Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study

Yingxian Pang^{1

2}, Jing Zhang³, Jingjing Jiang³, Christina Pamporaki^{4

5}, Minghao Li^{1

5}, Nicole Bechmann^{4

5}, Leah Meuter⁶, Yongbao Wei⁷, Haijian Huang⁷, Shenghui Huang⁷, Xunbin Yu⁷, Mercedes Robledo⁸, Miguel J Soria⁹, Dewen Zhong¹⁰, Shangyuan Xu¹⁰, Henri J L M Timmers¹¹, Johan F Langenhuijsen¹², Xiaofeng Chen¹³, Wanglong Deng¹³, Timo Deutschbein^{14

15}, Hanna Remde¹⁴, Long Wang¹⁶, Hanyu Yao¹⁶, Bin Yan¹⁷, Annika M A Berends¹⁸, Michiel N Kerstens¹⁸, Yazhuo Jiang¹⁹, Joakim Crona²⁰, Ning Xu²¹, Hai Cai²¹, Yanlin Wen²², Anguo Wang²², Ji Wu²², Zongpin Zhang²², Jinzhuo Ning²³, Fan Cheng²³, Xiang Chen¹, Jing Wang²⁴, Bin Xie²⁴, Danlei Chen²⁵, Yujun Liu²⁶, Longfei Liu^{1

2}, Karel Pacak⁶, Graeme Eisenhofer^{4

5}, Jacques W M Lenders^{5

11}

Affiliations

¹ Department of Urology, Xiangya Hospital, Central South University, Changsha, China.
² National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, China.
³ Department of Endocrinology and Metabolism, Zhongshan Hospital Fudan University, Shanghai, China.
⁴ Institute of Clinical Chemistry and Laboratory Medicine, Technische Universität Dresden, Dresden, Germany.
⁵ Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität, Dresden, Germany.
⁶ Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.
⁷ Shengli Clinical Medical College of Fujian Medical University and Department of Urology, Fujian Provincial Hospital, Fuzhou, China.
⁸ Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center and Centro de Investigación Biomédica en Red de Enfermedades Raras, Madrid, Spain.
⁹ Department of Medical Oncology, European University of Madrid, University Getafe Hospital, Madrid, Spain.
¹⁰ Department of Urology, Longyan First Affiliated Hospital of Fujian Medical University, Longyan, China.
¹¹ Department of Internal Medicine, Radboud University Medical Center, Nijmegen, Netherlands.
¹² Department of Urology, Radboud University Medical Center, Nijmegen, Netherlands.
¹³ Department of Urology, The First People's Hospital of Chenzhou, Chenzhou, China.
¹⁴ Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, Würzburg, Germany.
¹⁵ Medicover Oldenburg MVZ, Oldenburg, Germany.
¹⁶ Department of Urology, the Third Xiangya Hospital, Central South University, Changsha, China.
¹⁷ Department of Urology, the Second Xiangya Hospital, Central South University, Changsha, China.
¹⁸ Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.
¹⁹ Department of Urology, Shaanxi Provincial People's Hospital, Xi'an, China.
²⁰ Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
²¹ Department of Urology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.
²² Department of Urology, Nanchong Central Hospital, The Second Clinical Hospital of North Sichuan Medical College, Szechwan, China.
²³ Department of Urology, Renmin Hospital of Wuhan University, Wuhan, China.
²⁴ Department of Pathology, Xiangya Hospital, Central South University, Changsha, China.
²⁵ Xiangya School of Medicine, Central South University, Changsha, China.
²⁶ Department of Urology, Zhongshan Hospital, Fudan University, Shanghai, China.

PMID: 38606576
DOI: 10.1111/cen.15058

Observational Study

Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study

Yingxian Pang et al. Clin Endocrinol (Oxf). 2024 Sep.

. 2024 Sep;101(3):234-242.

doi: 10.1111/cen.15058. Epub 2024 Apr 12.

Authors

Affiliations

¹ Department of Urology, Xiangya Hospital, Central South University, Changsha, China.
² National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, China.
³ Department of Endocrinology and Metabolism, Zhongshan Hospital Fudan University, Shanghai, China.
⁴ Institute of Clinical Chemistry and Laboratory Medicine, Technische Universität Dresden, Dresden, Germany.
⁵ Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität, Dresden, Germany.
⁶ Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.
⁷ Shengli Clinical Medical College of Fujian Medical University and Department of Urology, Fujian Provincial Hospital, Fuzhou, China.
⁸ Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center and Centro de Investigación Biomédica en Red de Enfermedades Raras, Madrid, Spain.
⁹ Department of Medical Oncology, European University of Madrid, University Getafe Hospital, Madrid, Spain.
¹⁰ Department of Urology, Longyan First Affiliated Hospital of Fujian Medical University, Longyan, China.
¹¹ Department of Internal Medicine, Radboud University Medical Center, Nijmegen, Netherlands.
¹² Department of Urology, Radboud University Medical Center, Nijmegen, Netherlands.
¹³ Department of Urology, The First People's Hospital of Chenzhou, Chenzhou, China.
¹⁴ Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, Würzburg, Germany.
¹⁵ Medicover Oldenburg MVZ, Oldenburg, Germany.
¹⁶ Department of Urology, the Third Xiangya Hospital, Central South University, Changsha, China.
¹⁷ Department of Urology, the Second Xiangya Hospital, Central South University, Changsha, China.
¹⁸ Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.
¹⁹ Department of Urology, Shaanxi Provincial People's Hospital, Xi'an, China.
²⁰ Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
²¹ Department of Urology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.
²² Department of Urology, Nanchong Central Hospital, The Second Clinical Hospital of North Sichuan Medical College, Szechwan, China.
²³ Department of Urology, Renmin Hospital of Wuhan University, Wuhan, China.
²⁴ Department of Pathology, Xiangya Hospital, Central South University, Changsha, China.
²⁵ Xiangya School of Medicine, Central South University, Changsha, China.
²⁶ Department of Urology, Zhongshan Hospital, Fudan University, Shanghai, China.

PMID: 38606576
DOI: 10.1111/cen.15058

Abstract

Objective: Paragangliomas 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 measurements: A 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.

Results: Among 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.

Conclusions: Most 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.

Keywords: diagnosis; paraganglioma; postsurgical; presurgical; urinary bladder.

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References

REFERENCES

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Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study

Affiliations

Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study

Authors

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Medical