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Disease monitoring of patients with pheochromocytoma or paraganglioma by biomarkers and imaging studies.
Best Pract Res Clin Endocrinol Metab. 2020 03; 34(2):101347.BP

Abstract

Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, a large proportion of which secrete catecholamines. PPGL are associated with a high cardiovascular morbidity and come with a risk of malignancy. The therapy of choice is surgical resection. Nevertheless, PPGL are associated with a lifelong risk of tumor persistence or recurrence. Currently, there are no clinical, biochemical, histopathological or imaging characteristics, which can predict or exclude malignant behavior or tumor recurrence. Therefore, long-term follow-up is recommended even after apparent complete surgical removal. Early detection of recurrence is essential to reduce cardiovascular morbidity and mortality due to catecholamine secretion, to prevent morbidity by mass effects of paraganglioma (PGL) or by metastatic spread of disease. Due to the rarity of these tumors, no prospective data on long-term surveillance exist. In fact, current recommendations are based on retrospective analyses, expert opinions and case studies. The aim of this review is to provide an overview on the current state of knowledge with regard to known factors that increase the risk of recurrence and might impact disease monitoring as well as the available possibilities for biochemical and imaging follow-up. Based on this overview, we aim to propose a practical approach for a patient-oriented follow-up after surgical removal of a PPGL.

Authors+Show Affiliations

Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zurich, Switzerland.Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zurich, Switzerland; Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany. Electronic address: felix.beuschlein@usz.ch.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

31662271

Citation

Schreiner, Florentine, and Felix Beuschlein. "Disease Monitoring of Patients With Pheochromocytoma or Paraganglioma By Biomarkers and Imaging Studies." Best Practice & Research. Clinical Endocrinology & Metabolism, vol. 34, no. 2, 2020, p. 101347.
Schreiner F, Beuschlein F. Disease monitoring of patients with pheochromocytoma or paraganglioma by biomarkers and imaging studies. Best Pract Res Clin Endocrinol Metab. 2020;34(2):101347.
Schreiner, F., & Beuschlein, F. (2020). Disease monitoring of patients with pheochromocytoma or paraganglioma by biomarkers and imaging studies. Best Practice & Research. Clinical Endocrinology & Metabolism, 34(2), 101347. https://doi.org/10.1016/j.beem.2019.101347
Schreiner F, Beuschlein F. Disease Monitoring of Patients With Pheochromocytoma or Paraganglioma By Biomarkers and Imaging Studies. Best Pract Res Clin Endocrinol Metab. 2020;34(2):101347. PubMed PMID: 31662271.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Disease monitoring of patients with pheochromocytoma or paraganglioma by biomarkers and imaging studies. AU - Schreiner,Florentine, AU - Beuschlein,Felix, Y1 - 2019/10/21/ PY - 2019/10/31/pubmed PY - 2020/11/6/medline PY - 2019/10/31/entrez KW - diagnostic tests KW - follow-up KW - genetic disease KW - paraganglioma KW - pheochromocytoma KW - recurrent or persistent disease SP - 101347 EP - 101347 JF - Best practice & research. Clinical endocrinology & metabolism JO - Best Pract Res Clin Endocrinol Metab VL - 34 IS - 2 N2 - Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, a large proportion of which secrete catecholamines. PPGL are associated with a high cardiovascular morbidity and come with a risk of malignancy. The therapy of choice is surgical resection. Nevertheless, PPGL are associated with a lifelong risk of tumor persistence or recurrence. Currently, there are no clinical, biochemical, histopathological or imaging characteristics, which can predict or exclude malignant behavior or tumor recurrence. Therefore, long-term follow-up is recommended even after apparent complete surgical removal. Early detection of recurrence is essential to reduce cardiovascular morbidity and mortality due to catecholamine secretion, to prevent morbidity by mass effects of paraganglioma (PGL) or by metastatic spread of disease. Due to the rarity of these tumors, no prospective data on long-term surveillance exist. In fact, current recommendations are based on retrospective analyses, expert opinions and case studies. The aim of this review is to provide an overview on the current state of knowledge with regard to known factors that increase the risk of recurrence and might impact disease monitoring as well as the available possibilities for biochemical and imaging follow-up. Based on this overview, we aim to propose a practical approach for a patient-oriented follow-up after surgical removal of a PPGL. SN - 1878-1594 UR - https://www.unboundmedicine.com/medline/citation/31662271/Disease_monitoring_of_patients_with_pheochromocytoma_or_paraganglioma_by_biomarkers_and_imaging_studies_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1521-690X(19)30098-3 DB - PRIME DP - Unbound Medicine ER -