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Malignant prediction in paragangliomas: analysis for clinical risk factors

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Abstract

Introduction

Paragangliomas are infrequent neuroendocrine tumours whose only criterion for malignancy is presence of metastases; thus, all paragangliomas show malignant potential. Actually, different risk factors have been analyzed to predict metastases but they remain unclear.

Purpose

To analyze clinical, histological, and genetic factors to predict the occurrence of metastasis.

Patients and method

A multicentre retrospective observational analysis was performed between January 1990 and July 2019. Patients diagnosed with paraganglioma were selected. Clinical, histological, and genetic features were analyzed for the prediction of malignancy.

Results

A total of 83 patients diagnosed with paraganglioma were included, of which nine (10.8%) had malignant paraganglioma. Tumour size was greater in malignant tumours than in benign (6 cm vs. 4 cm, respectively; p = 0.027). The most frequent location of malignancy was the thorax-abdomen-pelvis area observed in six cases (p = 0.024). No differences were observed in histological differentiation, age, symptoms, and catecholaminergic production. The most frequent genetic mutation was SDHD followed by SDHB but no differences were observed between benign and malignant tumours. In the univariate analysis for predictive factors for malignancy, location, tumour size, and histological differentiation showed statistical significance (p = 0.025, p = 0.014, and p = 0.046, respectively); however, they were not confirmed as predictive factors for malignancy in the multivariate analysis.

Conclusion

In this study, no risk factors for malignancy have been established; therefore, we recommend follow-up of all patients diagnosed with paraganglioma.

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Correspondence to Miquel Torres-Costa.

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Ethics approval

This study was approved by the Ethics Committee of the study’s reference hospital, and the participation of other hospitals was approved by the appropriate competent entities.

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Informed consent was obtained from all individual participant included in the genetic analyses.

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The authors declare no competing interests.

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Torres-Costa, M., Flores, B., Torregrosa, N. et al. Malignant prediction in paragangliomas: analysis for clinical risk factors. Langenbecks Arch Surg 406, 2441–2448 (2021). https://doi.org/10.1007/s00423-021-02222-9

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