Fine-Needle Aspiration Features of BCOR-CCNB3 Sarcoma

Author:

Gajdzis Pawel12,Laé Marick13,Pierron Gaëlle1,Brisse Hervé J4,Orbach Daniel56,Fréneaux Paul1,Laurence Valerie7,Klijanienko Jerzy1

Affiliation:

1. Department of Pathology, Institut Curie, Paris, France

2. Department of Pathomorphology and Oncological Cytology, Medical University of Wroclaw, Wroclaw, Poland

3. Service de Pathologie, Centre Henri Becquerel, Rouen, France

4. Department of Radiology, Institut Curie, Paris, France

5. SIREDO (Care, Innovation and Research for Children, Adolescents and Young Adults With Cancer) Oncology Center, Institut Curie, Paris, France

6. PSL University, Paris, France

7. Department of Medical Oncology, Institut Curie, Paris, France

Abstract

Abstract Objectives BCOR-CCNB3 sarcoma (BCS) is an undifferentiated tumor that has some clinical and morphologic similarity to classic Ewing sarcoma, but it is characterized by a distinct BCOR-CCNB3 gene fusion. There are no reports describing its cytomorphologic findings. Methods We describe cytologic findings of five molecularly proven BCS cases (four males and one female, aged 8.5-22 years). Results Smears showed mainly round cells, but some spindle cells and rhabdoid-like cells were also observed. Dispersed cells dominated in smears, but also distinct pseudopapillary structures with vascular cores were noted in four cases. Scant connective tissue fragments were found in four cases. There was no rosette formation in any case. Conclusions BCS should be differentiated from other round cell tumors. Some cytologic features, especially rhabdoid-like cells, connective tissue fragments, and pseudopapillary formations, combined with immunohistochemical and molecular studies, may be helpful in making the appropriate diagnosis.

Publisher

Oxford University Press (OUP)

Subject

General Medicine

Reference33 articles.

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2. High prevalence of CIC fusion with double-homeobox (DUX4) transcription factors in EWSR1-negative undifferentiated small blue round cell sarcomas;Italiano;Genes Chromosomes Cancer.,2012

3. The Ewing family of tumors—a subgroup of small-round-cell tumors defined by specific chimeric transcripts;Delattre;N Engl J Med.,1994

4. BCOR-CCNB3 fusion positive sarcomas: a clinicopathologic and molecular analysis of 36 cases with comparison to morphologic spectrum and clinical behavior of other round cell sarcomas;Kao;Am J Surg Pathol.,2018

5. Robust diagnosis of Ewing sarcoma by immunohistochemical detection of super-enhancer-driven EWSR1-ETS targets;Baldauf;Oncotarget.,2018

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