Primary Pancreatic Ewing's Sarcoma with Portal Vein Tumor Thrombosis

    Authors

    C. Reilly; S. Zenoni; M. K. Hasan; S. Varadarajulu; T. A. Tran; S. G. de la Fuente;J. P. Arnoletti

    Comments

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    Abbreviated Journal Title

    J. Gastrointest. Surg.

    Keywords

    Pancreatic Ewing's sarcoma; Portal vein tumor thrombosis; PRIMITIVE NEUROECTODERMAL TUMOR; OF-THE-LITERATURE; EXTREMELY RARE; TUMOR; PRECOCIOUS PUBERTY; Gastroenterology & Hepatology; Surgery

    Abstract

    Extraosseous Ewing's sarcoma (EES) is a mesenchyme-derived small blue cell tumor, which is distinguished by its rarity, aggressiveness, dismal prognosis, and distinct pathogenesis. Occurring almost exclusively among children and young adults, EES can arise from a variety of organs and portends a rapid clinical deterioration and high likelihood of recurrence. We present the first reported case of a primary pancreatic Ewing's sarcoma in a patient with concomitant portal vein thrombosis. The atypical presentation of this extraordinarily rare tumor underscores the imperative to maintain EES in the differential diagnosis of suspicious, indistinct pancreatic lesions in young patients. In addition, we review the available literature describing additional cases of primary pancreatic Ewing's sarcoma.

    Journal Title

    Journal of Gastrointestinal Surgery

    Volume

    17

    Issue/Number

    5

    Publication Date

    1-1-2013

    Document Type

    Article

    Language

    English

    First Page

    1015

    Last Page

    1019

    WOS Identifier

    WOS:000317862200023

    ISSN

    1091-255X

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