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Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.

Akihiko Yoshida^1,2

¹Department of Diagnostic Pathology, National Cancer Center Hospital, Tokyo, Japan.

Pathology International

|December 9, 2022

Summary

This summary is machine-generated.

The World Health Organization classification redefined Ewing sarcoma and Ewing-like sarcomas based on genetic fusions. This review emphasizes morphology and immunohistochemistry for diagnosing these small round cell tumors, especially where molecular testing is limited.

Keywords:

Ewing sarcoma Ewing-like sarcoma diagnosis fusion gene immunohistochemistry

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Area of Science:

Oncology
Pathology
Molecular Diagnostics

Background:

The 5th edition WHO classification redefined Ewing sarcoma based on EWSR1/FUS-ETS fusions.
Ewing-like sarcomas include CIC-rearranged, BCOR-altered, and EWSR1::non-ETS fusion types.
Diagnosis relies on distinct genetics and robust phenotypic characteristics.

Purpose of the Study:

To review the morphological features of Ewing sarcoma and specific Ewing-like sarcomas.
To highlight diagnostic pitfalls and immunohistochemical markers.
To underscore the importance of phenotypic expertise in diagnosis.

Main Methods:

Review of histological findings in Ewing sarcoma and Ewing-like sarcomas.
Discussion of uncommon variations and diagnostic challenges.
Evaluation of immunohistochemical markers (NKX2.2, PAX7, ETV4, BCOR, CCNB3, NKX3.1).

Main Results:

Morphological and phenotypic features are critical for diagnosing Ewing and Ewing-like sarcomas.
Immunohistochemistry aids in differentiating these entities.
Phenotypic expertise complements genetic testing, particularly in resource-limited settings.

Conclusions:

Morphological analysis and immunohistochemistry are vital for accurate diagnosis of Ewing and Ewing-like sarcomas.
Phenotypic expertise can expedite diagnosis and is crucial where molecular testing is inaccessible.
Accurate diagnosis impacts patient management and therapeutic strategies.