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Updated: Jan 10, 2026
Human Neuroendocrine Tumor Cell Lines as a Three-Dimensional Model for the Study of Human Neuroendocrine Tumor Therapy
Published on: August 14, 2012
Emerging Diagnostics and Therapies in Neuroendocrine Neoplasms: A Critical Review
Jorge H Hernandez-Felix1,2, Monica Isabel Meneses-Medina2, Rachel Riechelmann3
1Developmental Therapeutics Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
Abstract:
Neuroendocrine neoplasms (NENs) are biologically diverse tumors. This article is a critical review of recent evidence focusing on systemic therapies (through mid-2025). We summarize what is most practice-relevant and where gaps remain. In diagnosis, somatostatin-receptor PET/CT has largely replaced older scintigraphy, and adding FDG PET can flag more aggressive disease. Blood-based tests and selected tissue markers (e.g., MGMT, DAXX/ATRX/ALT) show promise but require cautious interpretation in routine care. In treatment, radioligand therapy (PRRT) is used earlier in appropriate receptor-positive disease; cabozantinib improves progression-free survival after prior therapy; and belzutifan offers a biomarker-guided option for malignant pheochromocytoma/paraganglioma. Immunotherapy remains limited to defined subsets, including high-grade neoplasms. We appraise strengths and limitations of key trials, note issues of access and toxicity, and highlight active areas in development (SSTR antagonists, alpha emitters, and dose-guided PRRT). Our goal is to provide a concise, evidence-based map of the field to support informed clinical judgment and future research priorities.
Insights
Recent advances in neuroendocrine neoplasms (NENs) systemic therapies include improved diagnostics like somatostatin-receptor PET/CT and new treatments such as radioligand therapy (PRRT). Evidence guides clinical judgment for diverse NENs.
Area of Science:
- Oncology
- Nuclear Medicine
- Pharmacology
Background:
- Neuroendocrine neoplasms (NENs) represent a heterogeneous group of tumors with evolving therapeutic strategies.
- Accurate diagnosis and staging are crucial for effective management of NENs.
- Systemic therapies have advanced, requiring a review of current evidence and future directions.
Purpose of the Study:
- To critically review recent evidence on systemic therapies for neuroendocrine neoplasms (NENs) up to mid-2025.
- To identify practice-relevant findings and remaining gaps in the diagnosis and treatment of NENs.
- To provide an evidence-based overview for clinical decision-making and research prioritization.
Main Methods:
- Review of recent scientific literature focusing on systemic therapies for NENs.
- Analysis of diagnostic advancements, including PET/CT imaging and molecular markers.
- Evaluation of clinical trial data for systemic treatments like PRRT, cabozantinib, belzutifan, and immunotherapy.
Main Results:
- Somatostatin-receptor PET/CT is now standard for diagnosis, with FDG PET identifying aggressive disease.
- Radioligand therapy (PRRT) is increasingly used early for receptor-positive NENs.
- Cabozantinib and belzutifan show efficacy in specific NEN contexts, while immunotherapy is limited.
Conclusions:
- The diagnostic and therapeutic landscape for NENs is rapidly evolving, with significant progress in systemic therapies.
- Emerging treatments like SSTR antagonists and alpha emitters warrant further investigation.
- An evidence-based approach is essential for optimizing NEN management and guiding future research.
