Kai Yu1,2, Andreas Ladefoged Ebbehøj3,4, Hiba Obeid5, Anand Vaidya6, Tobias Else5, Heather Wachtel7, Ailsa Maria Main8,9, Esben Søndergaard3,4, Louise Lehmann Christensen10, Christofer Juhlin11,12, Jan Calissendorff13,14, Debbie L Cohen15, Bonita Bennett15, Marianne Skovsager Andersen10, Catharina Larsson11, Madson Q Almeida16,17, Lauren Fishbein18, Stephen A Boorjian19, William F Young2, Irina Bancos2. 1. Adrenal Center, Division of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu, Sichuan, 610041, China. 2. Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, 55905, USA. 3. Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, 8200, Denmark. 4. Department of Clinical Medicine, Aarhus University, Aarhus, 8200, Denmark. 5. Department of Internal Medicine, Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, MI, 48105, USA. 6. Center for Adrenal Disorders, Brigham and Women's Hospital, Harvard Medical School, MA, 02115, USA. 7. Department of Surgery, Division of Endocrine and Oncologic Surgery, University of Pennsylvania, PA, 19104, USA. 8. Department of Endocrinology and Metabolism, Copenhagen University Hospital, Copenhagen, 2100, Denmark. 9. Faculty of Health and Medical Sciences, Copenhagen University, Copenhagen, 2100, Denmark. 10. Department of Endocrinology, Odense University Hospital, Odense, 5000, Denmark. 11. Department of Oncology-Pathology, Karolinska Institute, Solna, 17164, Sweden. 12. Department of Pathology and Cancer Diagnostics, Karolinska University Hospital, Solna, 17176, Sweden. 13. Department of Molecular Medicine and Surgery, Karolinska Institute, Stockholm, 17176, Sweden. 14. Department of Endocrinology, Karolinska University Hospital, Stockholm, 17176, Sweden. 15. Department of Medicine, Renal Division, University of Pennsylvania, PA, 19104, USA. 16. Unidade de Adrenal, Laboratório de Hormônios e Genética Molecular LIM/42, Serviço de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, 05403-900, Brasil. 17. Servico de Endocrinologia, Instituto do Câncer do Estado de São Paulo (ICESP), Faculdade de Medicina da Universidade de São Paulo, São Paulo, 05403-900, Brasil. 18. Division of Endocrinology, University of Colorado, Denver, CO, 80045, USA. 19. Department of Urology, Mayo Clinic, Rochester, MN 55905, USA.
Abstract
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.
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.
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