Unusual and rare cause of PUJ obstruction with a brief review of the literature

Abstract

Paragangliomas or extra adrenal pheochromocytoma of the genitourinary tract are infrequent and arise from chromaffin cells of the autonomic nervous system. They have been described in the kidney, bladder, prostate, urethra and spermatic cord. However, the occurrence of paragangliomas in the ureter is very unusual. We present one such case in a patient who presented with signs and symptoms of pelvi-ureteric junction obstruction, wherein the initial clinical and imaging findings were deceptive. The patient underwent right-sided open end-to-end uretero-ureterostomy with excision of the strictured segment. Histopathological examination of excised tissue revealed a tumour with clear cell morphology, and in conjunction with immunohistochemistry a final diagnosis of paraganglioma of the ureter was rendered. The case is discussed here with differential diagnosis in the context of its location.