CIC-rearranged Sarcomas

Authors:
  • AYAkihiko Yoshida
    Akihiko Yoshida
  • MKMakoto Kodaira
    Makoto Kodaira
  • EKEisuke Kobayashi
    Eisuke Kobayashi
  • HKHiroshi Kawamoto
    Hiroshi Kawamoto
  • TMTaisuke Mori
    Taisuke Mori
  • SYSeiichi Yoshimoto
    Seiichi Yoshimoto
  • OEOtone Endo
    Otone Endo
  • NKNarihito Kodama
    Narihito Kodama
  • RKRyoji Kushima
    Ryoji Kushima
  • NHNobuyoshi Hiraoka
    Nobuyoshi Hiraoka
  • TMToru Motoi
    Toru Motoi
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Abstract
The CIC gene rearrangement exists in a subset of small round cell sarcomas. As the nosologic relationship of these sarcomas to Ewing sarcomas remains undetermined, we examined 20 CIC-rearranged sarcomas to compare their clinicopathologic features with those of Ewing sarcomas. The CIC-rearranged sarcomas were from a group of 14 men and 6 women with a median age of 24.5 years. The primary tumor sites included the limbs, trunk wall, internal trunk, lung, cerebrum, and pharynx. A comparison of the demographic and clinical characteristics of the 20 patients with CIC-rearranged sarcomas with those of the 53 near-consecutive patients with EWSR1-rarranged Ewing sarcomas showed that there were no differences with respect to their ages and sexes. Although none of the CIC-rearranged sarcomas arose in the bone, 40% of the Ewing sarcomas primarily affected the skeleton. The overall survival of patients with Ewing sarcomas was significantly better than that for patients with CIC-rearranged sarcomas. A histologic comparison of the CIC-rearranged sarcomas with 20 EWSR1-rearranged Ewing sarcomas showed significantly higher degrees of lobulation, nuclear pleomorphism, the prominence of the nucleoli, spindle cell elements, and myxoid changes in the CIC-rearranged sarcomas. Distinguishing immunohistochemical features included heterogenous CD99 reactivity, nuclear WT1 expression, and calretinin expression in the CIC-rearranged sarcomas and NKX2.2 expression in the Ewing sarcomas. CIC-rearranged sarcomas are distinct from Ewing sarcomas clinically, morphologically, and immunohistochemically, and they should be considered a separate entity rather than being grouped within the same family of tumors.

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