BCOR–CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma

Wan Shan Li, I. Chuang Liao, Mei Chin Wen, Howard Haw Chang Lan, Shih Chen Yu, Hsuan Ying Huang

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Aims: BCOR–CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR–CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. Methods and results: By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR–CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years). The tumours originated in the back (n = 2), pelvis (n = 1), and thigh (n = 1), and were 70–140 mm in size (mean, 107 mm). All tumours showed sheets of primitive round or ovoid cells with vesicular nuclei, active mitosis (28–41/10 high-power fields), variably prominent nucleoli, and geographical necrosis. This major component transformed into fascicles of elongated spindle cells with staghorn vessels and a myxoid reticular stroma, accounting for 10–50% of areas. All cases were positive for CD99, three were positive for TLE1, and one was positive for EMA, indicating poorly differentiated synovial sarcomas (PDSSs). Nuclear cyclin B3 reactivity was present in all cases, but not in molecularly confirmed atypical ESs and PDSSs. At the last follow-up (median, 13.5 months), one patient had died of lung metastasis, two were alive with tumours, and one was tumour-free. Conclusions: BCOR–CCNB3-positive sarcomas may primarily occur in soft tissues of adults and show PDSS-mimicking round-cell and spindle-cell histology with aggressive behaviour. Cyclin B3 is useful for selecting candidates for BCOR–CCNB3 molecular testing.

Original languageEnglish
Pages (from-to)792-801
Number of pages10
JournalHistopathology
Volume69
Issue number5
DOIs
Publication statusPublished - 2016 Nov 1

Fingerprint

Synovial Sarcoma
Sarcoma
Histology
Cyclins
Neoplasms
Ewing's Sarcoma
Thigh
Pelvis
Mitosis
Reverse Transcription
Necrosis
Neoplasm Metastasis
Bone and Bones
Polymerase Chain Reaction
Lung

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Histology

Cite this

@article{0fa92eef91824d4a98bf0743fb8682d4,
title = "BCOR–CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma",
abstract = "Aims: BCOR–CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR–CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. Methods and results: By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR–CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years). The tumours originated in the back (n = 2), pelvis (n = 1), and thigh (n = 1), and were 70–140 mm in size (mean, 107 mm). All tumours showed sheets of primitive round or ovoid cells with vesicular nuclei, active mitosis (28–41/10 high-power fields), variably prominent nucleoli, and geographical necrosis. This major component transformed into fascicles of elongated spindle cells with staghorn vessels and a myxoid reticular stroma, accounting for 10–50{\%} of areas. All cases were positive for CD99, three were positive for TLE1, and one was positive for EMA, indicating poorly differentiated synovial sarcomas (PDSSs). Nuclear cyclin B3 reactivity was present in all cases, but not in molecularly confirmed atypical ESs and PDSSs. At the last follow-up (median, 13.5 months), one patient had died of lung metastasis, two were alive with tumours, and one was tumour-free. Conclusions: BCOR–CCNB3-positive sarcomas may primarily occur in soft tissues of adults and show PDSS-mimicking round-cell and spindle-cell histology with aggressive behaviour. Cyclin B3 is useful for selecting candidates for BCOR–CCNB3 molecular testing.",
author = "Li, {Wan Shan} and Liao, {I. Chuang} and Wen, {Mei Chin} and Lan, {Howard Haw Chang} and Yu, {Shih Chen} and Huang, {Hsuan Ying}",
year = "2016",
month = "11",
day = "1",
doi = "10.1111/his.13001",
language = "English",
volume = "69",
pages = "792--801",
journal = "Histopathology",
issn = "0309-0167",
publisher = "Wiley-Blackwell",
number = "5",

}

BCOR–CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology : a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma. / Li, Wan Shan; Liao, I. Chuang; Wen, Mei Chin; Lan, Howard Haw Chang; Yu, Shih Chen; Huang, Hsuan Ying.

In: Histopathology, Vol. 69, No. 5, 01.11.2016, p. 792-801.

Research output: Contribution to journalArticle

TY - JOUR

T1 - BCOR–CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology

T2 - a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma

AU - Li, Wan Shan

AU - Liao, I. Chuang

AU - Wen, Mei Chin

AU - Lan, Howard Haw Chang

AU - Yu, Shih Chen

AU - Huang, Hsuan Ying

PY - 2016/11/1

Y1 - 2016/11/1

N2 - Aims: BCOR–CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR–CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. Methods and results: By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR–CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years). The tumours originated in the back (n = 2), pelvis (n = 1), and thigh (n = 1), and were 70–140 mm in size (mean, 107 mm). All tumours showed sheets of primitive round or ovoid cells with vesicular nuclei, active mitosis (28–41/10 high-power fields), variably prominent nucleoli, and geographical necrosis. This major component transformed into fascicles of elongated spindle cells with staghorn vessels and a myxoid reticular stroma, accounting for 10–50% of areas. All cases were positive for CD99, three were positive for TLE1, and one was positive for EMA, indicating poorly differentiated synovial sarcomas (PDSSs). Nuclear cyclin B3 reactivity was present in all cases, but not in molecularly confirmed atypical ESs and PDSSs. At the last follow-up (median, 13.5 months), one patient had died of lung metastasis, two were alive with tumours, and one was tumour-free. Conclusions: BCOR–CCNB3-positive sarcomas may primarily occur in soft tissues of adults and show PDSS-mimicking round-cell and spindle-cell histology with aggressive behaviour. Cyclin B3 is useful for selecting candidates for BCOR–CCNB3 molecular testing.

AB - Aims: BCOR–CCNB3 sarcoma is a genetically defined undifferentiated malignancy with Ewing sarcoma (ES)-like round cells, and preferentially affects the bones of male adolescents. Sarcomas harbouring BCOR–CCNB3 rarely arise from soft tissues; therefore, we aimed to report four cases to expand the clinicopathological spectrum. Methods and results: By reverse transcription polymerase chain reaction and confirmatory sequencing, we detected a BCOR–CCNB3 transcript in primary undifferentiated sarcomas of the deep musculature of four male patients, comprising two teenagers (aged 14 and 17 years) and two adults (aged 34 and 44 years). The tumours originated in the back (n = 2), pelvis (n = 1), and thigh (n = 1), and were 70–140 mm in size (mean, 107 mm). All tumours showed sheets of primitive round or ovoid cells with vesicular nuclei, active mitosis (28–41/10 high-power fields), variably prominent nucleoli, and geographical necrosis. This major component transformed into fascicles of elongated spindle cells with staghorn vessels and a myxoid reticular stroma, accounting for 10–50% of areas. All cases were positive for CD99, three were positive for TLE1, and one was positive for EMA, indicating poorly differentiated synovial sarcomas (PDSSs). Nuclear cyclin B3 reactivity was present in all cases, but not in molecularly confirmed atypical ESs and PDSSs. At the last follow-up (median, 13.5 months), one patient had died of lung metastasis, two were alive with tumours, and one was tumour-free. Conclusions: BCOR–CCNB3-positive sarcomas may primarily occur in soft tissues of adults and show PDSS-mimicking round-cell and spindle-cell histology with aggressive behaviour. Cyclin B3 is useful for selecting candidates for BCOR–CCNB3 molecular testing.

UR - http://www.scopus.com/inward/record.url?scp=84978802735&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84978802735&partnerID=8YFLogxK

U2 - 10.1111/his.13001

DO - 10.1111/his.13001

M3 - Article

C2 - 27228320

AN - SCOPUS:84978802735

VL - 69

SP - 792

EP - 801

JO - Histopathology

JF - Histopathology

SN - 0309-0167

IS - 5

ER -