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[CIC-rearranged sarcoma with rhabdoid features: a clinicopathological analysis]
1Department of Pathology, Jiangsu Province People's Hospital (the First Affiliated Hospital of Nanjing Medical University), Nanjing 210029, China.
Zhonghua Bing Li Xue Za Zhi = Chinese Journal of Pathology
|November 2, 2022
Summary
CIC-rearranged sarcoma (CRS) is a rare bone and soft tissue cancer that can present with rhabdoid features. Accurate diagnosis requires understanding its unique morphology and molecular characteristics.
Area of Science:
- Oncology
- Pathology
- Molecular Genetics
Context:
- CIC-rearranged sarcoma (CRS) is an uncommon bone and soft tissue malignancy.
- Rhabdoid features can present atypically in CRS.
- Accurate diagnosis is crucial for patient outcomes.
Purpose:
- To investigate the histopathologic, immunohistochemical, and molecular genetic features of CIC-rearranged sarcoma (CRS) with rhabdoid morphology.
- To analyze two cases of CRS with rhabdoid features.
- To review relevant literature on CRS.
Summary:
- Two female patients with CRS and rhabdoid features were analyzed.
- Tumors exhibited small round to medium-sized cells with rhabdoid phenotype and myxoid changes.
- Immunohistochemistry showed CD99 and c-myc positivity, with variable WT1 reactivity and no INI1 loss.
- Fluorescence in situ hybridization (FISH) confirmed CIC gene rearrangements in both cases.
- Both patients had poor prognoses, succumbing to the disease within months.
Impact:
- Highlights the importance of recognizing rare morphologic and immunophenotypic variations in CRS.
- Emphasizes the necessity of molecular pathologic detection for accurate diagnosis.
- Contributes to a better understanding of this aggressive sarcoma subtype.
