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  • Surgical Pathology Criteria



    • Carcinoma of the pancreas exhibiting multiple lines of differentiation including acinar and squamous

    Alternate/Historical Names

    • Infantile pancreatic carcinoma
    • Pancreatic carcinoma of childhood
    • Pancreaticoblastoma

    Diagnostic Criteria

    • 2/3 of cases under age 5
    • Usually well circumscribed
      • May infiltrate
      • Cellular lobules separated by fibrous bands
      • Some congenital cases may be cystic
    • Predominant acinar component
      • Sheets of cells merge with acinar formations
      • Sharp cell borders
      • Nuclei central to basal
      • Prominent nucleoli
      • Cytoplasm may be amphophilic to granular eosinophilic
        • PASd positivity variable
      • Positive for trypsin, chymotrypsin and lipase (variable)
    • Squamoid nests required for diagnosis
      • Abundant eosinophilic cytoplasm
      • May be vague or with overt keratinization
      • Frequently with biotin rich optically clear nuclei
    • Endocrine component in 2/3 of cases
      • May be detectable only with stains
    • Ductal / glandular component occasionally present
      • Usually focal
      • May be mucin positive
    • Variably cellular stroma
      • More cellular in pediatric cases
      • Rarely forms bone or cartilage
      • Rarely appears neoplastic
    • Primitive round cell component rarely present
      • Uniform immature appearing small cells

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

    Original posting/updates: 1/6//08, 1/2/12

    Supplemental studies


    • Acinar component
      • Trypsin, chymotrypsin positive
      • Lipase variable
      • Alpha fetoprotein variable
    • Squamoid nests
      • CK 8, 18, 19 positive
      • CK5/6 occasionally focal
    • Endocrine differentiation
      • Chromogranin, synaptophysin positive scattered cells in 2/3 of cases
      • Islet polypeptides usually negative

    Differential diagnosis

    Acinar Cell Carcinoma of the Pancreas Pancreatoblastoma
    Typically older patients (40-70 years), rare in children 2/3 <5 years, adult cases mean age 40
    Lacks squamoid nests Squamoid nests present
    Distinction may not be critical in adults as both are aggressive in adults


    Pancreatoblastoma Adenosquamous Carcinoma of the Pancreas
    Predominantly pediatric but 1/3 in adults Not reported under age 20
    Prominent acinar differentiation No acinar differentiation
    May have primitive round cell component No primitive round cell component


    Pancreatoblastoma Solid Pseudopapillary Neoplasm of the Pancreas
    Both male and female Overwhelmingly female
    2/3 <5 years of age Very rare <5
    Trypsin, chymotrypsin positive Trypsin, chymotrypsin negative
    Keratin positive Keratin variable
    Lacks pseudopapillary architecture Frequent pseudopapillary architecture
    May have true lumens No true lumens
    Squamoid nests always present Lacks squamous differentiation
    Frequently increased mitotic figures Infrequent mitotic figures


    Pancreatoblastoma Well Differentiated Pancreatic Neuroendocrine (Islet Cell) Tumor
    Trypsin, chymotrypsin positive Trypsin, chymotrypsin negative
    Squamoid nests always present Lacks squamous differentiation
    Usually prominent nucleoli Salt and pepper chromatin
    Chromogranin, synaptophysin scattered positive Chromogranin or synaptophysin widespread staining
    Islet polypeptide markers negative or very focal Islet polypeptide markers frequently positive
    Cellular stroma frequent Lacks cellular stroma


    • Most cases under age 5
      • Congenital cases reported with Beckwith-Wiedemann syndrome
    • 1/3 of cases in adults
      • Adult cases mean age 40
    • Pediatric cases curable if resectable, otherwise aggressive
    • Adult cases aggressive
    • Lipase hypersecretion syndrome not reported
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    Pancreatic Carcinomas

    Pancreatic Acinar Cell Lesions


    • Solcia E, Capella C, Kloppel G . Tumors of the Pancreas, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 20, 1997.
    • Bosman FT, Carneiro F, Hruban RH, Thiese ND (Eds). WHO Classifiication of Tumors of the Digestive System, IARC, Lyon 2010.
    • Klimstra DS, Wenig BM, Adair CF, Heffess CS. Pancreatoblastoma. A clinicopathologic study and review of the literature. Am J Surg Pathol. 1995 Dec;19(12):1371-89.
    • Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, Cameron JL, Hruban RH. Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas : frequent alterations in the APC/beta-catenin pathway and chromosome 11p. Am J Pathol. 2001 Nov;159(5):1619-27.
    • Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol. 2007 Feb;20 Suppl 1:S94-112.


    Printed from Surgical Pathology Criteria: http://surgpathcriteria.stanford.edu/
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