Clinicopathologic Diversity of Undifferentiated Sarcoma with BCOR-CCNB3 Fusion

Atsuji Matsuyama, Eisuke Shiba, Yoshihisa Umekita, Kanae Nosaka, Takihiro Kamio, Hiroyuki Yanai, Chika Miyasaka, Reiko Watanabe, Ichiro Ito, Tomoko Tamaki, Shinichi Hayashi, Masanori Hisaoka

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12 Citations (Scopus)

Abstract

Undifferentiated sarcoma harboring the BCOR-CCNB3 fusion is characterized by its predilection to affect skeletons of adolescent males, cellular small round/spindle cell morphology, and CCNB3 immunoreactivity. We analyzed 11 cases of BCOR-CCNB3 sarcoma, 10 of which were identified in a reverse transcription-polymerase chain reaction-based screen of 85 patient samples recorded in our database as unclassified small round or spindle cell sarcomas. BCOR rearrangements were confirmed by fluorescence in situ hybridization in 8 tumors. All patients were males aged between 6 and 31 years. In addition to 5 tumors in soft tissue and 4 in the axial or appendicular skeletons, which are typical locations, a tumor was located in the paranasal sinus and another in the lung. Microscopically, the tumors comprised proliferating atypical spindle and/or small round cells with diverse morphologic features such as small concentric whorls, myxoid stroma, a hemangiopericytomatous appearance, and/or hyalinized collagen resembling a solitary fibrous tumor, and angiomatous or slit-like spaces containing extravasated erythrocytes. Tumor cells were immunoreactive to CCNB3 (9/11), BCOR (10/10), TLE1 (6/10), bcl-2 (9/11), CD99 (8/10), CD56 (8/10), c-kit (4/10), and cyclin D1 (10/10). In an immunohistochemical analysis of an additional 412 small round or spindle cell tumors, CCNB3 was detected in 6 (1.5%) and BCOR in 18 (4.4%). Our analysis highlights the varying clinicopathologic features of this tumor, which partially overlap with other small round or spindle cell tumors, including solitary fibrous tumor and vascular tumors. Because CCNB3 and BCOR immunohistochemistry lacks adequate sensitivity and specificity, a molecular genetic approach remains essential for diagnosis.

Original languageEnglish
Pages (from-to)1713-1721
Number of pages9
JournalAmerican Journal of Surgical Pathology
Volume41
Issue number12
DOIs
Publication statusPublished - Jan 1 2017

Fingerprint

Sarcoma
Neoplasms
Solitary Fibrous Tumors
Skeleton
Small Cell Sarcoma
Paranasal Sinuses
Cyclin D1
Fluorescence In Situ Hybridization
Reverse Transcription
Blood Vessels
Molecular Biology
Collagen
Erythrocytes
Immunohistochemistry
Databases
Sensitivity and Specificity
Polymerase Chain Reaction
Lung

Keywords

  • BCOR-CCNB3
  • FISH
  • immunohistochemistry
  • RT-PCR
  • undifferentiated sarcoma

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Clinicopathologic Diversity of Undifferentiated Sarcoma with BCOR-CCNB3 Fusion. / Matsuyama, Atsuji; Shiba, Eisuke; Umekita, Yoshihisa; Nosaka, Kanae; Kamio, Takihiro; Yanai, Hiroyuki; Miyasaka, Chika; Watanabe, Reiko; Ito, Ichiro; Tamaki, Tomoko; Hayashi, Shinichi; Hisaoka, Masanori.

In: American Journal of Surgical Pathology, Vol. 41, No. 12, 01.01.2017, p. 1713-1721.

Research output: Contribution to journalArticle

Matsuyama, A, Shiba, E, Umekita, Y, Nosaka, K, Kamio, T, Yanai, H, Miyasaka, C, Watanabe, R, Ito, I, Tamaki, T, Hayashi, S & Hisaoka, M 2017, 'Clinicopathologic Diversity of Undifferentiated Sarcoma with BCOR-CCNB3 Fusion', American Journal of Surgical Pathology, vol. 41, no. 12, pp. 1713-1721. https://doi.org/10.1097/PAS.0000000000000934
Matsuyama, Atsuji ; Shiba, Eisuke ; Umekita, Yoshihisa ; Nosaka, Kanae ; Kamio, Takihiro ; Yanai, Hiroyuki ; Miyasaka, Chika ; Watanabe, Reiko ; Ito, Ichiro ; Tamaki, Tomoko ; Hayashi, Shinichi ; Hisaoka, Masanori. / Clinicopathologic Diversity of Undifferentiated Sarcoma with BCOR-CCNB3 Fusion. In: American Journal of Surgical Pathology. 2017 ; Vol. 41, No. 12. pp. 1713-1721.
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