Myxoid pseudotumor is a pseudoneoplastic fibroblastic proliferation that has been described in the perinephric and renal sinus fat tissue. It is characterized by the presence of a myxoid matrix, intermingled with the adipocytes, and a hypocellular population of spindle-shaped and stellate cells. We report a myxoid pseudotumor involving the distal ureter, which broadens the spectrum of possible localizations of this lesion around the urinary tract. It occurred in an 80-year-old patient who underwent a
nephroureterectomy indicated after an incidental radiological finding of a thickening of the distal left ureter wall which suggested a
ureteral neoplasm. He had two voided urine and one ureteroscopic sample cytologies diagnosed as high-grade urothelial
carcinoma, as well as a retrograde ureteroscopy ureteral biopsy which was diagnosed as urothelial
carcinoma in situ. This emphasizes the problem of the possible misdiagnosis of myxoid pseudotumor as a ureteral infiltrative
carcinoma due to the radiological findings being badly interpreted, compounded by the preoperative cytohistologic data on
malignancy. A diffuse urothelial
carcinoma in situ was seen in our specimen without infiltrative or papillary
tumors. This would not support an obstructive pathogenetic mechanism as has been hypothesized for myxoid pseudotumor.