From the Guidelines
Neuroendocrine cell proliferation should be managed based on the principles outlined for sporadic small intestinal (SI)-NENs and pancreatic NENs (Pan-NENs), considering key features such as proliferative activity, somatostatin receptor (SSTR) expression, tumour growth rate, and extent of the disease. When dealing with neuroendocrine cell proliferation, it's crucial to understand that these cells can give rise to well-differentiated neuroendocrine tumours (NETs) and poorly differentiated neuroendocrine carcinomas (NECs), with NECs representing only 10%–20% of all NENs 1. The management of neuroendocrine cell proliferation involves several factors including the location of the tumor, its size, and whether it has metastasized. Key considerations in managing neuroendocrine cell proliferation include:
- Proliferative activity of the tumor
- Expression of somatostatin receptors (SSTR)
- Tumor growth rate
- Extent of the disease Treatment options may vary but generally include surgical resection for localized tumors, and for metastatic or unresectable disease, treatments such as somatostatin analogs, peptide receptor radionuclide therapy (PRRT), targeted therapies, and chemotherapy may be considered, as outlined in guidelines such as those for gastroenteropancreatic neuroendocrine neoplasms 1. Monitoring disease progression and treatment response with imaging studies and biomarkers is essential for effective management.
From the Research
Neuroendocrine Cell Proliferation
- Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms with an annual incidence of ~35 cases per 100,000 people in the United States 2.
- Well-differentiated NETs are often indolent and slow-growing, while poorly differentiated neuroendocrine carcinomas (NECs) are aggressive and have a poor prognosis 2.
- Somatostatin analogues (SSAs) have been shown to have an anti-proliferative effect in patients with well- to moderately differentiated NETs 2, 3, 4.
Treatment Options
- Long-acting SSAs, such as octreotide and lanreotide, are commonly used for symptoms control and have been shown to have anti-proliferative effects in patients with NETs 2, 3, 5.
- The combination of SSA with peptide receptor radionuclide therapy (PRRT) has proven efficacy in the treatment of small intestinal NETs 3.
- Radiolabeled SSAs, such as 177Lu-DOTATATE, have been shown to significantly improve progression-free survival in patients with progressive midgut NETs 6.
Clinical Practice
- The use of SSAs in clinical practice is guided by evidence and guidelines, but some clinical indications remain controversial 5.
- The sequencing of somatostatin-receptor-based therapies in NET patients is a key question in management, particularly given the heterogeneity of NETs and the near absence of randomized trials comparing active treatment options 6.
- Treatment decisions should be made on a case-by-case basis, taking into account the individual patient's characteristics and the availability of treatment options 5, 6.