Alpha-1-antitrypsin-positive "signet-ring" bile duct adenoma in a patient with MMALTON mutation

Marco Gambarotti, Daniela Medicina, Carla Baronchelli, Luisa Bercich, Fausta Bonetti, Fabio Facchetti

Research output: Contribution to journalArticlepeer-review


A 65-year-old man with a colonic adenocarcinoma and a single small liver nodule underwent surgery. The nodule showed the histological features of a bile duct adenoma. Large eosinophilic, periodic acid-Schiff-positive diastase-resistant intracytoplasmic globules conferred a signet-ring appearance to the cells. Similar globules were found in the biliary epithelium in perinodular portal tracts, whereas smaller globules were detected in periportal hepatocytes. All globules showed strong immunoreactivity for alpha-1-antitrypsin, and the sequence of the alpha-1-antitrypsin gene showed a heterozygous Mmalton genotype. The selective recurrence of large alpha-1-antitrypsin globules within the bile ducts may indicate a neoplastic rather than a reactive or hamartomatous nature of the nodule.

Original languageEnglish
Pages (from-to)218-221
Number of pages4
JournalInternational Journal of Surgical Pathology
Issue number2
Publication statusPublished - Apr 2008


  • Alpha-1-antitrypsin
  • Bile duct adenoma
  • Liver
  • M
  • Signet-ring

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery


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