Primary capicua transcriptional repressor-rearranged sarcoma of the lung

Yasushi Mizukami; Yuki Takahashi; Shintaro Sugita; Kento Wakabayashi; Hiroaki Suzuki; Tadashi Hasegawa; Hiroko Noguchi; Hirofumi Adachi

doi:10.1093/jjco/hyaa240

Primary capicua transcriptional repressor-rearranged sarcoma of the lung

Jpn J Clin Oncol. 2021 Apr 1;51(4):654-656. doi: 10.1093/jjco/hyaa240.

Authors

Yasushi Mizukami¹, Yuki Takahashi¹, Shintaro Sugita², Kento Wakabayashi³, Hiroaki Suzuki³, Tadashi Hasegawa², Hiroko Noguchi⁴, Hirofumi Adachi¹

Affiliations

¹ Department of Thoracic Surgery, National Hospital Organization, Hokkaido Cancer Center, Sapporo, Japan.
² Department of Surgical Pathology, Sapporo Medical University Hospital, Sapporo, Japan.
³ Department of Pathology, National Hospital Organization, Hokkaido Cancer Center, Sapporo, Japan.
⁴ Department of Pathology, Kimitsu Chuo Hospital, Kisarazu, Japan.

PMID: 33338234
DOI: 10.1093/jjco/hyaa240

Abstract

A 60-year-old man had a malignant left lower lung tumour with no metastases and underwent video-assisted thoracoscopic left lower lobectomy and lymphadenectomy. Pathological examination led to a diagnosis of capicua transcriptional repressor (CIC)-rearranged sarcoma. He has had 3.5 years of recurrence-free survival. CIC-rearranged sarcoma is a Ewing-like sarcoma that shows pathological findings similar to Ewing sarcoma. Most of CIC-rearranged sarcoma is CIC-double homeobox 4 protein (DUX4) fusion. Pulmonary CIC-rearranged sarcoma is extremely rare and has an unfavourable prognosis. However, complete resection can produce prognosis of long-term survival, and thus, surgery is an important option.

Keywords: CIC-rearranged sarcoma; lung pathology; lung sarcoma.

Publication types

Case Reports

MeSH terms

Gene Rearrangement*
Humans
Lung Neoplasms / diagnostic imaging
Lung Neoplasms / genetics*
Male
Middle Aged
Repressor Proteins / genetics*
Sarcoma / diagnostic imaging
Sarcoma / genetics*
Sarcoma / pathology

Substances

CIC protein, human
Repressor Proteins