Diagnosis and management of urinary bladder paragangliomas: A Sino‐American‐European retrospective observational study,Clinical Endocrinology

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Diagnosis and management of urinary bladder paragangliomas: A Sino‐American‐European retrospective observational study
Clinical Endocrinology ( IF 3.2 ) Pub Date : 2024-04-12 , DOI: 10.1111/cen.15058
Yingxian Pang _{1,

2} , Jing Zhang ₃ , Jingjing Jiang ₃ , Christina Pamporaki _{4,

5} , Minghao Li _{1,

5} , Nicole Bechmann _{4,

5} , Leah Meuter ₆ , Yongbao Wei ₇ , Haijian Huang ₇ , Shenghui Huang ₇ , Xunbin Yu ₇ , Mercedes Robledo ₈ , Miguel J. Soria ₉ , Dewen Zhong ₁₀ , Shangyuan Xu ₁₀ , Henri J. L. M. Timmers ₁₁ , Johan F. Langenhuijsen ₁₂ , Xiaofeng Chen ₁₃ , Wanglong Deng ₁₃ , Timo Deutschbein _{14,

15} , Hanna Remde ₁₄ , Long Wang ₁₆ , Hanyu Yao ₁₆ , Bin Yan ₁₇ , Annika M. A. Berends ₁₈ , Michiel N. Kerstens ₁₈ , Yazhuo Jiang ₁₉ , Joakim Crona ₂₀ , Ning Xu ₂₁ , Hai Cai ₂₁ , Yanlin Wen ₂₂ , Anguo Wang ₂₂ , Ji Wu ₂₂ , Zongpin Zhang ₂₂ , Jinzhuo Ning ₂₃ , Fan Cheng ₂₃ , Xiang Chen ₁ , Jing Wang ₂₄ , Bin Xie ₂₄ , Danlei Chen ₂₅ , Yujun Liu ₂₆ , Longfei Liu _{1,

2} , Karel Pacak ₆ , Graeme Eisenhofer _{4,

5} , Jacques W. M. Lenders _{5,

11}

Affiliation

Department of Urology, Xiangya Hospital Central South University Changsha China
National Clinical Research Center for Geriatric Disorders, Xiangya Hospital Central South University Changsha China
Department of Endocrinology and Metabolism Zhongshan Hospital Fudan University Shanghai China
Institute of Clinical Chemistry and Laboratory Medicine Technische Universität Dresden Dresden Germany
Department of Medicine III, University Hospital Carl Gustav Carus Technische Universität Dresden Germany
Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA
Shengli Clinical Medical College of Fujian Medical University and Department of Urology Fujian Provincial Hospital Fuzhou China
Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center and Centro de Investigación Biomédica en Red de Enfermedades Raras Madrid Spain
Department of Medical Oncology European University of Madrid, University Getafe Hospital Madrid Spain
Department of Urology Longyan First Affiliated Hospital of Fujian Medical University Longyan China
Department of Internal Medicine Radboud University Medical Center Nijmegen Netherlands
Department of Urology Radboud University Medical Center Nijmegen Netherlands
Department of Urology The First People's Hospital of Chenzhou Chenzhou China
Department of Internal Medicine I, Division of Endocrinology and Diabetes University Hospital, University of Würzburg Würzburg Germany
Medicover Oldenburg MVZ Oldenburg Germany
Department of Urology, the Third Xiangya Hospital Central South University Changsha China
Department of Urology, the Second Xiangya Hospital Central South University Changsha China
Department of Endocrinology University of Groningen, University Medical Center Groningen Groningen Netherlands
Department of Urology Shaanxi Provincial People's Hospital Xi'an China
Department of Medical Sciences Uppsala University Uppsala Sweden
Department of Urology The First Affiliated Hospital of Fujian Medical University Fuzhou China
Department of Urology, Nanchong Central Hospital The Second Clinical Hospital of North Sichuan Medical College Szechwan China
Department of Urology Renmin Hospital of Wuhan University Wuhan China
Department of Pathology, Xiangya Hospital Central South University Changsha China
Xiangya School of Medicine Central South University Changsha China
Department of Urology, Zhongshan Hospital Fudan University Shanghai China

ObjectiveParagangliomas of the urinary bladder (UBPGLs) are rare neuroendocrine tumours and pose a diagnostic and surgical challenge. It remains unclear what factors contribute to a timely presurgical diagnosis. The purpose of this study is to identify factors contributing to missing the diagnosis of UBPGLs before surgery.Design, Patients and MeasurementsA total of 73 patients from 11 centres in China, and 51 patients from 6 centres in Europe and 1 center in the United States were included. Clinical, surgical and genetic data were collected and compared in patients diagnosed before versus after surgery. Logistic regression analysis was used to identify clinical factors associated with initiation of presurgical biochemical testing.ResultsAmong all patients, only 47.6% were diagnosed before surgery. These patients were younger (34.0 vs. 54.0 years, p < .001), had larger tumours (2.9 vs. 1.8 cm, p < .001), and more had a SDHB pathogenic variant (54.7% vs. 11.9%, p < .001) than those diagnosed after surgery. Patients with presurgical diagnosis presented with more micturition spells (39.7% vs. 15.9%, p = .003), hypertension (50.0% vs. 31.7%, p = .041) and catecholamine‐related symptoms (37.9% vs. 17.5%, p = .012). Multivariable logistic analysis revealed that presence of younger age (<35 years, odds ratio [OR] = 6.47, p = .013), micturition spells (OR = 6.79, p = .007), hypertension (OR = 3.98, p = .011), and sweating (OR = 41.72, p = .013) increased the probability of initiating presurgical biochemical testing.ConclusionsMost patients with UBPGL are diagnosed after surgery. Young age, hypertension, micturition spells and sweating are clues in assisting to initiate early biochemical testing and thus may establish a timely presurgical diagnosis.

更新日期：2024-04-12

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