Table 1.
Characteristics of patients with urinary bladder paraganglioma
| Variable | Patients with UBPGLn = 110 |
|---|---|
| Women, n (%) | 56 (51%) |
| Age at diagnosis with UBPGL, years, median (IQR) | 50 (36-61) |
| Mode of discovery, n (%) | |
| Symptoms of catecholamine excess | 33 (30%) |
| Urinary symptoms | 32 (29%) |
| Incidental on imaging | 31 (28%) |
| Genetic case detection | 3 (3%) |
| Incidental on pathology | 3 (3%) |
| Mass effect symptoms | 1 (1%) |
| Other abnormalities | 3 (3%) |
| Unknown | 4 (4%) |
| Family history of PPGL, n (%) | |
| Yes | 20 (18%) |
| No | 79 (72%) |
| Unknown | 11 (10%) |
| Genetic association, n (%) | |
| SDHB | 26 (24%) |
| SDHA | 2 (2%) |
| SDHAF2 | 1 (1%) |
| SDHC | 2 (2%) |
| SDHD | 1 (1%) |
| VHL | 2 (2%) |
| Tested, negative | 27 (25%) |
| Not tested or unknown | 49 (45%) |
| UBPGL characteristics | |
| Multinodular, n (%) | 16 (15%) |
| Muscular invasion, n (%), available for n = 71 | 40 (56%) |
| UBPGL tumor size a , cm, median (IQR) | 2 (1-4) |
| Catecholamine excess, n (%) | |
| Present | 48 (44%) |
| Not present | 21 (19%) |
| Unknown | 41 (37%) |
| Degree of catecholamine excess, n (%) | |
| ≥10 times above the ULN | 14 (29%) |
| 4-10 times above the ULN | 13 (27%) |
| 2-4 times above the ULN | 8 (17%) |
| 1-2 times above the ULN | 10 (21%) |
| Unknown | 3 (6%) |
| Concomitant PPGL, n (%) | |
| Concurrent PPGL | 21 (19%) |
| New PPGL | 15 (14%) |
| Initial therapy for UBPGL, available for n = 108 | |
| Surgery, n (%) | 102 (94%) |
| Partial cystectomy | 61 (60%) |
| TURBT | 28 (27%) |
| Radical cystectomy | 5 (5%) |
| Other or unspecified surgery | 8 (8%) |
| Nonsurgical management, n (%) | 6 (6%) |
| Additional therapy for UBPGL, available for n = 106 | |
| Repeat bladder surgery, n (%) | 10 (9%) |
| Patients treated with more than 1 therapy, n (%) | 26 (25%) |
| Recurrent UBPGL, n (%), available for n = 104 | 13 (13%) |
| Interval to recurrence, years, median (IQR) | 3 (2-5) |
| Persistent UBPGL, n (%), available for n = 106 | 10 (9%) |
|
Metastatic PPGL, n (%), available for n = 108 Synchronous |
33 (30%) 9 (8%) |
| Metachronous | 24 (22%) |
| Time to metachronous metastasis, years, median (IQR) | 4 (2-10) |
| Progression of metastatic disease, n (%), available for n = 32 | |
| Indolenta | 13 (41%) |
| Progressive | 19 (59%) |
| Duration of follow-up after metastasis development, years, median (IQR) | 4 (1-7) |
| Follow-up data | |
| Duration of follow-up, years, median (IQR) | 4 (1-11) |
| Status at the end of follow-up, n (%) | |
| Alive | 89 (81%) |
| Deceased of PPGL | 11 (10%) |
| Deceased of other reasons | 10 (9%) |
Abbreviations: IQR, interquartile range; PPGL, pheochromocytoma/paraganglioma; SDH, Succinate dehydrogenase complex; TURBT, transurethral resection of bladder tumor; UBPGL, urinary bladder paraganglioma; ULN, upper limit of normal; VHL, Von Hippel-Lindau.
aIndolent disease was defined as metastatic disease that had stable tumor size and numbers of metastatic spread based on imaging study at last follow-up. Incidental on pathology referred to UBPGLs that were incidentally found on the pathology post resection of other pelvic tumors. Tumor size was determined based on the largest diameter of the UBPGL on histopathology or imaging (in patients not treated by surgery); in patients with multinodular UBPGL, tumor size was defined as the largest diameter among all the nodules.