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Endocrine Abstracts (2022) 81 OC6.1 | DOI: 10.1530/endoabs.81.OC6.1

1University Hospital Carl Gustav Carus at the TU Dresden, Department of Medicine III, Dresden, Germany; 2National Institutes of Health, Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, United States; 3University of Groningen, University Medical Center Groningen, Department of Endocrinology, Groningen, Netherlands; 4University Hospital Carl Gustav Carus at the TU Dresden, Institute of Clinical Chemistry and Laboratory Medicine, Dresden, Germany; 5Institute of Cardiology, Warsaw, Department of Hypertension, Warsaw, Poland; 6University Hospital of Würzburg, 6Department of Internal Medicine, Würzburg, Germany; 7Radboud University Hospital, Nijmegen, Department of Internal Medicine, Nijmegen, Netherlands; 8University Hospital of Munich, Department of Internal Medicine, Munich, Germany; 9University Hospital, Zurich, Department of Endocrinology, Diabetology, and Clinical Nutrition, Switzerland; 10University Medical Center Schleswig-Holstein, Luebeck, Department of Medicine, Luebeck, Germany; 11Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Hereditary Endocrine Cancer Group, Spain

Background: It is well established that life-long follow-up is required for patients with hereditary pheochromocytomas and paragangliomas (PPGLs), due to the potential of developing recurrent disease. However, whether follow-up of patients with sporadic PPGLs is necessary, remains unclear.

Aims: To examine the prevalence and predictors of recurrent disease in patients with sporadic PPGLs.Materials and method: This multicenter study included retrospective clinical data of 528 patients with PPGLs. Recurrent disease was defined as presence of new tumor and/or locoregional recurrence and/or metastases one year after initial tumor diagnosis. Patients with sporadic PPGLs were defined as those without germline mutations in known genes associated with hypoxia (cluster 1) or kinase (cluster 2) signaling pathways.

Results: Fifty-three percent of the patients had sporadic PPGLs and presented with a recurrence rate of 17.3%, which mainly reflected metastatic disease (10%). This was significantly lower than those with cluster 1 (54.3%), but similar to those with cluster 2 mutations (14.1%). Among patients with sporadic PPGLs and recurrent disease, 70.7% developed recurrence within 10 years from initial tumor diagnosis. Multivariable Cox regression analysis showed that larger (>4.5 cm) size (HR 1.8, 95% CI 1.13-3.0, P=0.015) and extra-adrenal location (HR 2.4, 95% CI 1.4-4.11, P=0.001) of the primary tumor, were independent predictors of recurrence in patients with sporadic PPGLs. Indeed, patients with small (≤4.5 cm) sporadic pheochromocytomas presented with the lowest (7.8%) rate of recurrent disease (P<0.001).

Conclusion: Among patients with sporadic PPGLs, prevalence of recurrence was mainly due to metastases and high enough to mandate long-term follow-up. Importantly, our findings indicate that size and tumor location are important to consider for further stratification and management of patients with sporadic PPGLs.

Volume 81

European Congress of Endocrinology 2022

Milan, Italy
21 May 2022 - 24 May 2022

European Society of Endocrinology 

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