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Presentation, Management, and Outcomes of Urinary Bladder Paraganglioma: Results from a Multi-center Study

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机构: [1]Adrenal Center, Division of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu, Sichuan, China. [2]Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA. [3]Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark. [4]Department of Clinical Medicine, Aarhus University, Aarhus, Denmark. [5]Department of Internal Medicine, Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, MI, USA. [6]Center for Adrenal Disorders, Brigham and Women's Hospital, Harvard Medical School, MA, USA. [7]Department of Surgery, Division of Endocrine and Oncologic Surgery, University of Pennsylvania, PA, USA. [8]Department of Endocrinology and Metabolism, Copenhagen University Hospital, Copenhagen, Denmark. [9]Faculty of Health and Medical Sciences, Copenhagen University, Copenhagen, Denmark. [10]Department of Endocrinology, Odense University Hospital, Denmark. [11]Department of Oncology-Pathology, Karolinska Institute, Solna, Sweden. [12]Department of Pathology and Cancer Diagnostics, Karolinska University Hospital, Solna, Sweden. [13]Department of Molecular Medicine and Surgery, Karolinska Institute, Stockholm, Sweden. [14]Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden. [15]Department of Medicine, Renal Division, University of Pennsylvania, PA, 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, Brasil. [17]Servico de Endocrinologia, Instituto do Câncer do Estado de São Paulo (ICESP), Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brasil. [18]Division of Endocrinology, University of Colorado, Denver, CO, USA. [19]Department of Urology, Mayo Clinic, Rochester, MN, USA.
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Urinary bladder paraganglioma (UBPGL) is rare. We aimed to characterize the presentation and outcomes of patients diagnosed with UBPGL.We conducted a multi-center study of consecutive patients with pathologically-confirmed UBPGL evaluated between 1971 and 2021. Outcomes included repeat bladder surgery, metastases, and disease-specific mortality.110 patients (56, 51% women) were diagnosed with UBPGL at a median age of 50 years (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 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 of 1.69, 95%CI of 1.31-2.17), and a higher degree of catecholamine excess (HR of 5.48, 95%CI of 1.40-21.39). Disease-specific mortality was higher in patients with synchronous metastases (HR of 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.Only a minority of patients were diagnosed before biopsy/surgery, reflecting the need of better diagnostic strategies. All patients with UBPGL should be monitored life-long for development of recurrence and metastases.© 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.

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出版当年[2022]版:
大类 | 2 区 医学
小类 | 2 区 内分泌学与代谢
最新[2023]版:
大类 | 2 区 医学
小类 | 2 区 内分泌学与代谢
第一作者:
第一作者机构: [1]Adrenal Center, Division of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu, Sichuan, China. [2]Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.
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通讯机构: [2]Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA. [*1]Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN 55905, USA
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