Therapeutic options span from curative surgery to systemic therapies, including temozolomide, tyrosine kinase inhibitors, and nuclide therapy...Improve knowledge about the different Nuclear Medicine and functional imaging techniques in detecting lesions, depending on their location, secretory function and underlying genetic mutation. Discuss the multiple radiopharmaceuticals available for Scintigraphy and PET-CT, according to the paraganglioma site and mutational pattern.
Moreover, in response to these clinical difficulties, significant progress has been made in developing preclinical models that simulate the development and progression of PNETs. Therefore, this review systematically summarized the latest research advances in the diagnosis, treatment, and research models of PNETs.
In line with previous studies in non-MEN1 associated NET, in MEN1, a circulating NET gene expression assay identified patients without any manifestations from PanNETs. The mNET assay did not predict PanNET development, progression, or metastasis. The underlying genetic condition, epigenetic modifications, microadenomas and coexistence of multiple manifestations may impact circulating RNA profiles. Further research should explore alternative biomarkers or develop clinico-molecular algorithms for personalized management strategies in MEN1.
Surgical resection remains the cornerstone of treatment for localized disease, while systemic therapies such as everolimus, somatostatin analogs, platinum-based chemotherapy, and peptide receptor radionuclide therapy (PRRT) are options for advanced cases...Advances in molecular profiling, novel imaging techniques, and systemic therapies offer hope for improved outcomes. Given the disease rarity, continued collaboration through registries and multicenter studies is essential to refine evidence-based management strategies.
Interdisciplinary collaborations and global consortia are crucial for overcoming current limitations and translating research findings into clinical practice. These insights hold promise for improving prevention, early detection, and tailored treatments, ultimately enhancing patient outcomes.
According to current systematic review, the value of the NETest both for diagnosis and for prognosis of GEP-NET emerged as robust across different studies. Further prospective analysis on larger GEP-NET series is encouraged to validate this tool, improving patients' diagnosis, management, and follow-up.
The assay also demonstrated improved sensitivity for differentiating NETs from other gastrointestinal, pancreatic and lung malignancies. The validation of this tool in geographically diverse cohorts highlights their potential for widespread clinical use.
NETest 2.0 simplified the disease scoring system exhibiting improved diagnostic and prognostic capabilities over NETest 1.0. This optimized, validated blood-based molecular tool provides a powerful approach for diagnostic, prognostic and patient monitoring.
Advances in sequencing technologies may enhance the clinical utility of LB in NENs. Future research should focus on refining LB methods, standardising protocols and exploring applications in high-grade NENs.