Ureteral amyloidosis is a unique and infrequent form of amyloidosis characterized by the deposition of amyloid proteins within the ureters.

These tubes, responsible for transporting urine from the kidneys to the bladder, can become obstructed due to this protein accumulation, potentially leading to renal complications. We are presenting a case ureteral amyloidosis.

A 48-year-old male with no known prior medical conditions presented with a three-month history of right-sided pain, frequent and painful urination, reduced urine output, and hematuria. Blood tests showed a hemoglobin level of 12.8 g/dL and MCV of 73. Urinalysis revealed pyuria and hematuria. An upright abdominal X-ray indicated hydronephrosis, and an abdominal CT scan detected grade 2 hydronephrosis on the right side and a suspicious mass in a 1 cm segment of the distal right ureter outside the bladder. The mass was excised, and histological examination with crystal violet and Congo red staining showed a strong positive reaction for amyloid. Immunohistochemical analysis confirmed the diagnosis of lambda light chain amyloidoma. Systemic screening for amyloid deposition was negative except for the ureter. Nine months post-operation, the patient returned with recurrent pain and oliguria. A CT scan revealed a mass at the excision site, consistent with lambda light chain amyloidoma. Considering it a recurrent disease, the patient underwent intensity-modulated radiation therapy (IMRT) with a total dose of 20 Gy in 10 fractions of 2 Gy each. Two months post-radiation, with recurring symptoms, the patient received four cycles of bortezomib-dexamethasone treatment. Post-treatment, the patient's symptoms improved, and CT imaging showed the disappearance of the mass lesion.

Ureteral amyloidosis, though rare, can present with significant clinical symptoms. Early detection and a combination of surgical and medical interventions, as demonstrated in this case, can lead to symptom resolution and improved patient outcomes.