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 journalArticle

2 Citations (Scopus)

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 1 2018

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Cholangiocarcinoma
Mutation
Survival
Microsatellite Repeats
Adenocarcinoma
Immunohistochemistry

Keywords

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Mucinous intrahepatic cholangiocarcinoma : a distinct variant. / Chi, Zhikai; Bhalla, Amarpreet; Saeed, Omer; Cheng, Liang; Curless, Kendra; Wang, Hanlin L.; Patil, Deepa T.; Lin, Jingmei.

In: Human Pathology, Vol. 78, 01.08.2018, p. 131-137.

Research output: Contribution to journalArticle

Chi, Z, Bhalla, A, Saeed, O, Cheng, L, Curless, K, Wang, HL, Patil, DT & Lin, J 2018, 'Mucinous intrahepatic cholangiocarcinoma: a distinct variant', Human Pathology, vol. 78, pp. 131-137. https://doi.org/10.1016/j.humpath.2018.04.010
Chi, Zhikai ; Bhalla, Amarpreet ; Saeed, Omer ; Cheng, Liang ; Curless, Kendra ; Wang, Hanlin L. ; Patil, Deepa T. ; Lin, Jingmei. / Mucinous intrahepatic cholangiocarcinoma : a distinct variant. In: Human Pathology. 2018 ; Vol. 78. pp. 131-137.
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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.",
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AB - 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.

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