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Management of Functional Pancreatic Neuroendocrine Neoplasms
Ludovica Magi1, Matteo Marasco1, Maria Rinzivillo1
1Digestive Disease Unit, Sant'Andrea University Hospital, ENETS Center of Excellence, Via di Grottarossa 1035, 00189, Rome, Italy.
Opinion Statement:
Functional pancreatic neuroendocrine neoplasms (pNENs) are rare and heterogeneous diseases in terms of both clinical and pathological aspects. These tumors secrete hormones or peptides, which may cause a wide variety of symptoms related to a clinical syndrome. The management of functional pNENs is still challenging for clinicians due to the need to control both tumor growth and specific symptoms. Surgery remains the cornerstone in the management of local disease because it can definitively cure the patient. However, when the disease is not resectable, a broad spectrum of therapeutic options, including locoregional therapy, somatostatin analogs (SSAs), targeted therapies, peptide-receptor radionuclide therapy (PRRT), and chemotherapy, are available. The present review summarizes the main key issues regarding the clinical management of these tumors, providing a specific highlight on their therapeutic approach.
Insights
Functional pancreatic neuroendocrine neoplasms (pNENs) are rare tumors causing hormonal symptoms. Management requires controlling tumor growth and symptoms using surgery or therapies like SSAs, PRRT, and chemotherapy.
Area of Science:
- Endocrinology
- Oncology
- Gastroenterology
Background:
- Functional pancreatic neuroendocrine neoplasms (pNENs) are rare, heterogeneous tumors.
- These neoplasms secrete hormones, leading to diverse clinical syndromes.
- Managing pNENs presents challenges in controlling both tumor progression and associated symptoms.
Purpose of the Study:
- To review the key issues in the clinical management of functional pNENs.
- To highlight the therapeutic approaches for these rare tumors.
Main Methods:
- Literature review summarizing current clinical and pathological knowledge.
- Focus on therapeutic strategies for both resectable and non-resectable pNENs.
Main Results:
- Surgery is the primary curative treatment for localized pNENs.
- For non-resectable disease, options include locoregional therapies, somatostatin analogs (SSAs), targeted therapies, peptide-receptor radionuclide therapy (PRRT), and chemotherapy.
Conclusions:
- Effective management of functional pNENs necessitates a multidisciplinary approach.
- A range of therapeutic options exists to manage tumor growth and symptoms when surgery is not feasible.
