Functioning neuroendocrine tumors (NET): Minimum requirements for a NET specialist

Area of Science:

• Endocrinology
• Oncology
• Gastroenterology

Background:

• Functioning neuroendocrine tumors (f-NETs) are rare but challenging NETs impacting patient survival and quality of life.
• Paraneoplastic syndromes (PNS) can complicate NETs management due to ectopic hormone production.
• Limited dedicated guidelines exist for f-NETs and PNS management.

Purpose of the Study:

• To review the literature on functioning neuroendocrine tumors (f-NETs).
• To propose a clinical tool for improved diagnosis and management of f-NETs.
• To enhance understanding and treatment of paraneoplastic syndromes associated with NETs.

Main Methods:

• A narrative review of existing literature on functioning neuroendocrine tumors (f-NETs).

Main Results:

• Common f-NETs include insulinomas, gastrinomas, and carcinoid syndrome (CS)-associated NETs.
• Symptoms often overlap with other disorders, necessitating multidisciplinary management.
• Somatostatin analogs (SSAs) are first-line treatment; other therapies include surgery, liver-directed, targeted, and radionuclide therapies.
• Paraneoplastic syndromes can complicate clinical course and treatment response.

Conclusions:

• Management of hormonal syndromes from f-NETs requires a multidisciplinary approach.
• Establishing minimal requirements for NET specialists is crucial for diagnosis and treatment.
• Increased awareness, understanding of pathogenesis, and improved management strategies for f-NETs are needed.