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Undifferentiated round cell sarcomas of the skin

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11140%2F25%3A10489095" target="_blank" >RIV/00216208:11140/25:10489095 - isvavai.cz</a>

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=9FR0ESbEoU" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=9FR0ESbEoU</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1016/j.mpdhp.2024.11.001" target="_blank" >10.1016/j.mpdhp.2024.11.001</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Undifferentiated round cell sarcomas of the skin

  • Original language description

    Undifferentiated round cell sarcomas are a heterogenous group of bone/soft tissue neoplasms characterized by relatively monotonous small blue round cell morphology, with high nuclear/cytoplasmic ratio and unspecific or overlapping immunohistochemical findings. According to the WHO Classification of Tumors of Soft tissue and Bone, this group includes Ewing sarcoma, CIC-rearranged sarcoma, sarcoma with BCOR genetic alterations, and round cell sarcoma with EWSR1::non-ETS fusions. Although they are quite rare in cutaneous/subcutaneous localization, they should always be included in the differential diagnosis of poorly differentiated round cell cutaneous tumors. Among them, subcutaneous/cutaneous Ewing sarcoma and superficial CIC- rearranged sarcoma are the most common entities and distinction between them is extremely important, as Ewing sarcoma has a significantly better prognosis, particularly in the superficial location, as opposed to CIC-rearranged sarcoma which is a highly aggressive sarcoma with a poor clinical outcome. Molecular analysis is required for diagnostic confirmation, and it includes FISH, standard sequencing-based methods and recently also DNA methylation profiling. This article provides a summary on contemporary knowledge regarding undifferentiated cutaneous round cell sarcomas, with an emphasis on characteristic histologic and immunohistochemical features as well as diagnostic pitfalls, including differential diagnosis and potential false-negative results in molecular assays.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>SC</sub> - Article in a specialist periodical, which is included in the SCOPUS database

  • CEP classification

  • OECD FORD branch

    30109 - Pathology

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2025

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Diagnostic Histopathology

  • ISSN

    1756-2317

  • e-ISSN

    1876-7621

  • Volume of the periodical

    31

  • Issue of the periodical within the volume

    2

  • Country of publishing house

    GB - UNITED KINGDOM

  • Number of pages

    10

  • Pages from-to

    45-54

  • UT code for WoS article

  • EID of the result in the Scopus database

    2-s2.0-85212324094