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Assessing the response to systemic therapy in neuroendocrine tumors (NET) is challenging since morphological imaging response is often delayed and not necessarily reflective of clinical benefit. Peptide receptor radionuclide therapy (PRRT) has a complex mechanism of action, further complicating response assessment. In response to these challenges, the European Neuroendocrine Tumor Society (ENETS) Theranostics Task Force conducted a statement-based survey among experts to identify the current landscape and unmet needs in PRRT response assessment.

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Article Synopsis
  • This paper presents guidelines from major Italian medical associations on the use of radioligand therapy (RLT) for treating neuroendocrine neoplasms (NENs).
  • It addresses 10 key questions regarding RLT’s effectiveness in treating gastroenteropancreatic neuroendocrine tumors (GEP-NETs) based on literature review and expert opinions.
  • The focus is on well-differentiated GEP-NETs that express somatostatin receptors, identifying which patients are appropriate candidates for RLT according to established international protocols.
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Article Synopsis
  • Evidence-based treatment for advanced gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) requires personalized decisions that take into account individual patient and cancer characteristics. !* -
  • A multidisciplinary panel reviewed 131 publications to develop a guidance document with 24 recommendations and 5 good clinical practice statements focusing on diagnostic features and biomarkers for managing unresectable or metastatic GEP-NENs. !* -
  • The document emphasizes treatment strategies based on tumor characteristics and specific biomarkers, and it received endorsements from major neuroendocrine tumor organizations. !*
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International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.

Nat Rev Endocrinol

December 2024

Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70-80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. Key differences in the tumour biology and management, together with rare disease incidence and therapeutic challenges in paediatric compared with adult patients, mandate close expert cross-disciplinary teamwork.

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