Mucinous intrahepatic cholangiocarcinoma: a distinct variant

Zhikai Chi, Amarpreet Bhalla, Omer Saeed, Liang Cheng, Kendra Curless, Hanlin L. Wang, Deepa T. Patil, Jingmei Lin

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)131-137
Number of pages7
JournalHuman Pathology
Volume78
DOIs
StatePublished - Aug 2018
Externally publishedYes

Keywords

  • Cholangiocarcinoma
  • Immunohistochemistry
  • Intrahepatic cholangiocarcinoma
  • Mucinous adenocarcinoma
  • Mucinous intrahepatic cholangiocarcinoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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