151-20231025-00307.

Abstract

To investigate the clinicopathological features of colorectal adenocarcinoma with enteroblastic differentiation (CAED). Eight cases of CAED diagnosed at the Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China from January 2017 to August 2023 were collected. The histopathological, immunohistochemical, molecular and prognostic features of 8 CAED cases were analyzed. The relevant studies were also reviewed. Among the eight patients, there were six males and two females, with an average age of 58 years (range: 29-77 years, median age: 61.5 years). Preoperative serum alpha-fetoprotein levels were elevated in five patients (14.0-286.6 μg/L). Four tumors were located in the colon, and four tumors in the rectum. Two patients were clinically staged as advanced stage (stage Ⅳ), and distant metastasis occurred at the initial diagnosis (one case had liver metastasis, and the other had lung, bone and multiple lymph nodes metastases). Six patients were clinically staged as locally-advanced stage (Stage Ⅱ-Ⅲ). Three of them developed distant metastases after surgery (one case had liver metastasis, one case had lung metastasis, and one case had peritoneal metastasis). Additionally, two patients died at 9 months and 24 months after surgery, respectively. The tumors were composed of various proportions of adenocarcinoma components with enteroblastic differentiation (30%-100%) and classical tubular adenocarcinoma components. The component with enteroblastic differentiation exhibited morphology similar to embryonic intestinal epithelium: cuboidal or columnar tumor cells arranged in tubular, papillary, cribriform, or solid nest patterns, with clear cytoplasm. Immunohistochemical studies showed that tumor cells expressed at least one oncofetal protein (SALL4, Glypican-3, and AFP). In addition, focal squamous differentiation was observed in 3 cases (3/8). Compared to the primary tumor, both CAED and squamous differentiation components were increased in the metastatic tumors. Based on the sequencing results of KRAS, NRAS and BRAF of the primary and/or metastatic tumors, 5 cases were wild-type, while KRAS exon 2 (G13D) mutations were identified in 2 cases. CAED is a rare colorectal malignancy with a dismal prognosis. Accurate pathological diagnosis is prognostically valuable. The histological features of enteroblastic differentiation, elevated serum AFP levels, and the expression of oncofetal proteins play an important role in the tumor diagnosis.

摘要

探讨具有成肝细胞样分化的结直肠癌(CAED)的临床病理特征。收集了2017年1月至2023年8月在中国上海复旦大学附属肿瘤医院病理科诊断的8例CAED病例。分析了8例CAED病例的组织病理学、免疫组织化学、分子和预后特征,并对相关研究进行了回顾。8例患者中,男性6例,女性2例,平均年龄58岁(范围:29 - 77岁,中位年龄:61.5岁)。5例患者术前血清甲胎蛋白水平升高(14.0 - 286.6μg/L)。4例肿瘤位于结肠,4例肿瘤位于直肠。2例患者临床分期为晚期(Ⅳ期),初诊时即发生远处转移(1例有肝转移,另1例有肺、骨和多处淋巴结转移)。6例患者临床分期为局部晚期(Ⅱ - Ⅲ期)。其中3例术后发生远处转移(1例有肝转移,1例有肺转移,1例有腹膜转移)。此外,2例患者分别在术后9个月和24个月死亡。肿瘤由不同比例的具有成肝细胞样分化的腺癌成分(30% - 100%)和经典管状腺癌成分组成。具有成肝细胞样分化的成分表现出与胚胎肠上皮相似的形态:立方或柱状肿瘤细胞呈管状、乳头状、筛状或实性巢状排列,细胞质清晰。免疫组织化学研究显示肿瘤细胞表达至少一种癌胚蛋白(SALL4、磷脂酰肌醇蛋白聚糖-3和甲胎蛋白)。此外,3例(3/8)观察到局灶性鳞状分化。与原发肿瘤相比,转移瘤中的CAED和鳞状分化成分均增加。根据原发和/或转移瘤的KRAS、NRAS和BRAF测序结果,5例为野生型,2例鉴定出KRAS外显子2(G13D)突变。CAED是一种罕见的结直肠恶性肿瘤,预后较差。准确的病理诊断具有预后价值。成肝细胞样分化的组织学特征、血清甲胎蛋白水平升高和癌胚蛋白的表达在肿瘤诊断中起重要作用。

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