Cytological features of BCOR-CCNB3 sarcoma: Comparison with Ewing sarcoma and synovial sarcoma

Cytopathology. 2021 Nov;32(6):771-778. doi: 10.1111/cyt.13034. Epub 2021 Jul 31.

Abstract

Introduction: BCOR-CCNB3 sarcoma (BCS) is one of the histological types classified as an undifferentiated small round cell sarcoma of bone and soft tissue. This sarcoma frequently develops in males under 20 years of age. Histologically, a delicate capillary network has been reported as a conspicuous finding. In this study, the cytological findings of BCS were observed in two cases of primary lesions and one case of a lung metastatic lesion. The cytological findings of BCS were compared with its histological mimics, and the characteristic findings of BCS were examined.

Methods: Three cases of BCS were studied, and a cytological comparison was performed with 8 cases of Ewing sarcoma (ES) and 10 cases of synovial sarcoma (SS; monophasic type: 7 cases, biphasic type: 2 cases, poorly differentiated: 1 case).

Results: In all BCS cases, small clusters with thin and delicate vascular cores and tiny vascular fragments were conspicuous. In ES and SS cases, although small clusters with vascular cores were observed, the vascular cores were thicker than in BCS, and no tiny vascular fragments appeared in most cases. Cytomorphological differences of tumour cells were also observed among BCS, ES, and SS. Predominantly rounded nuclei with fine chromatin and inconspicuous nucleoli can be cytological clues for BCS.

Conclusions: BCS shows characteristic cytological findings that make the diagnosis of BCS more likely than that of ES and SS. Cytological evaluation is a useful tool for appropriate differential diagnosis that leads to a more accurate final diagnosis and rapid treatment.

Keywords: BCOR-CCNB3 sarcoma; Ewing sarcoma; Ewing-like sarcoma; cytomorphology; small round cell sarcoma; synovial sarcoma.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Biomarkers, Tumor / analysis
  • Buttocks / diagnostic imaging
  • Buttocks / pathology
  • Cyclin B / analysis
  • Diagnosis, Differential
  • Femur / diagnostic imaging
  • Femur / pathology
  • Heel / diagnostic imaging
  • Heel / pathology
  • Humans
  • Immunohistochemistry
  • Male
  • Proto-Oncogene Proteins / analysis
  • Repressor Proteins / analysis
  • Sarcoma* / diagnosis
  • Sarcoma* / pathology
  • Sarcoma, Ewing* / diagnosis
  • Sarcoma, Ewing* / pathology
  • Sarcoma, Synovial* / diagnosis
  • Sarcoma, Synovial* / pathology
  • Soft Tissue Neoplasms / diagnosis
  • Soft Tissue Neoplasms / pathology

Substances

  • BCOR protein, human
  • Biomarkers, Tumor
  • CCNB3 protein, human
  • Cyclin B
  • Proto-Oncogene Proteins
  • Repressor Proteins