Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male
mimicking low-grade fibromyxoid sarcoma - A case report

J 2023

Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male mimicking low-grade fibromyxoid sarcoma - A case report

SOUKUP, Jiri, Ondrej VALTR, Jindra BRTKOVA, Zdenek ZOUL, Iva STANICZKOVÁ ZAMBO et. al.

Basic information

Original name

Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male mimicking low-grade fibromyxoid sarcoma - A case report

Authors

SOUKUP, Jiri (203 Czech Republic), Ondrej VALTR (203 Czech Republic), Jindra BRTKOVA (203 Czech Republic), Zdenek ZOUL (203 Czech Republic), Iva STANICZKOVÁ ZAMBO (203 Czech Republic, belonging to the institution), Jan HOJNY (203 Czech Republic) and Katerina KAMARADOVA (203 Czech Republic)

Edition

Pathology Research and Practice, MUNICH, ELSEVIER GMBH, 2023, 0344-0338

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30109 Pathology

Country of publisher

Germany

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 2.800 in 2022

RIV identification code

RIV/00216224:14110/23:00133286

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1016/j.prp.2023.154831

UT WoS

001098794100001

Keywords in English

BCOR; Sarcoma; Ossifying fibromyxoid tumour; Soft tissue

Abstract

V originále

Diagnosis of soft tissue tumors is often challenging, given the large number of entities, often with non-specific or overlapping morphology. Although morphology still plays an important part in diagnostic process, additional studies including immunohistochemistry and molecular genetics are often needed to arrive at correct diagnosis. We report a case of 61-year-old male with subcutaneous tumor in right hip area, that was surgically removed. The tumor was composed of uniform bland spindle cells in mild to moderately cellular myxoid nodules, with limited areas of collagenization and the diagnosis of low grade fibromyxoid sarcoma was made. The tumor recurred 3 years after the initial diagnosis and the new sample showed a high-grade round cell sarcoma with limited residual low-grade areas and non-specific immunoprofile after extended immunohistochemical work-up. Molecular analysis demonstrated ZC3H7B::BCOR fusion. Sarcomas with ZC3H7B::BCOR fusion occurring outside of uterus are exceedingly rare. A comprehensive review of previously published cases and a short discussion about classification of the entity is provided, together with data about morphology and immunoprofile of the lesions. The case also underscores the necessity of extended work up of soft tissue tumors with unusual immunohistochemical or morphological features in order to accurately assess their biological potential.

Links

LM2023033, research and development project

Name: Síť českých biobank

Investor: Ministry of Education, Youth and Sports of the CR

Citovat

SOUKUP, Jiri, Ondrej VALTR, Jindra BRTKOVA, Zdenek ZOUL, Iva STANICZKOVÁ ZAMBO, Jan HOJNY and Katerina KAMARADOVA. Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male mimicking low-grade fibromyxoid sarcoma - A case report. Pathology Research and Practice. MUNICH: ELSEVIER GMBH, 2023, vol. 251, November 2023, p. 1-5. ISSN 0344-0338. Available from: https://dx.doi.org/10.1016/j.prp.2023.154831.

@article{2367137,
   author = {Soukup, Jiri and Valtr, Ondrej and Brtkova, Jindra and Zoul, Zdenek and Staniczková Zambo, Iva and Hojny, Jan and Kamaradova, Katerina},
   article_location = {MUNICH},
   article_number = {November 2023},
   doi = {http://dx.doi.org/10.1016/j.prp.2023.154831},
   keywords = {BCOR; Sarcoma; Ossifying fibromyxoid tumour; Soft tissue},
   language = {eng},
   issn = {0344-0338},
   journal = {Pathology Research and Practice},
   title = {Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male mimicking low-grade fibromyxoid sarcoma - A case report},
   url = {https://www.sciencedirect.com/science/article/pii/S0344033823005319?via%3Dihub},
   volume = {251},
   year = {2023}
}

TY  - JOUR
ID  - 2367137
AU  - Soukup, Jiri - Valtr, Ondrej - Brtkova, Jindra - Zoul, Zdenek - Staniczková Zambo, Iva - Hojny, Jan - Kamaradova, Katerina
PY  - 2023
TI  - Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male mimicking low-grade fibromyxoid sarcoma - A case report
JF  - Pathology Research and Practice
VL  - 251
IS  - November 2023
SP  - 1-5
EP  - 1-5
PB  - ELSEVIER GMBH
SN  - 03440338
KW  - BCOR
KW  - Sarcoma
KW  - Ossifying fibromyxoid tumour
KW  - Soft tissue
UR  - https://www.sciencedirect.com/science/article/pii/S0344033823005319?via%3Dihub
N2  - Diagnosis of soft tissue tumors is often challenging, given the large number of entities, often with non-specific or overlapping morphology. Although morphology still plays an important part in diagnostic process, additional studies including immunohistochemistry and molecular genetics are often needed to arrive at correct diagnosis. We report a case of 61-year-old male with subcutaneous tumor in right hip area, that was surgically removed. The tumor was composed of uniform bland spindle cells in mild to moderately cellular myxoid nodules, with limited areas of collagenization and the diagnosis of low grade fibromyxoid sarcoma was made. The tumor recurred 3 years after the initial diagnosis and the new sample showed a high-grade round cell sarcoma with limited residual low-grade areas and non-specific immunoprofile after extended immunohistochemical work-up. Molecular analysis demonstrated ZC3H7B::BCOR fusion. Sarcomas with ZC3H7B::BCOR fusion occurring outside of uterus are exceedingly rare. A comprehensive review of previously published cases and a short discussion about classification of the entity is provided, together with data about morphology and immunoprofile of the lesions. The case also underscores the necessity of extended work up of soft tissue tumors with unusual immunohistochemical or morphological features in order to accurately assess their biological potential.
ER  -

SOUKUP, Jiri, Ondrej VALTR, Jindra BRTKOVA, Zdenek ZOUL, Iva STANICZKOVÁ ZAMBO, Jan HOJNY and Katerina KAMARADOVA. Soft tissue sarcoma with ZC3H7B::BCOR fusion in a male mimicking low-grade fibromyxoid sarcoma - A case report. \textit{Pathology Research and Practice}. MUNICH: ELSEVIER GMBH, 2023, vol.~251, November 2023, p.~1-5. ISSN~0344-0338. Available from: https://dx.doi.org/10.1016/j.prp.2023.154831.

Detailed Information on Publication Record