Low grade fibromyxoid sarcoma is an uncommon soft tissue tumor.
We present the case of a 83–year-old female who developed large mass in the right kidney. A nephrectomy was performed. The tumor measured 18,5×17,5×11,5 cm and on pathology evaluation was diagnosed as low grade fibromyxoid sarcoma. Currently, the patient is doing well without evidence of local recurrence and distant metastasis.
Low grade fibromyxoid sarcoma of the Kidney is a very rare tumor. The metastasizing potential is high. Sometimes long interval between tumor presentation and metastasis is observed. The treatment of choice is surgery. The role of radiotherapy and chemotherapy is uncertain.
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