Presentation, Management, and Outcomes of Urinary Bladder Paraganglioma: Results From a Multicenter Study

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DOI

  • Kai Yu, Sichuan University, Mayo Clinic Rochester, MN
  • ,
  • Andreas Ladefoged Ebbehøj
  • Hiba Obeid, University of Michigan, Ann Arbor
  • ,
  • Anand Vaidya, Harvard University
  • ,
  • Tobias Else, University of Michigan, Ann Arbor
  • ,
  • Heather Wachtel, Pennsylvania State University
  • ,
  • Ailsa Maria Main, University of Copenhagen
  • ,
  • Esben Søndergaard
  • Louise Lehmann Christensen, University of Southern Denmark
  • ,
  • Christofer Juhlin, Karolinska Institutet
  • ,
  • Jan Calissendorff, Karolinska Institutet
  • ,
  • Debbie L. Cohen, Pennsylvania State University
  • ,
  • Bonita Bennett, Pennsylvania State University
  • ,
  • Marianne Skovsager Andersen
  • Catharina Larsson, Karolinska Institutet
  • ,
  • Madson Q. Almeida, Universidade de Sao Paulo
  • ,
  • Lauren Fishbein, University of Colorado Denver
  • ,
  • Stephen A. Boorjian, Mayo Clinic Rochester, MN
  • ,
  • William F. Young, Mayo Clinic Rochester, MN
  • ,
  • Irina Bancos, Mayo Clinic Rochester, MN

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.

Original languageEnglish
JournalThe Journal of clinical endocrinology and metabolism
Volume107
Issue10
Pages (from-to)2811-2821
Number of pages11
ISSN0021-972X
DOIs
Publication statusPublished - 2022

Bibliographical note

Publisher Copyright:
© The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

    Research areas

  • catecholamine, diagnosis, micturition, prognosis

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