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<p>The individualized systemic treatment for neuroendocrine tumors (NET) of the gastroenteropancreatic (GEP) system needs to consider primary tumor location, tumor grading, tumor load, clinical need for remission, tumor dynamics/growth rate, functionality and somatostatin receptor expression status (theranostics). Treatment decision making should also include quality of life (QoL) aspects. Established systemic treatment options include the somatostatin analogues octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTA-TATE, chemotherapy with capecitabine/temozolomide or streptozotocin/5-fluorouracil and targeted treatment using everolimus, sunitinib or cabozantinib. For symptom control of diarrhea in carcinoid syndrome the oral serotonin synthesis inhibitor telotristat ethyl is also available. Current guideline recommendations from various specialist medical societies, such as the European Neuroendocrine Tumor Society (ENETS) or the American Society of Clinical Oncology (ASCO) provide specific recommendations for action on differentiated systemic treatment options of NETs of the jejunum and ileum, pancreatic NET and in the relatively rare subgroup of highly proliferative NET G3 (which needs to be differentiated from neuroendocrine carcinoma, NEC). So far, immunotherapy with checkpoint inhibitors does not have an established role in unselected NET G1/G2. Novel emerging treatment strategies, such as the oral nonpeptidergic somatostatin receptor agonist paltusotine or molecular pathology/personalized treatment in NETs need to be further investigated.</p>

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Medikamentöse Systemtherapien bei neuroendokrinen Tumoren

  • 期刊论文

  • 发表日期：

    2025年11月11日

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  • Christoph Josef Auernhammer,
  • Julian Walter Holch,
  • Bernhard Ismann,
  • Kathrin Heinrich,
  • Rudolf Alexander Werner,
  • Christine Spitzweg

摘要

The individualized systemic treatment for neuroendocrine tumors (NET) of the gastroenteropancreatic (GEP) system needs to consider primary tumor location, tumor grading, tumor load, clinical need for remission, tumor dynamics/growth rate, functionality and somatostatin receptor expression status (theranostics). Treatment decision making should also include quality of life (QoL) aspects. Established systemic treatment options include the somatostatin analogues octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTA-TATE, chemotherapy with capecitabine/temozolomide or streptozotocin/5-fluorouracil and targeted treatment using everolimus, sunitinib or cabozantinib. For symptom control of diarrhea in carcinoid syndrome the oral serotonin synthesis inhibitor telotristat ethyl is also available. Current guideline recommendations from various specialist medical societies, such as the European Neuroendocrine Tumor Society (ENETS) or the American Society of Clinical Oncology (ASCO) provide specific recommendations for action on differentiated systemic treatment options of NETs of the jejunum and ileum, pancreatic NET and in the relatively rare subgroup of highly proliferative NET G3 (which needs to be differentiated from neuroendocrine carcinoma, NEC). So far, immunotherapy with checkpoint inhibitors does not have an established role in unselected NET G1/G2. Novel emerging treatment strategies, such as the oral nonpeptidergic somatostatin receptor agonist paltusotine or molecular pathology/personalized treatment in NETs need to be further investigated.