Differences in clinical presentation and management between pre- and postsurgical diagnoses of urinary bladder paraganglioma: is there clinical relevance? A systematic review

Abstract

Purpose Paraganglioma of the urinary bladder (UBPGL) is a rare neuroendocrine tumor diagnosed in many patients only after surgery. We, therefore, assessed clinical clues relevant to presurgical diagnosis and clinical consequences in patients with a missed presurgical diagnosis of UBPGL. Materials and methods Case reports describing a UBPGL (published from 1–1–2001 and 31–12–2020) were identified in Pubmed. Two authors independently performed data extraction and assessed data quality according to the PRISMA guideline. Patients were divided into two groups: UBPGL diagnosis before and after surgery. Results We included 177 articles reporting 194 cases. In 90 (46.4%) patients, the UBPGL was diagnosed before and in 104 (53.6%) after surgery. In presurgically diagnosed UBPGL, hypertension and catecholamine-associated symptoms were 2- to 3-fold (p < 0.001) more frequent than in postsurgically diagnosed patients whereas hematuria was twofold (p = 0.003) more prevalent in those with postsurgical diagnosis. Hypertension was an independent factor for presurgical biochemical testing (OR 4.45, 95% CI 1.66–11.94) while hematuria (OR 0.23, 95% CI 0.09–0.60) was an independent factor for not performing presurgical biochemical testing. Most patients diagnosed after surgery were not pretreated with alpha-adrenoceptor blockade (95.2%), underwent more frequently transurethral resection instead of cystectomy (70.2% vs. 23.1%) and had more frequent peroperative complications and residual tumor mass. Conclusions In nearly half of all patients with a UBPGL, the diagnosis was not established before surgery. Hypertension and hematuria contributed independently to a presurgical diagnosis. Postsurgical diagnosis, which was associated with suboptimal presurgical and surgical management, resulted in more peroperative complications and incomplete tumor resections.

Case Report: Octreotide plus CVD chemotherapy for the treatment of multiple metastatic paragangliomas after double resection for functional bladder paraganglioma and urothelial papilloma

“…The results of CT and VMA in 24-h urine during octreotide plus six courses of CVD chemotherapy and the flowchart of the timeline for diagnosis and treatment process. feasibility and safety of TURBT in patients with bladder PGLs (21). In the present case report, radical cystectomy would have increased surgical trauma to increase the risk of lower quality of life.…”

Case report: A novel somatic SDHB variant in a patient with bladder paraganglioma

“…PCCs are rare neuroendocrine tumors deriving from chromaffin cells of the adrenal glands, and approximately 10% of these neoplasms are in the extra-adrenal sites and are referred to as PGLs. Among them, UBPGLs are one of the rarest forms with only a couple of hundred cases reported in the literature ( 3 , 5 ). They can manifest with a range of symptoms, although they may remain clinically silent.…”

Bladder Paraganglioma Associated With Succinate Dehydrogenase A Mutation Presenting as Pelvic Pain

“…Definitive diagnosis did not occur until after cystoscopy with biopsy. In patients without a presurgical diagnosis of paraganglioma, the majority undergo TUR [ 9 ]. In contrast, most patients with a presurgical diagnosis undergo cystectomy with only 5% of patients undergoing TUR.…”