Neuroendocrine neoplasms (NENs) are rare, heterogeneous neoplasms with increasing incidence and prevalence, particularly within the gastroenteropancreatic (GEP) system. A significant proportion of patients present with or develop metastatic disease, most commonly to the liver, which profoundly influences prognosis and therapeutic strategies. Despite the indolent nature of many NENs, liver metastases (NELMs) are associated with reduced survival and increased morbidity, particularly in functional tumors. Surgical intervention remains a cornerstone in the management of NENs, even in the metastatic setting. Resection of the primary tumor, particularly in small bowel and pancreatic NENs, has showed survival benefits and reduced complication rates, even when liver metastases are unresectable. Hepatic resection, when feasible, offers the only curative potential and is associated with 5-year overall survival rates of 61–74%. Cytoreductive surgery, aiming to remove ≥70% of tumor burden, provides symptom relief and survival benefits, even in noncurative settings. Liver transplantation (LT) is a radical but potentially curative option for selected patients with liver-only disease. Under strict criteria, well-differentiated tumors, limited hepatic burden, absence of extrahepatic disease, and disease stability, LT has shown excellent long-term outcomes, with 10-year survival rates exceeding 80% in some highly specific cohorts. The integration of systemic therapies, including somatostatin analogs, targeted agents, and peptide receptor radionuclide therapy (PRRT), further enhances treatment outcomes. Ultimately, the management of metastatic NETs requires a multidisciplinary, individualized approach. Surgical strategies, when appropriately applied, play a pivotal role in prolonging survival, improving quality of life, and enabling access to other therapeutic modalities.

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Role of Surgery: Metastatic Disease

  • Jorgelina Coppa

摘要

Neuroendocrine neoplasms (NENs) are rare, heterogeneous neoplasms with increasing incidence and prevalence, particularly within the gastroenteropancreatic (GEP) system. A significant proportion of patients present with or develop metastatic disease, most commonly to the liver, which profoundly influences prognosis and therapeutic strategies. Despite the indolent nature of many NENs, liver metastases (NELMs) are associated with reduced survival and increased morbidity, particularly in functional tumors. Surgical intervention remains a cornerstone in the management of NENs, even in the metastatic setting. Resection of the primary tumor, particularly in small bowel and pancreatic NENs, has showed survival benefits and reduced complication rates, even when liver metastases are unresectable. Hepatic resection, when feasible, offers the only curative potential and is associated with 5-year overall survival rates of 61–74%. Cytoreductive surgery, aiming to remove ≥70% of tumor burden, provides symptom relief and survival benefits, even in noncurative settings. Liver transplantation (LT) is a radical but potentially curative option for selected patients with liver-only disease. Under strict criteria, well-differentiated tumors, limited hepatic burden, absence of extrahepatic disease, and disease stability, LT has shown excellent long-term outcomes, with 10-year survival rates exceeding 80% in some highly specific cohorts. The integration of systemic therapies, including somatostatin analogs, targeted agents, and peptide receptor radionuclide therapy (PRRT), further enhances treatment outcomes. Ultimately, the management of metastatic NETs requires a multidisciplinary, individualized approach. Surgical strategies, when appropriately applied, play a pivotal role in prolonging survival, improving quality of life, and enabling access to other therapeutic modalities.