About this Research Topic
One of authentic features of neuroendocrine tumors (NETs) is a potential to synthesize and secrete biogenic peptides and hormones. Typically arising from tumors originating from the pancreas, lungs and intestine, these products can cause recognizable clinical hormone syndromes. Even rarer among the rare, functional NETs are in the minority compared to nonfunctional tumors (i.e. 10-40% in a group of pancreatic NETs), but they represent great diagnostic and therapeutic challenges. For any case of functional NET, hormonal hypersecretion syndrome causes excess morbidity, sometimes even more threatening than the cancer itself. This makes control of hormonal hypersecretion not only additional, but sometimes in fact a primary therapeutic goal. On the other hand, secretory product(s) can be useful as tumor markers and facilitate monitoring of treatment response. Unfortunately, many of these tumors become refractory to treatment over time, and new modalities are required.
Even with advances in diagnostics and therapy, we are dealing with many unanswered questions regarding functional NETs. Although increasingly recorded, functional NETs are still lacking systemic documentation, and relevant research is widely dispersed. Presentations are highly variable, since development of hormonal hypersecretion syndrome does not always correlate with tumor stage, grade, and patient outcomes. Occult tumors may present with florid hormonal syndromes causing significant morbidity, so new diagnostic techniques are required especially in the field of nuclear medicine. Patients with diffuse metastatic disease may be refractory to standard of care, so new therapeutic modalities need to be explored. A special interest goes to exploration of genetic alterations that drive malignant transformation, which could become potential therapeutic targets and/or help predict clinical outcomes.
Given the aforementioned gaps in research and understanding of functional NETs, we are aiming to gather as much knowledge, from as many angles as available. This includes original research from small and large patient series, as well as reviews. We hope to gather experience regarding:
• Difficult clinical presentations
• Diagnostic challenges
• Promising therapeutic strategies
• New antisecretory therapies
• Molecular targets
• Predictive factors of response and survival
Even with advances in diagnostics and therapy, we are dealing with many unanswered questions regarding functional NETs. Although increasingly recorded, functional NETs are still lacking systemic documentation, and relevant research is widely dispersed. Presentations are highly variable, since development of hormonal hypersecretion syndrome does not always correlate with tumor stage, grade, and patient outcomes. Occult tumors may present with florid hormonal syndromes causing significant morbidity, so new diagnostic techniques are required especially in the field of nuclear medicine. Patients with diffuse metastatic disease may be refractory to standard of care, so new therapeutic modalities need to be explored. A special interest goes to exploration of genetic alterations that drive malignant transformation, which could become potential therapeutic targets and/or help predict clinical outcomes.
Given the aforementioned gaps in research and understanding of functional NETs, we are aiming to gather as much knowledge, from as many angles as available. This includes original research from small and large patient series, as well as reviews. We hope to gather experience regarding:
• Difficult clinical presentations
• Diagnostic challenges
• Promising therapeutic strategies
• New antisecretory therapies
• Molecular targets
• Predictive factors of response and survival
Keywords: Neuroendocrine tumors, functional tumors, hypersecretoy syndromes, paraneoplastic syndromes
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