Composite paraganglioma-ganglioneuroma of the urinary bladder

Presented herein is the case of a 73-year-old man, complaining of dysuria, who had a composite paraganglioma-ganglioneuroma of the urinary bladder (CPGUB). At cystoscopy a submucosal tumor was found in the urinary bladder and resected after transurethral biopsy. The levels of serum catecholamine and 24 h urinary excretion of catecholamine and vanillylmandelic acid were elevated. Grossly, the resected tumor, measuring 4 x 3 x 2.5 cm, had a brownish cut surface with no necrosis and hemorrhage. Histologically, the tumor had alternating cellular and fibrous areas. The cellular areas consisted of polygonal cells, arranged in well-defined nests (Zellballen) and positive for Grimelius staining, with abundant amphophilic to acidophilic cytoplasm, occasionally containing eosinophilic hyaline globules and brown pigments. Although the nuclei of several polygonal cells were bizarre, mitoses and vascular invasion were not found. Fibrous areas consisted of spindle cells, resembling Schwann cell, admixed with ganglionic cells. To the authors' knowledge, only four cases of CPGUB have been reported in the English-language literature. Detailed reported cases and the present case showed no malignant features, such as extra-bladder infiltration and metastasis, and no recurrence in the short length of follow up. Accumulation of long-term follow-up cases may provide valuable prognostic information on this composite tumor.