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Solid-pseudopapilläre Neoplasien

Weiblich orientiert und rätselhaft

Solid pseudopapillary neoplasms

Enigmatic entity with female preponderance

  • Schwerpunkt: Pankreaspathologie
  • Published:
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Zusammenfassung

Solid-pseudopapilläre Neoplasien (SPN) stellen eine morphologisch wie biologisch besondere Tumorentität des Pankreas dar. Es handelt sich um große solitäre Tumoren, die typischerweise bei jungen Frauen auftreten. Histologisch zeigen sie in variabler Zusammensetzung solide, pseudopapilläre und pseudozystische Muster. Die Tumorzellen sind monomorph und ihr Immunprofil ist durch eine Positivität für Vimentin, neuronspezifische Enolase (NSE), β-Catenin und den Progesteronrezeptor charakterisiert. Etwa 90% der Patienten werden durch eine komplette Resektion des Tumors geheilt. Da jedoch in wenigen Fällen Rezidive und Metastasen auftreten können, werden sie als niedrigmaligne eingestuft. Histologisch kann am Primärtumor ein malignes Verhalten nicht vorhergesagt werden. Die wichtigste Differenzialdiagnose ist der neuroendokrine Tumor des Pankreas. Die Ursache und Herkunft der SPN sind unklar.

Abstract

Solid pseudopapillary neoplasms (SPN) of the pancreas represent a special tumor entity, both morphologically and biologically. They form large solitary tumors that occur predominantly in young women. Histologically, they show solid, pseudopapillary, and pseudocystic patterns. The tumor cells are monomorphous and typically express vimentin, neuron-specific enolase, nuclear β-catenin, and the progesterone receptor. Complete resection cures the tumor in about 90% of the cases. However, because recurrences and even metastases may occur in a small number of cases, SPN are classified as low-grade malignant tumors. Predicting malignancy histologically is not yet possible. The most important differential diagnosis to consider is neuroendocrine tumor of the pancreas. The etiology and pathogenesis of SPN are obscure.

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Authors and Affiliations

  1. Institut für Allgemeine Pathologie, Universität Kiel

    M. Kosmahl, K. Peters, M. Anlauf, B. Sipos, U. Pauser, J. Lüttges & G. Klöppel

  2. Institut für Allgemeine Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Michaelisstraße 11, 24105, Kiel

    M. Kosmahl

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  1. M. Kosmahl
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  2. K. Peters
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  3. M. Anlauf
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  4. B. Sipos
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  5. U. Pauser
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  6. J. Lüttges
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  7. G. Klöppel
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Correspondence to M. Kosmahl.

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Kosmahl, M., Peters, K., Anlauf, M. et al. Solid-pseudopapilläre Neoplasien. Pathologe 26, 41–45 (2005). https://doi.org/10.1007/s00292-004-0729-y

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  • DOI: https://doi.org/10.1007/s00292-004-0729-y

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