Comparative radiological pathological study of biliary intraductal tubulopapillary neoplasm and biliary intraductal papillary mucinous neoplasm

Abdom Radiol (NY). 2017 Oct;42(10):2460-2469. doi: 10.1007/s00261-017-1167-7.


Purpose: Biliary tract intraductal tubulopapillary neoplasms (BT-ITPNs) and intraductal papillary mucinous neoplasms (BT-IPMNs) are rare and poorly described. Herein, we examined the magnetic resonance imaging (MRI) features of BT-ITPNs and BT-IPMNs and correlated them with key gross and microscopic pathological findings.

Methods: We retrospectively identified five patients with definitive pathological findings of BT-ITPN and available diagnostic MRI findings. Key MRI features were correlated to the gross and microscopic pathology and compared to those of BT-IPMNs (19 patients).

Results: All BT-ITPNs showed ductal dilatation and visible intraductal soft tissue with peribiliary liver parenchyma enhancement. One BT-ITPN patient had synchronous lung metastases, and another showed rapid tumor growth rate. The intraductal soft tissue proportion of BT-ITPNs was significantly more than that of BT-IPMNs (p < 0.05). CA-199 level was elevated in 60% of BT-ITPN cases. The overall combined 1-year and 3-year survival rates in the BT-ITPN group was 100% and 40%, and in the BT-IPMN group was 100% and 58%, respectively. A high intraductal soft tissue proportion, a lack of intraluminal mucin, and immunohistochemical absence of MUC5AC are radiological and pathological characteristics that differentiate BT-ITPN from BT-IPMN.

Conclusions: Although rare, BT-ITPN should be suspected when solid intraductal soft tissue and peribiliary liver parenchyma enhancement are present, particularly if the bile duct upstream and downstream of the lesion have a normal diameter, without mucin. Owing to the aggressive nature of the tumor, recognition of these features may indicate the need for more aggressive treatment in selected patients.

Keywords: Bile duct; Biliary intraductal papillary mucinous neoplasm; Biliary intraductal tubulopapillary neoplasm; MUC5AC; Magnetic resonance imaging.

Publication types

  • Comparative Study

MeSH terms

  • Adenocarcinoma, Mucinous / diagnostic imaging*
  • Adenocarcinoma, Mucinous / pathology*
  • Adult
  • Aged
  • Biliary Tract Neoplasms / diagnostic imaging*
  • Biliary Tract Neoplasms / pathology*
  • Carcinoma, Papillary / diagnostic imaging*
  • Carcinoma, Papillary / pathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged
  • Retrospective Studies
  • Survival Rate