Table 4.
Characteristics and outcomes of patients with benign and malignant urinary bladder paraganglioma
| Variable | Nonmetastatic n = 73 | Metastatica n = 33 |
P value |
|---|---|---|---|
| Baseline data | |||
| Women, n (%) | 40 (55%) | 16 (48%) | 0.695 |
| Age at diagnosis with UBPGL, years, median (IQR) | 57 (42-64) | 38 (21-47) | <0.001 |
| Mode of discovery, n (%), available for n = 104 | 0.001 | ||
| Symptoms of catecholamine excess | 15 (21%) | 18 (56%) | |
| Other | 57 (79%) | 14 (44%) | |
| SDHB genetic association, n (%) | 15 (35%) | 12 (52%) | 0.302 |
| UBPGL characteristics | |||
| Multinodular, n (%) | 10 (14%) | 5 (15%) | 1.000 |
| Muscular invasion, n (%), available for n = 69 | 31 (56%) | 7 (50%) | 0.899 |
| UBPGL tumor size a , cm, median (IQR) | 2 (1-3) | 4 (3-6) | <0.001 |
| Catecholamine excess, available for n = 71 | 0.009 | ||
| Present, n (%) | 23 (56%) | 24 (89%) | |
| Absent, n (%) | 18 (44%) | 3 (11%) | |
| Degree of catecholamine excess, n (%), available or n = 44 | < 0.001 | ||
| ≥10 times above the ULN | 1 (5%) | 12 (55%) | |
| < 10 times the ULN | 20 (95%) | 10 (45%) | |
| History or active concomitant PPGL | |||
| PPGL past history, n (%) | 5 (7%) | 4 (12%) | 0.455 |
| Concurrent PPGL, n (%) | 6 (8%) | 14 (42%) | <0.001 |
| Therapeutic data | |||
| Initial therapy for UBPGL | 0.005 | ||
| Surgery, n (%) | 71 (97%) | 30 (91%) | |
| Partial cystectomy | 41 (58%) | 19 (63%) | |
| TURBT | 25 (35%) | 3 (10%) | |
| Radical cystectomy | 2 (3%) | 3 (10%) | |
| Other or unspecified surgery | 3 (4%) | 5 (17%) | |
| Nonsurgical management, n (%) | 2 (3%) | 3 (9%) | |
| Patients treated with more than 1 therapy a , n (%) | 4 (6%) | 21 (66%) | <0.001 |
| Outcome data of patients initially managed with TURBT or partial cystectomy, n = 88 | |||
| Repeat bladder surgery, available for n = 86 | 1 (2%) | 5 (24%) | 0.003 |
| Recurrent UBPGL, n (%), available for n = 86 | 3 (5%) | 5 (24%) | 0.020 |
| Interval to recurrence, years, median (IQR) | 4 (4-4) | 3 (2-5) | 0.693 |
| Persistent UBPGL, n (%), available for n = 86 | 0 (0%) | 3 (14%) | 0.013 |
| Follow-up data | |||
| Status at the end of follow-up | 0.005b | ||
| Alive, n (%) | 60 (91%) | 18 (82%) | |
| Deceased of PPGL, n (%) | 0 (0%) | 4 (18%) | |
| Deceased of other reasons, n (%) | 6 (9%) | 0 (0%) | |
| Duration of follow-up, years, median (IQR) | 4 (1-7) | 8 (4-15) | 0.012 |
P values were estimated by Mann-Whitney U test for continuous variables and Chi-square test for categorical variables.
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.
aAdditional therapy included repeat surgery, chemotherapy, nuclear medicine therapy, targeted therapy, and local therapy with radiation or ablation. Indolent disease was defined as metastatic disease that had stable tumor size and numbers of metastatic spread based on imaging study at last follow-up. Metastatic urinary bladder paraganglioma included all the synchronously and metachronously metastatic urinary bladder paraganglioma. 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.
b P value was according to patients who died of PPGL vs patients who were alive or deceased for other reasons.