Mucinous intrahepatic cholangiocarcinoma: a distinct variant

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

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2 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

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Keywords

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Chi, Z., Bhalla, A., Saeed, O., Cheng, L., Curless, K., Wang, H. L., Patil, D. T., & Lin, J. (2018). Mucinous intrahepatic cholangiocarcinoma: a distinct variant. Human pathology, 78, 131-137. https://doi.org/10.1016/j.humpath.2018.04.010