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

Kai Yu, Andreas Ladefoged Ebbehøj, Hiba Obeid, Anand Vaidya, Tobias Else, Heather Wachtel, Ailsa Maria Main, Esben Søndergaard, Louise Lehmann Christensen, Christofer Juhlin, Jan Calissendorff, Debbie L. Cohen, Bonita Bennett, Marianne Skovsager Andersen, Catharina Larsson, Madson Q. Almeida, Lauren Fishbein, Stephen A Boorjian, William F. Young, Irina Bancos

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)2811-2821
Number of pages11
JournalThe Journal of clinical endocrinology and metabolism
Volume107
Issue number10
DOIs
StatePublished - Sep 28 2022

Keywords

  • catecholamine
  • diagnosis
  • micturition
  • prognosis

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Endocrinology
  • Clinical Biochemistry
  • Biochemistry, medical

Fingerprint

Dive into the research topics of 'Presentation, Management, and Outcomes of Urinary Bladder Paraganglioma: Results From a Multicenter Study'. Together they form a unique fingerprint.

Cite this