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    Solid-pseudopapillary Neoplasm of the Pancreas

    Definition

    • Bland low grade malignant neoplasm characterized by solid and discohesive pseudopapillary areas

    Alternate/Historical Names

    • Papillary cystic neoplasm or tumor
    • Solid-cystic tumor
    • Solid and papillary epithelial neoplasm

    Diagnostic Criteria

    • Variably solid, pseudopapillary and cystic pattern
      • Occasionally completely solid
        • Usually smaller tumors
        • May be sclerotic
        • May have foamy macrophages and cholesterol clefts
      • Frequent pseudopapillary pattern
        • Formed by separation of vascular cores with adherent cells
          • Typically thin walled vessels in cores
        • Spaces may be acellular or contain RBCs (blood lakes)
      • Frequent cystic pattern
        • Degenerative changes may cause predominantly cystic structure
    • Uniform polygonal cells
      • Cytoplasm clear to eosinophilic
        • Lack glycogen and mucin
        • May have intra or extracytoplasmic PASd+ globules
      • Bland round to oval nuclei
        • Mitotic figures rare
        • Frequent longitudinal nuclear grooves
      • Frequently radially oriented around vessels
        • May resemble rosettes
    • Features proposed as predictive of malignant behavior
      • Perineural invasion
      • Vascular invasion
      • Deep invasion of pancreatic parenchyma
      • Invasion of surrounding organs
      • Nuclear atypia
      • Elevated mitotic rate
      • It is not clear if any of these are statistically significant predictors
      • Rare tumors lacking these features may still metastasize
    • Rare anaplastic transformation has been reported
      • Very high mitotic rate, necrosis, atypia and high nuclear:cytoplasmic ratio

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

    Original posting / last update: 2/10/07, 7/14/11, 1/4/12

    Supplemental studies

    Histochemistry

    • Mucin stain negative
    • Glycogen negative
    • PASd positive intra and extracellular globules may be present

    Immunohistology

    CD99 paranuclear dots positive
    Alpha 1 antitrypsin positive
    Alpha 1 antichymotrypsin positive
    Neuron specfic enolase positive
    Vimentin positive
    Progesterone receptor positive
    Beta-catenin positive
    CD10 positive
    CD56 positive
    FLI-1 positive
    Galectin 3 positive
    CD117 positive
    Keratin variable
    Synaptophysin 50%
    CD34 negative
    Estrogen receptor negative
    Chromogranin negative
    • One report describes paranuclear dot like staining seen in solid-pseudopapillary tumor but not in neuroendocrine tumors or adenocarcinoma (Guo 2012)
      • Other neoplasms have membrane or diffuse cytoplasmic staining
      • If confirmed, this may be a very useful marker
    • Alpha 1 antitrypsin and antichymotrypsin may identify only scattered cells, but they should be intensely stained
    • Keratin usually negative or at most focally positive
    • One report on the use of claudins
      Claudin 5 Claudin 7
    Solid Pseudopapillary Tumor Strong membrane positive Membrane negative, occasional cytoplasm

    Endocrine Tumor

    Membrane negative Membrane positive
    Acinar cell carcinoma Membrane negative Membrane positive
    Pancreatoblastoma Membrane negative Membrane positive
    From Comper 2009

     

    Molecular Genetics

    • EWS/FLI-1 translocation not present even though FLI-1 positive by immunohistochemistry

    Differential Diagnosis

    Well Differentiated Pancreatic Neuroendocrine (Islet Cell) Tumor Solid Pseudopapillary Neoplasm of the Pancreas
    Lacks pseudopapillary architecture Frequent pseudopapillary architecture
    Chromogranin usually strongly positive Chromogranin focal to negative
    Keratin positive Keratin variable
    Galectin 3 negative Galectin 3 positive (small numbers)
    Vimentin, CD10 negative Vimentin, CD10 positive
    Nuclear beta catenin negative Nuclear beta catenin positive
    No PAS+ granules in cytoplasm PAS+ granules may be present
    CD99 variable but not paranuclear dots CD99 paranuclear dots (one report)
    Solid pseudopapillary neoplasm may be synaptophysin positive

