Undifferentiated round cell sarcomas of the skin

Kód výsledku v 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>

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Undifferentiated round cell sarcomas of the skin

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Undifferentiated round cell sarcomas of the skin

Popis výsledku anglicky

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.

Druh

J_SC - Článek v periodiku v databázi SCOPUS

CEP obor

—

OECD FORD obor

30109 - Pathology

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2025

Kód důvěrnosti údajů

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

Název periodika

Diagnostic Histopathology

ISSN

1756-2317

e-ISSN

1876-7621

Svazek periodika

31

Číslo periodika v rámci svazku

2

Stát vydavatele periodika

GB - Spojené království Velké Británie a Severního Irska

Počet stran výsledku

10

Strana od-do

45-54

Kód UT WoS článku

—

EID výsledku v databázi Scopus

2-s2.0-85212324094