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  • Surgical Pathology Criteria

    Acinar Cell Cystadenoma of the Pancreas

    Definition

    • Benign cystic lesion of the pancreas lined by cells exhibiting acinar differentiation

    Alternate/Historical Names

    • Acinar cystic transformation (may be a better name, as the lesion is not clonal (Singhi))

    Diagnostic Criteria

    • Wide range of cystic architectural patterns
      • Unilocular to multilocular
      • Cysts may range from minimally dilated acini to prominent cysts
        • Mean size 7 cm, microscopic up to 15 cm
      • Solitary to multiple
        • One diffuse case reported
      • Intracystic nodules may be present
    • Only rarely communicates with duct system
    • Cysts predominantly lined by bland cells with acinar differentiation
      • Moderate to abundant granular eosinophilic cytoplasm
        • PAS positive diastase resistant
        • Usually apical
      • Basal cytoplasm may be basophilic
      • Lining may be multilayered
        • Lumenal cells may be attenuated
      • Multiloculated lesions focally lined by patches of normal appearing ductal cells or squamous cells
      • May form patches with acini
      • May be associated with ducts lined by mucinous columnar epithelium / low grade PanIN
    • Cytologically bland
      • May have at most mild atypia
      • Nucleoli present but small
      • Mitotic figures, necrosis absent
    • No invasion
    • No solid acinar cell adenomas have been reported (see Acinar Cell Nodule)

    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting, last update: 1/2/08, 12/28/13

    Supplemental studies

    Immunohistology

    Immunohistochemistry is not usually necessary for diagnosis
      Acinar Type Cells Ductal Type Cells
    CK7 Pos (fainter) Pos (strong)
    CK19 Neg Pos
    Trypsin Pos Neg
    Chymotrypsin Pos Neg
    Lipase Variable Neg
    Synaptophysin Neg Neg
    Chromogranin Neg Neg
    (Normal, non-reactive acinar cells are negative for CK7)

     

    Differential diagnosis

    Acinar Cell Cystadenoma of the Pancreas Mucinous Cystic Lesions Serous Cystic Lesions
    Lined at least in some areas by cells with granular eosinophilic apical cytoplasm Mucin positive but lacks granular cytoplasm Clear cytoplasm, PASd negative

    Acinar Cell Cystadenoma of the Pancreas Acinar Cell Cystadenocarcinoma of the Pancreas
    Cytologically bland Cytologically moderately atypical with prominent nucleoli
    Mitotic figures absent to very rare Mitotic figures frequent
    No infiltration Frequently infiltrative
    Both are very rare

    Clinical

    • Mean age in forties
    • 90% female
    • Very rare
    • Benign

    Lists

    Cystic Pancreatic Lesions

    Pancreatic Acinar Cell Lesions

    Bibliography

    • Hruban RH, Pitman MB, Klimstra DS. Tumors of the Pancreas, Atlas of Tumor Pathology, AFIP Fourth Series, Fascicle 6, 2007.
    • Bosman FT, Carneiro F, Hruban RH, Thiese ND (Eds). WHO Classifiication of Tumors of the Digestive System, IARC, Lyon 2010.
    • Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol. 2007 Feb;20 Suppl 1:S94-112.
    • Zamboni G, Terris B, Scarpa A, Kosmahl M, Capelli P, Klimstra DS, Lam PW, Klöppel G. Acinar cell cystadenoma of the pancreas: a new entity? Am J Surg Pathol. 2002 Jun;26(6):698-704.
    • Albores-Saavedra J. Acinar cystadenoma of the pancreas: a previously undescribed tumor. Ann Diagn Pathol. 2002 Apr;6(2):113-5.
    • Chatelain D, Paye F, Mourra N, Scoazec JY, Baudrimont M, Parc R, Flejou JF. Unilocular acinar cell cystadenoma of the pancreas an unusual acinar cell tumor. Am J Clin Pathol. 2002 Aug;118(2):211-4.
    • Basturk O, Coban I, Adsay NV. Pancreatic cysts: pathologic classification, differential diagnosis, and clinical implications. Arch Pathol Lab Med. 2009 Mar;133(3):423-38.
    • Khor TS, Badizadegan K, Ferrone C, Fernández-del Castillo C, Desai GS, Saenz A, Le L, Lauwers GY, Deshpande V. Acinar cystadenoma of the pancreas: a clinicopathologic study of 10 cases including multilocular lesions with mural nodules. Am J Surg Pathol. 2012 Nov;36(11):1579-91
    • Singhi AD, Norwood S, Liu TC, Sharma R, Wolfgang CL, Schulick RD, Zeh HJ, Hruban RH. Acinar cell cystadenoma of the pancreas: a benign neoplasm or non-neoplastic ballooning of acinar and ductal epithelium? Am J Surg Pathol. 2013 Sep;37(9):1329-35.

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