    Acinar Cell Carcinoma of the Pancreas Solid Pseudopapillary Neoplasm of the Pancreas
    Predominantly male Overwhelmingly female
    Granular PASd+ cytoplasm PASd+ only scattered globules
    Beta-catenin/CD56/CD10 negative Beta-catenin/CD56/CD10 positive
    BCL10, trypsin, chymotrypsin positive BCL10, trypsin, chymotrypsin negative
    Alpha 1 antitrypsin negative Alpha 1 antitrypsin positive
    Keratin positive Keratin variable
    Usually infiltrative Usually circumscribed
    Lacks pseudopapillary architecture Frequent pseudopapillary architecture
    May have true lumens No true lumens
    Frequently increased mitotic figures Infrequent mitotic figures
    CD99 negative CD99 paranuclear dots (one report)

    Serous Microcystic 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
    Median age 60's Mean age 20's
    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 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

    Clinical

    • Female predominance
    • Mean age 30-35
      • Range 8-85
    • Rare male cases at same mean age (4-7%)
    • Not associated with abnormal endocrine states or endocrine therapy
    • Best considered low grade malignant
      • >95% cured by resection
      • Most recurrences or metastases associated with atypical features
      • Rare non-atypical cases may metastasize
        • May occur years later
      • Most common metastatic sites
        • Liver
        • Peritoneum / omentum
        • Local lymph nodes involved rarely
      • Death rare even with metastases

    Grading / Staging / Report

    • Grading
      • Not applicable
    • Staging
      • Not applicable
    • Report
      • Comment on presence or absence of features predictive of malignant behavior
      • Comment that rare cases may behave aggressively even in the absence of atypical features

    Lists

    Cystic Pancreatic Lesions

    Bibliography

    • 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.
    • Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayashi I. Papillary cystic tumors of the pancreas. Assessment of their malignant potential. Cancer. 1993 Jan 1;71(1):82-92.
    • Tiemann K, Kosmahl M, Ohlendorf J, Krams M, Kloppel G. Solid pseudopapillary neoplasms of the pancreas are associated with FLI-1 expression, but not with EWS/FLI-1 translocation. Mod Pathol. 2006 Nov;19(11):1409-13.
    • Geers C, Moulin P, Gigot JF, Weynand B, Deprez P, Rahier J, Sempoux C. Solid and pseudopapillary tumor of the pancreas--review and new insights into pathogenesis. Am J Surg Pathol. 2006 Oct;30(10):1243-9.
    • Albores-Saavedra J, Simpson KW, Bilello SJ. The clear cell variant of solid pseudopapillary tumor of the pancreas: a previously unrecognized pancreatic neoplasm. Am J Surg Pathol. 2006 Oct;30(10):1237-42.
    • Notohara K, Hamazaki S, Tsukayama C, Nakamoto S, Kawabata K, Mizobuchi K, Sakamoto K, Okada S, Solid-pseudopapillary tumor of the pancreas: immunohistochemical localization of neuroendocrine markers and CD10. Am J Surg Pathol. 2000 Oct;24(10):1361-71.
    • Adams AL, Siegal GP, Jhala NC. Solid pseudopapillary tumor of the pancreas: a review of salient clinical and pathologic features. Adv Anat Pathol. 2008 Jan;15(1):39-45.
    • 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.
    • Tang LH, Aydin H, Brennan MF, Klimstra DS. Clinically aggressive solid pseudopapillary tumors of the pancreas: a report of two cases with components of undifferentiated carcinoma and a comparative clinicopathologic analysis of 34 conventional cases. Am J Surg Pathol. 2005 Apr;29(4):512-9.
    • Comper F, Antonello D, Beghelli S, Gobbo S, Montagna L, Pederzoli P, Chilosi M, Scarpa A. Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. Am J Surg Pathol. 2009 May;33(5):768-74.
    • Guo Y, Yuan F, Deng H, Wang HF, Jin XL, Xiao JC. Paranuclear dot-like immunostaining for CD99: a unique staining pattern for diagnosing solid-pseudopapillary neoplasm of the pancreas. Am J Surg Pathol. 2011 Jun;35(6):799-806.
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