Sung Han Kim, Yoon Seok Suh, Jung Kwon Kim, Weon Seo Park, Seog Yun Park, Ji Hyeon Lee, Sun Ho Kim, Jae Young Joung, Ho Kyung Seo, Kang Hyun Lee, and Jinsoo Chung
A 45-year-old previously healthy woman presented to our hospital with a right abdominal mass. Abdominal computed tomography (CT), magnetic resonance imaging, and bone scanning revealed a 15-×10-×14-cm, welldelineated, retroperitoneal tumor originating from the right kidney. No metastases detected. The patient underwent successful extirpation of the renal tumor. The tumor showed 50% necrosis without perirenal fat infiltration, and it was diagnosed as a high-grade (grade 3) myxofibrosarcoma without nodal positivity, according to the French Federation of Cancer Centers Sarcoma (FNCLCC) grading system. Tumor immunohistochemical staining revealed positive staining for CD34, Ki67, smooth muscle actin (SMA), and cluster of differentiation 68 (CD68), and negative staining for S100 protein, desmin, mast/stem cell growth factor receptor (c-kit), and melanoma marker antibody (HMB45). Three months post-surgery, follow-up CT revealed no new abdominal metastasis, and the patient is currently receiving routine follow-up without any additional systemic therapy. Myxofibrosarcoma of the kidney is an uncommon soft tissue tumor, and radical surgery is the treatment of choice. Long-term follow-up is recommended because of the tumor’s aggressive invasiveness and potential for distant metastasis.
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