[Systemic treatment of patients with metastatic neuroendocrine Neoplasia]

Area of Science:

• Oncology
• Endocrinology
• Pharmacology

Background:

• Metastatic neuroendocrine neoplasms (NEN) present complex and heterogeneous challenges.
• Current treatment strategies require an interdisciplinary expert team for individualized approaches.

Purpose of the Study:

• To review the standard of care for systemic therapy in metastatic NEN.
• To outline criteria for treatment selection.
• To discuss new developments in systemic therapy for metastatic NEN.

Main Methods:

• Literature review of current standards and emerging treatments for metastatic NEN.
• Analysis of treatment options based on tumor type (NET vs. NEC), receptor status, and proliferative index.
• Evaluation of first- and second-line therapies, including somatostatin analogs (SSA), peptide receptor radionuclide therapy (PRRT), everolimus, and tyrosine kinase inhibitors.

Main Results:

• SSA are the mainstay for antisecretory treatment and first-line antiproliferative therapy in receptor-positive, low-proliferative NET.
• PRRT is established as second-line treatment for intestinal metastatic disease.
• Therapeutic options for pancreatic NET are diverse, lacking a standard sequence, while lung NET has limited approved options (everolimus).
• Standard first-line treatment for metastatic neuroendocrine carcinoma (NEC) remains platinum + etoposide, with urgent need for novel options.

Conclusions:

• Individualized treatment strategies are crucial for metastatic NEN, guided by tumor characteristics and expert consensus.
• While SSA and PRRT are mainstays, new agents and combinations are under investigation, particularly for NEC and refractory NET.
• Further research is needed to improve outcomes for patients with metastatic NEN, especially NEC.