This case report highlights a patient with a leiomyosarcoma originating in the ureter. A chart review was performed on a single patient who presented with a malignant retroperitoneal mass measuring 11.5 × 8.2 × 6.5 cm with subsequent metastasis sites to the breast, pancreas, liver, and lungs. The diagnosis of a leiomyosarcoma is uncommon, accounting for 0.1-0.4% of all cancer diagnoses in the United States. The diagnosis of a leiomyosarcoma originating from the ureter is extremely rare with fewer than 20 reported cases to date. Lack of typical urinary tract cancer signs and symptoms prevented an early presentation, allowing for considerable tumor growth and making complete surgical resection unlikely. We present this case as an example of a rare presentation of a very rare disease and to emphasize the necessity for further research of leiomyosarcoma and early diagnosis.

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http://dx.doi.org/10.1177/00031348221091972DOI Listing

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