Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN - 46202, USA.
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA - 90095, USA.
Hum Pathol. 2018 Aug;78:131-137. doi: 10.1016/j.humpath.2018.04.010. Epub 2018 Apr 23.
Mucinous variant of intrahepatic cholangiocarcinoma (iCC) is rare, and its clinicopathological features and prognosis are far less clear. Six patients who had iCCs with more than 50% of mucinous component and 79 conventional iCCs were included in the study. The mean size of mucinous and conventional iCCs was 6.2 and 6.0 cm, respectively. Most patients (83%) with mucinous iCC presented at T3 stage or above compared with 28% of the conventional group (P < .01). Three patients with mucinous iCC (50%) died within 1 year. The average survival time of patients with mucinous iCCs was significantly reduced compared with that of the conventional group (9 months versus 2 years; P < .001). Immunohistochemistry was performed on 6 mucinous and 12 conventional iCCs with matched age, sex, and stage, which revealed positive immunoreactivity in MUC1 (83% versus 58%), MUC2 (33% versus 17%), MUC5AC (100% versus 42%), MUC6 (50% versus 0), CK7 (83% versus 83%), CK20 (0 versus 17%), CDX2 (17% versus 0), p53 (67% versus 67%), Smad4 (67% versus 58%), and EGFR (83% versus 42%) in mucinous and conventional iCCs, respectively. Molecular studies showed one mucinous iCC with KRAS G12C mutation and no BRAF or IDH1/2 mutations. Mucinous iCC is a unique variant that constitutes 7% of iCCs. It is more immunoreactive for MUC1, MUC2, MUC5AC, and MUC6. Unlike adenocarcinomas of colorectal primary, mucinous iCCs are often CK7+/CK20-/CDX2- and microsatellite stable. Patients with mucinous iCC likely present at advanced stage upon diagnosis with shorter survival time compared with the conventional counterparts.
肝内胆管细胞癌（iCC）的黏液变异型较为罕见，其临床病理特征和预后仍不清楚。本研究纳入了 6 例黏液成分超过 50%的 iCC 患者和 79 例常规 iCC 患者。黏液型和常规型 iCC 的平均大小分别为 6.2cm 和 6.0cm。与常规组的 28%相比，黏液型 iCC 患者中 83%在 T3 期或以上分期（P<0.01）。3 例（50%）黏液型 iCC 患者在 1 年内死亡。与常规组相比，黏液型 iCC 患者的平均生存时间显著缩短（9 个月与 2 年；P<0.001）。对 6 例黏液型和 12 例常规型 iCC 进行了匹配年龄、性别和分期的免疫组织化学染色，结果显示 MUC1（83%比 58%）、MUC2（33%比 17%）、MUC5AC（100%比 42%）、MUC6（50%比 0%）、CK7（83%比 83%）、CK20（0 比 17%）、CDX2（17%比 0%）、p53（67%比 67%）、Smad4（67%比 58%）和 EGFR（83%比 42%）在黏液型和常规型 iCC 中的阳性表达率存在差异。分子研究显示，1 例黏液型 iCC 存在 KRAS G12C 突变，而不存在 BRAF 或 IDH1/2 突变。黏液型 iCC 是一种独特的变异型，占 iCC 的 7%。它对 MUC1、MUC2、MUC5AC 和 MUC6 的免疫反应更强。与结直肠原发性腺癌不同，黏液型 iCC 通常 CK7+/CK20-/CDX2-且微卫星稳定。与常规型 iCC 相比，黏液型 iCC 患者在诊断时更可能处于晚期，生存时间更短。
