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<p>Paragangliomas (PPGLs) are neuroendocrine tumours with inherent malignant potential and the ability to metastasize to distant sites. The likelihood of metastatic disease increases in patients with extra-adrenal lesions, tumours larger than 5&#xa0;cm, Ki-67 ≥3%, younger age at diagnosis, positive germline testing (particularly involving <i>SDHB</i>), a less differentiated biochemical phenotype, and more advanced TNM stages. Since 2002, multiple scoring systems have been proposed, initially relying predominantly on histopathological data to improve metastatic risk stratification. More recently, additional scores have considered further tumour- and patient-related characteristics beyond histopathology, some of which can be applied in the preoperative setting. To date, however, no single scoring system has demonstrated sufficient reliability to identify patients at highest risk of metastasis, and the World Health Organization (WHO) does not endorse any particular score. In clinical practice, therefore, the concurrent use of all available scoring tools may facilitate a more individualised approach to patient surveillance and follow-up.</p>

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Paragangliomi: pro e contro degli score di rischio

  • 期刊论文

  • 发表日期：

    2026年1月19日

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  • Letizia Canu,
  • Lorenzo Zanatta

摘要

Paragangliomas (PPGLs) are neuroendocrine tumours with inherent malignant potential and the ability to metastasize to distant sites. The likelihood of metastatic disease increases in patients with extra-adrenal lesions, tumours larger than 5 cm, Ki-67 ≥3%, younger age at diagnosis, positive germline testing (particularly involving SDHB), a less differentiated biochemical phenotype, and more advanced TNM stages. Since 2002, multiple scoring systems have been proposed, initially relying predominantly on histopathological data to improve metastatic risk stratification. More recently, additional scores have considered further tumour- and patient-related characteristics beyond histopathology, some of which can be applied in the preoperative setting. To date, however, no single scoring system has demonstrated sufficient reliability to identify patients at highest risk of metastasis, and the World Health Organization (WHO) does not endorse any particular score. In clinical practice, therefore, the concurrent use of all available scoring tools may facilitate a more individualised approach to patient surveillance and follow-up.