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ORIGINAL RESEARCH article

Front. Endocrinol.
Sec. Adrenal Endocrinology
Volume 15 - 2024 | doi: 10.3389/fendo.2024.1419028
This article is part of the Research Topic Adrenal Related Hypertension: From Bench to Bedside, volume II View all 7 articles

Metastatic disease and major adverse cardiovascular events (MACE) preceding diagnosis are the main determinants of disease-specific survival of pheochromocytoma/paraganglioma: long-term follow-up of 303 patients

Provisionally accepted
Wolfgang Raber Wolfgang Raber 1*Raphael Schendl Raphael Schendl 1Melisa Arikan Melisa Arikan 2Andreas Scheuba Andreas Scheuba 2Peter Mazal Peter Mazal 3Valerie Stadlmann Valerie Stadlmann 4Reinhard Lehner Reinhard Lehner 4Petra Zeitlhofer Petra Zeitlhofer 5Sabina Baumgartner-Parzer Sabina Baumgartner-Parzer 1Cornelia Gabler Cornelia Gabler 6Harald Esterbauer Harald Esterbauer 7
  • 1 Clinical Department of Endocrinology and Metabolism, University Clinic for Internal Medicine III, Medical University of Vienna, Vienna, Vienna, Austria
  • 2 University Clinic for General Surgery, Medical University of Vienna, Vienna, Vienna, Austria
  • 3 Department of Pathology, Medical University of Vienna, Vienna, Austria
  • 4 Center for Pathobiochemistry and Genetics, Medical University of Vienna, Vienna, Vienna, Austria
  • 5 Labdia Labordiagnostik GmbH, Vienna, Austria
  • 6 Department of IT Systems and Communications, Medical University of Vienna, Vienna, Vienna, Austria
  • 7 Clinical Institute for Laboratory Medicine, Medical University of Vienna, Vienna, Vienna, Austria

The final, formatted version of the article will be published soon.

    Purpose: The natural history in unselected cohorts of patients with pheochromocytoma/ paraganglioma (PPGL) followed for a period >10 years remains limited. We aimed to describe baseline characteristics of a large cohort and to identify predictors of shorter survival.Methods: This retrospective single-center study included 303 patients with newly diagnosed PPGL from 1968 to December 31, 2023, in 199 prospectively supplemented since July 2020. Meandian follow-up was 11.4 (range 0.3-50) years, germline genetic analyses were available in 92.1%. The main outcome measures were overall (OAS), disease-specific (DSS), recurrence-free (RFS) survival and predictors of shorter survival evaluated in patients with metastases at first diagnosis (n=12), metastatic (n=24) and nonmetastatic (n=33) recurrences and without evidence of PPGL (n=234).Results: Age at study begin was 49.4±16.3 years. There were 72 (23.8%) deaths, 15 (5.0%), 29 (9.6%) and 28 (9.2%) due to PPGL, cardiovascular disease (CVD) and malignant or other diseases, respectively. Median OAS, DSS1 (tumor-related) and DSS2 (DSS1 and death caused by CVD) were 4.8, 5.9 and 5.2 years (patients with metastases at first diagnosis), 21.2, 21.2 and 19.9 years, and 38.0, undefined and 38.0 years (patients with metastatic and with nonmetastatic recurrences, respectively). Major adverse cardiovascular events (MACE) preceded the first diagnosis in 15% (n=44). Shorter DSS2 correlated with older age (P≤0.001), male sex (P≤0.02), MACE (P≤0.01) and primary metastases (P<0.0001, also for DSS1).Conclusion. The clinical course of unselected patients with PPGL is rather benign. Survival rates remain high for decades, unless there are MACE before diagnosis or metastatic disease.

    Keywords: Pheochromocytoma, Paraganglioma, Recurrence, Survival, Genetics, Natural History, long-term follow-up Abstand Vor: 0 Pt., Nach: 10 Pt.

    Received: 17 Apr 2024; Accepted: 29 Jul 2024.

    Copyright: © 2024 Raber, Schendl, Arikan, Scheuba, Mazal, Stadlmann, Lehner, Zeitlhofer, Baumgartner-Parzer, Gabler and Esterbauer. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Wolfgang Raber, Clinical Department of Endocrinology and Metabolism, University Clinic for Internal Medicine III, Medical University of Vienna, Vienna, 1090, Vienna, Austria

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