TY - JOUR
T1 - Presentation, management, and outcomes of urinary bladder paraganglioma
T2 - Results from a multicenter study
AU - Yu, Kai
AU - Ebbehøj, Andreas Ladefoged
AU - Obeid, Hiba
AU - Vaidya, Anand
AU - Else, Tobias
AU - Wachtel, Heather
AU - Main, Ailsa Maria
AU - Søndergaard, Esben
AU - Lehmann Christensen, Louise
AU - Juhlin, Christofer
AU - Calissendorff, Jan
AU - Cohen, Debbie L
AU - Bennett, Bonita
AU - Andersen, Marianne Skovsager
AU - Larsson, Catharina
AU - Almeida, Madson Q
AU - Fishbein, Lauren
AU - Boorjian, Stephen A
AU - Young, William F
AU - Bancos, Irina
N1 - © The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: [email protected].
PY - 2022/10
Y1 - 2022/10
N2 - CONTEXT: Urinary bladder paraganglioma (UBPGL) is rare.OBJECTIVE: We aimed to characterize the presentation and outcomes of patients diagnosed with UBPGL.METHODS: We conducted a multicenter study of consecutive patients with pathologically confirmed UBPGL evaluated between 1971 and 2021. Outcomes included repeat bladder surgery, metastases, and disease-specific mortality.RESULTS: Patients (n=110 total; n=56 [51%] women) were diagnosed with UBPGL at a median age of 50 years (interquartile range [IQR], 36-61 years). Median tumor size was 2 cm (IQR, 1-4 cm). UBPGL was diagnosed prior to biopsy in only 37 (34%), and only 69 (63%) patients had evaluation for catecholamine excess. In addition to the initial bladder surgery, 26 (25%) required multiple therapies, including repeat surgery in 10 (9%). Synchronous metastases were present in 9 (8%) patients, and 24 (22%) other patients with UBPGL developed metachronous metastases at a median of 4 years (IQR, 2-10 years) after the initial diagnosis. Development of metachronous metastases was associated with younger age (hazard ratio [HR] 0.97; 95% CI, 0.94-0.99), UBPGL size (HR 1.69; 95% CI, 1.31-2.17), and a higher degree of catecholamine excess (HR 5.48; 95% CI, 1.40-21.39). Disease-specific mortality was higher in patients with synchronous metastases (HR 20.80; 95% CI, 1.30-332.91). Choice of initial surgery, genetic association, sex, or presence of muscular involvement on pathology were not associated with development of metastases or mortality.CONCLUSIONS: Only a minority of patients were diagnosed before biopsy/surgery, reflecting need for better diagnostic strategies. All patients with UBPGL should have lifelong monitoring for development of recurrence and metastases.
AB - CONTEXT: Urinary bladder paraganglioma (UBPGL) is rare.OBJECTIVE: We aimed to characterize the presentation and outcomes of patients diagnosed with UBPGL.METHODS: We conducted a multicenter study of consecutive patients with pathologically confirmed UBPGL evaluated between 1971 and 2021. Outcomes included repeat bladder surgery, metastases, and disease-specific mortality.RESULTS: Patients (n=110 total; n=56 [51%] women) were diagnosed with UBPGL at a median age of 50 years (interquartile range [IQR], 36-61 years). Median tumor size was 2 cm (IQR, 1-4 cm). UBPGL was diagnosed prior to biopsy in only 37 (34%), and only 69 (63%) patients had evaluation for catecholamine excess. In addition to the initial bladder surgery, 26 (25%) required multiple therapies, including repeat surgery in 10 (9%). Synchronous metastases were present in 9 (8%) patients, and 24 (22%) other patients with UBPGL developed metachronous metastases at a median of 4 years (IQR, 2-10 years) after the initial diagnosis. Development of metachronous metastases was associated with younger age (hazard ratio [HR] 0.97; 95% CI, 0.94-0.99), UBPGL size (HR 1.69; 95% CI, 1.31-2.17), and a higher degree of catecholamine excess (HR 5.48; 95% CI, 1.40-21.39). Disease-specific mortality was higher in patients with synchronous metastases (HR 20.80; 95% CI, 1.30-332.91). Choice of initial surgery, genetic association, sex, or presence of muscular involvement on pathology were not associated with development of metastases or mortality.CONCLUSIONS: Only a minority of patients were diagnosed before biopsy/surgery, reflecting need for better diagnostic strategies. All patients with UBPGL should have lifelong monitoring for development of recurrence and metastases.
U2 - 10.1210/clinem/dgac427
DO - 10.1210/clinem/dgac427
M3 - Journal article
C2 - 35882219
SN - 0021-972X
VL - 107
SP - 2811
EP - 2821
JO - The Journal of Clinical Endocrinology & Metabolism
JF - The Journal of Clinical Endocrinology & Metabolism
IS - 10
ER -