ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2015
Intraductal tubulopapillary neoplasms of the bile ducts: clinicopathologic, immunohistochemical, and molecular analysis of 20 cases.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 34 papers, 2 of them syntheses that pooled it.
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Who cites it
34 citing papers in PubMed, 2 syntheses or guidelines pooled it, 103 citations in OpenAlex.
- Pancreatic ductal adenocarcinomas associated with intraductal papillary mucinous neoplasms (IPMNs) versus pseudo-IPMNs: relative frequency, clinicopathologic characteristics and differential diagnosis.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Pooled it
- HER2/HER3 pathway in biliary tract malignancies; systematic review and meta-analysis: a potential therapeutic target?Cancer metastasis reviews · 2017Pooled it
- Benign Biliary Tumors and Precursor Neoplasms: An Updated Clinicopathological and Molecular Review Based on the 2026 WHO Classification.Biomedicines · 2026Review
- Spectrum of Biliary and Nonbiliary Neoplasms Growing and Spreading Within the Lumen of the Bile Ducts.Cancers · 2026Review
- [Cholangiocarcinoma : Histopathological diagnosis, classification and molecular diagnostics-an update].Pathologie (Heidelberg, Germany) · 2025Review
- Neoplastic premalignant pancreatobiliary lesions: current update on the spectrum of lesions and their imaging appearances.Abdominal radiology (New York) · 2025Review
- Article
- Review
- Intraductal papillary neoplasm of the bile duct: Cytomorphologic and molecular features.Cancer cytopathology · 2023Article
- Intraductal papillary mucinous neoplasm of the biliary tract in the caudate lobe of the liver: a case report and literature review.Frontiers in oncology · 2023Article
- Intraductal tubulopapillary neoplasm.BMJ case reports · 2022Article
- Pathological, molecular, and clinical characteristics of cholangiocarcinoma: A comprehensive review.World journal of gastrointestinal oncology · 2022Review
- Up-to-Date Pathologic Classification and Molecular Characteristics of Intrahepatic Cholangiocarcinoma.Frontiers in medicine · 2022Review
- Intraductal tubulopapillary neoplasms of the pancreas and biliary tract: The black swan of hepatobiliary surgery.Annals of hepato-biliary-pancreatic surgery · 2021Article
- Mucin-producing Cystic Hepatobiliary Neoplasms: Updated Nomenclature and Clinical, Pathologic, and Imaging Features.Radiographics : a review publication of the Radiological Society of North America, Inc · 2021Article
- Whole Exome Sequencing of Biliary Tubulopapillary Neoplasms Reveals Common Mutations in Chromatin Remodeling Genes.Cancers · 2021Article
- Benign biliary neoplasms and biliary tumor precursors.Pathologica · 2021Review
- Intracholecystic tubular non-mucinous neoplasm (ICTN) of the gallbladder: a clinicopathologically distinct, invasion-resistant entity.Virchows Archiv : an international journal of pathology · 2021Article
- Mural Intracholecystic Neoplasms Arising in Adenomyomatous Nodules of the Gallbladder: An Analysis of 19 Examples of a Clinicopathologically Distinct Entity.The American journal of surgical pathology · 2020Article
- Balloon Extraction of an Intraductal Tubulopapillary Neoplasm of the Bile Duct During Endoscopic Retrograde Cholangiopancreatography.ACG case reports journal · 2020Article
Corrections and comments
- Erratum issued
Authors and funding
17 authors at 9 institutions in 6 countries.
Funding
Abstract
Intraductal tubulopapillary neoplasm is a well-established entity in the pancreas. A similar, if not identical, tumor occurs also in the biliary tract. We conducted a multicenter study of 20 such lesions, focusing on their clinicopathologic characteristics and molecular profile. Biliary intraductal tubulopapillary neoplasms were seen in patients in their 60s (mean 62 years). The tumors were intrahepatic 70%, extrahepatic 10%, and perihilar 20%; mean tumor size was 6.9 cm. Histologically, all intraductal tubulopapillary neoplasms showed, in addition to their typical tubular pattern, solid areas (70%) or abortive papillae (50%). Necrosis was common (85%), predominantly focal (40%), and with 'comedocarcinoma-like pattern' in 40%. Immunohistochemically, these neoplasms were characterized by the expression of MUC1 (80%) and MUC6 (30%) and by the absence of MUC2 and MUC5AC. Associated invasive carcinomas were present in 16 (80%), mainly conventional tubular adenocarcinoma (50%). The molecular alterations observed included CDKN2A/p16 (intraductal components 44%, invasive 33%) and TP53 (intraductal components 17%, invasive 9%). Mutations in KRAS (intraductal 6%, invasive 0%), PIK3CA (intraductal 6%, invasive 0%), and loss of SMAD4/DPC4 (intraductal 7%, invasive 0%) were rare. No alterations/mutations were identified in IDH1/2, BRAF, GNAS, EGFR, HER2, and β-catenin. Follow-up information was available for 17 patients (85%) with mean follow-up 44 months. Overall combined survival rates showed favorable prognosis: 1 year 100%, 3 years 90%, and 5 years 90%. In conclusion, despite the relatively high incidence of invasive carcinoma (80%), available follow-up suggests that biliary intraductal tubulopapillary neoplasms have an indolent behavior. Molecular analyses highlight the low prevalence of alterations of common oncogenic signaling pathways in intraductal tubulopapillary neoplasm. Further studies using whole-exome sequencing are required to discover yet unknown molecular changes and to understand the carcinogenesis of intraductal tubulopapillary neoplasms.
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