Extra-adrenal paraganglioma of a urinary bladder in an adolescent male: A rare case report

Int J Surg Case Rep. 2021 Dec:89:106535. doi: 10.1016/j.ijscr.2021.106535. Epub 2021 Oct 30.

Abstract

Introduction and importance: Paraganglioma of the urinary bladder is unusual and reported rarely. The patient usually presents with the complaint of hematuria and fluctuating blood pressure.

Case presentation: We discuss the case of a 21-year old male, who had been experiencing gross hematuria, was found to have a mass on ultrasonography. Further evaluation with contrast-enhanced computerized tomography (CECT) revealed an irregular soft tissue density of endophytic mass arising from the left posterior wall of the urinary bladder. The histopathological examination of the excised mass was suggestive of a tumor of neural origin, which was further confirmed as paraganglioma by immunohistochemistry.

Clinical discussion: Imaging prior to the surgery must be done for a provisional diagnosis of paraganglioma to avoid fluctuating blood pressure during elective surgery. On histopathological examination tumor cells are arranged in the nest like fashion forming a specific 'Zellballen' pattern. Positive staining for synaptophysin and chromogranin in immunohistochemistry confirms the diagnosis.

Conclusion: It is difficult to diagnose paraganglioma of the urinary bladder with the aid of imaging only, particularly if the patient presents without specific symptoms of fluctuating blood pressure. So, a multidisciplinary approach is essential for the diagnosis and proper therapy of this entity. However, prompt surgical resection is the mainstay of treatment.

Keywords: Endophytic; Paraganglioma; Zellballen.