PMID- 36323544 OWN - NLM STAT- MEDLINE DCOM- 20221107 LR - 20221107 IS - 0529-5807 (Print) IS - 0529-5807 (Linking) VI - 51 IP - 11 DP - 2022 Nov 8 TI - [CIC-rearranged sarcoma with rhabdoid features: a clinicopathological analysis]. PG - 1141-1146 LID - 10.3760/cma.j.cn112151-20220831-00752 [doi] AB - Objective: To investigate the histopathologic, immunohistochemical, molecular genetic characteristics of CIC-rearranged sarcoma (CRS) with rhabdoid features. Methods: The clinical and pathologic data of two cases of CRS diagnosed between 2019 and 2021 at the Department of Pathology, Jiangsu Province Hospital were analyzed. Immunohistochemical study and fluorescence in situ hybridization (FISH) were performed. The relevant literature was reviewed. Results: Both patients were female, one was 58 years old, with tumor located in left thigh; the other was 43 years old, with tumor located in left pelvic cavity. Microscopically, both tumors were composed of small to medium-sized round, oval cells, arranged in nodules or sheets. The tumor cells showed irregular nuclear outline, coarse chromatin with prominent nucleoli and brisk mitotic activity. Both cases showed rhabdoid phenotype with myxoid stromal changes. Immunohistochemically, both cases were positive for CD99 and c-myc. High WT1 reactivity was seen in classic area, with low reactivity in rhabdoid area. There was no INI1 lost in both cases. Both were negative for NKX2.2 and NKX3.1. By FISH both cases demonstrated convincing break-apart signals of CIC gene. One patient died of disease after 1 month, and the other died of disease after 3 months. Conclusions: CRS is a small round cell undifferentiated sarcoma of the bone and soft tissue defined by molecular genetic characteristics, and may show atypical morphologic and immunophenotypic characteristics such as rhabdoid features. A correct understanding of its rare morphologic and immunophenotypic characteristics, combined with molecular pathologic detection, is conducive to correct diagnosis. FAU - Pan, B J AU - Pan BJ AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Gong, Q X AU - Gong QX AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Li, H AU - Li H AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Ma, S Y AU - Ma SY AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Song, G X AU - Song GX AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Li, X AU - Li X AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Ding, Y AU - Ding Y AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Fan, Q H AU - Fan QH AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. FAU - Zhang, Z H AU - Zhang ZH AD - Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China. LA - chi PT - English Abstract PT - Journal Article PL - China TA - Zhonghua Bing Li Xue Za Zhi JT - Zhonghua bing li xue za zhi = Chinese journal of pathology JID - 0005331 RN - 0 (Biomarkers, Tumor) RN - 0 (Transcription Factors) SB - IM MH - Female MH - Humans MH - Male MH - Biomarkers, Tumor/genetics/analysis MH - In Situ Hybridization, Fluorescence MH - *Sarcoma/pathology MH - *Sarcoma, Small Cell/diagnosis/genetics/pathology MH - Transcription Factors/genetics MH - Rhabdoid Tumor/diagnosis/genetics/pathology EDAT- 2022/11/03 06:00 MHDA- 2022/11/05 06:00 CRDT- 2022/11/02 22:38 PHST- 2022/11/02 22:38 [entrez] PHST- 2022/11/03 06:00 [pubmed] PHST- 2022/11/05 06:00 [medline] AID - 10.3760/cma.j.cn112151-20220831-00752 [doi] PST - ppublish SO - Zhonghua Bing Li Xue Za Zhi. 2022 Nov 8;51(11):1141-1146. doi: 10.3760/cma.j.cn112151-20220831-00752.