Malignant non-functional paraganglioma of the bladder presenting with azotemia

Paragangliomas of the urinary bladder are rare tumors representing less than 1% of bladder tumors and are usually benign. Malignant paragangliomas are uncommon and are defined by their clinical behavior rather than the histologic features. We describe a patient with recurrent nonfunctioning paraganglioma of bladder presenting with hematuria and obstructive uropathy due to involvement of ureteroileal anastomoses. Treatment consisted of excision of recurrent lesion involving both lower ureters with the revision of ureteroileal anastomoses. Histology confirmed the presence of nests of tumor cells with abundant eosinophilic cytoplasm. Immunohistochemically the tumor cells were strongly positive for chromogranin A.