To review our experience with patients with upper urinary tract tumors of nonurothelial origin. Upper urinary tract tumors of nonurothelial origin are uncommon entities. To our knowledge, no series of patients with these neoplasms has been published in the past decade.

We reviewed the records at the University of Texas M. D. Anderson Cancer Center from 1990 to 2004 to identify patients with primary nonurothelial tumors of the upper urinary tract. We reviewed the patient records to collect data on tumor subtype, treatment, recurrence, and survival.

Sixteen patients (1.9% of our database of patients with upper urinary tract tumors) were identified; 12 had squamous cell carcinoma, 2 had adenocarcinoma, 1 had sarcomatoid carcinoma, and 1 had small cell carcinoma. The tumors were located in the renal pelvis in 10 and the ureter in 6. Of the 16 patients, 15 had been treated with nephrectomy or nephrouterectomy and 1 with chemotherapy and radiotherapy. Fifteen of the tumors were pathologic Stage T3 or worse. Ten patients received adjuvant chemotherapy. Of the 16 patients, 9 died (1 of unknown causes), 4 were alive without disease, 2 were alive with disease, and 1 was alive at last follow-up with the disease status unknown. The median follow-up was 30.1 months, the median overall survival time was 11.3 months, and 1-year survival rate was 46%. The median recurrence-free survival time and 1-year recurrence-free survival rate were 5.8 months and 38%, respectively.

Primary nonurothelial carcinomas of the renal pelvis and ureter are rare. Our analysis suggests a poor prognosis for most patients with these pathologic types, probably resulting from the advanced stage at diagnosis and poor responses to systemic therapy.