title: Primary acinar cell carcinoma of the liver.
abstract: We report a case of acinar cell carcinoma primary to the liver. The tumor was diagnosed in a 35-year-old woman complaining of abdominal pain and asthenia; serum alpha-fetoprotein (AFP) levels were increased at 6,000 IU/mL; imaging studies showed a hypervascular mass located in the left lobe of the liver. A left lobectomy was performed. The tumor had a heterogeneous appearance. In well-differentiated areas, tumor cells formed acinar structures, had a pyramidal shape and a highly eosinophilic, granular cytoplasm, PAS diastase resistant. In less-differentiated areas, tumor cells were endocrinelike. The immunohistochemical study showed that tumor cells expressed trypsin. Alpha-fetoprotein and alphal-antritrypsin were detected in about 30% of cells; HepPar1 was present in 15% of cells. Chromogranin A and synaptophysin were detected in rare cells. After surgery, serum AFP levels quickly returned to normal; no evidence of recurrence or metastasis was observed during follow-up. The final diagnosis, based on histological, immunohistochemical, and ultrastructural arguments, was extra-pancreatic acinar cell carcinoma, primary to the liver. This unusual lesion is likely to be the result of an abnormal differentiation pathway involving a transformed multipotential progenitor cell.
authors.affiliation: Service Central d'Anatomie et Cytologie Pathologiques, Hospices Civils de Lyon, Hôpital Edouard Herriot, 69437 Lyon Cedex 03, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Hervieu
authors.forename: Catherine
authors.initials: C
authors.lastname: Lombard-Bohas
authors.forename: Jérôme
authors.initials: J
authors.lastname: Dumortier
authors.forename: Olivier
authors.initials: O
authors.lastname: Boillot
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Scoazec
mesh_terms: D000328:Adult; D018267:Carcinoma, Acinar Cell / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D008113:Liver Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome; D000515:alpha 1-Antitrypsin / Q000032:analysis; D000509:alpha-Fetoproteins / Q000032:analysis
chemical_list: D000515:alpha 1-Antitrypsin; D000509:alpha-Fetoproteins