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    Serous Macrocystic / Oligocystic Adenoma of the Pancreas

    Definition

    • Benign pancreatic neoplasm composed of a small number of large cysts lined by bland clear cells

    Alternate/Historical Names

    • Ill-demarcated serous adenoma
    • Macrocystic serous cystadenoma
    • Serous macrocystic adenoma
    • Serous oligocystic ill-defined adenoma

    Diagnostic Criteria

    • Generally poorly circumscribed
      • Stroma may entrap other elements
        • May be hyalinized
      • No invasion of local organs
    • One or a few large cysts
      • Most 1-3 cm, up to 8 cm reported
    • Lined by single layer of cuboidal cells with clear cytoplasm
      • Abundant glycogen (PAS +, diastase digestible)
      • Rarely eosinophilic cytoplasm
      • May be denuded
    • Bland cytology
      • Round/oval nuclei
      • No mitotic figures
    • Lacks prominent central scar

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

    Original posting/updates: 1/4/07, 4/25/09, 1/4/12

    Supplemental studies

    Immunohistology

    Lining cells

    CK7 pos
    Cam5.2 pos
    Neuron specific enolase pos
    Alpha inhibin 90%
    Synaptophysin neg
    Chromogranin

    neg

    Estrogen receptor neg
    Progesterone receptor neg
    Alpha-1-antitrypsin neg

    Differential Diagnosis

    Serous Macrocystic / Oligocystic Adenoma Serous Microcystic Adenoma
    Small number of larger, variably sized cysts Innumerable small, uniform cysts
    Lacks prominent scar Frequent central scar
    May occur in adults and infants Not reported in infants

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    Serous Macrocystic / Oligocystic Adenoma Serous Cystadenocarcinoma of the Pancreas
    Mass limited to pancreas, without vascular invasion Requires vascular invasion, adjacent organ invasion or metastases

    Serous Macrocystic / Oligocystic Adenoma Mucinous Cystic Neoplasm of the Pancreas
    Cuboidal clear epithelial lining Mucinous lining
    Occurs in males and females Nearly all female
    Lack ovarian type stroma Ovarian type stroma

    Serous Macrocystic / Oligocystic Adenoma Solid Pseudopapillary Neoplasm of the Pancreas
    True cysts lined by a single layer of cells Variable thickness of lining of degenerate spaces
    Occurs in males and females Nearly all female
    Glycogen positive Glycogen negative
    Keratin positive Keratin variable
    Clear cell variant of solid pseudopapillary neoplasm is also reported to be glycogen negative

    Serous Macrocystic / Oligocystic Adenoma Acinar Cell Cystadenocarcinoma of the Pancreas
    Lined by clear cells Lined by cells with granular eosinophilic cytoplasm
    PAS+, diastase sensitive PAS+, diastase resistant
    BCL10, trypsin, chymotrypsin negative BCL10, trypsin, chymotrypsin positive

    Serous Macrocystic / Oligocystic Adenoma Lymphangioma
    Lined by clear cells Lined by endothelium
    Keratin positive CD31, CD34, D240 positive

    Serous Macrocystic / Oligocystic Adenoma Lymphoepithelial Cyst of the Pancreas
    Lined by clear cells Lined by squamous epithelium
    Lacks lymphoid stroma Prominent lymphoid stroma

    Serous Macrocystic / Oligocystic Adenoma Squamoid Cyst of Pancreatic Ducts
    Lined by clear cells Lined by squamoid cells
    GLUT1 positive GLUT1 at most focally positive

    von Hippel Lindau syndrome is associated with diffuse cystic transformation of the pancreas and occasionally with serous microcystic adenomas

    Clinical

    Grading / Staging / Report

    • Grading
      • Not applicable
    • Staging
      • Not applicable
    • Report
      • Comment that the lesion is benign

    Lists

    Cystic Pancreatic Lesions

    Bibliography

    • Bosman FT, Carneiro F, Hruban RH, Thiese ND (Eds). WHO Classifiication of Tumors of the Digestive System, IARC, Lyon 2010.
    • Solcia E, Capella C, Kloppel G . Tumors of the Pancreas, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 20, 1997.
    • Kosmahl M, Wagner J, Peters K, Sipos B, Kloppel G. Serous cystic neoplasms of the pancreas: an immunohistochemical analysis revealing alpha-inhibin, neuron-specific enolase, and MUC6 as new markers. Am J Surg Pathol. 2004 Mar;28(3):339-46.
    • 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.
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