Key result
First renal CIC-rearranged sarcoma case confirms LEUTX as the novel gene fusion partner.
Why the study?
CIC-rearranged sarcomas are heterogeneous, highly invasive tumors with poor prognosis that mostly occur in limb and trunk soft tissues, making primary renal presentations rare.
Case Report (n=1)
This case report describes the first known instance of a primary renal CIC-LEUTX rearranged sarcoma, highlighting its aggressive clinical course and poor response to therapy.
Answers are grounded in this paper and cite their sources.
Supports molecular testing in atypical renal sarcomas; leaves open optimal therapy for CIC-LEUTX cases.
BACKGROUND: CIC-rearranged sarcomas (CRS) are a group of heterogeneous tumors which mostly occur in the soft tissues of limbs and trunk, and are highly invasive with poor prognosis. Here, we describe a rare case of CRS that occurred in the left kidney with a CIC-LEUTX rearrangement. CASE PRESENTATION: A 45-year-old male was admitted to hospital with a dry cough for more than two months without obvious cause. Physical examination and laboratory tests revealed no notable abnormality. The CT scan demonstrated a mass in the left kidney and multiple nodules in both lungs. The percutaneous core needle biopsy showed similar histomorphology and immunophenotype of small round cell malignant tumors. Genetic test revealed a CIC-LEUTX gene fusion. CONCLUSIONS: We present a rare primary renal CRS with multiple pulmonary metastases, and LEUTX is confirmed as the fusion partner of CIC gene for the first time in a renal case.
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Tang et al. (2023) conducted a case report in Renal CIC-LEUTX rearranged sarcoma (n=1). A rare primary renal CIC-rearranged sarcoma with multiple pulmonary metastases was identified, confirming LEUTX as the fusion partner of the CIC gene for the first time in a renal case.
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