A 55-year-old man was referred to our department with the chief complaint of left flank pain. Computed tomography and magnetic resonance imaging demonstrated a left hydronephroureter due to the ureteral stenosis with a mass. We considered the possibility of a malignant neoplasm, and performed laparoscopic left total nephroureterectomy. Microscopic appearance showed ureteral wall thickening and perivascular deposition of heterogeneous amyloid. It stained positive by immune-histochemical staining using Congo-red. In addition, it stained positive by immune-histochemical staining with an anti-AA antibody. These findings indicated that the amyloid was type AA. AA amyloidosis is a systemic amyloidosis that arises secondarily to an inflammatory disease. He had been treated for systemic lupus erythematosus. It is compatible to secondary amyloidosis. Eighty seven months after diagnosis, he died of lung cancer. There were no signs or symptoms of deposition of the AA amyloid proteins.

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