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Horm Metab Res 2020; 52(08): 614-620
DOI: 10.1055/a-1110-7251
Review

Medical Treatment of Gastrointestinal Neuroendocrine Neoplasms

Marina Tsoli
1   1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Krystallenia Alexandraki
1   1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Christos Xanthopoulos
1   1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece
,
Eva Kassi
1   1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece
2   Department of Biological Chemistry, National and Kapodistrian University of Athens, Athens, Greece
,
Gregory Kaltsas
1   1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece
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Abstract

Neuroendocrine neoplasms (NENs) are rare tumours that arise mainly in the gastrointestinal or pulmonary system. Most NENs are well-differentiated and may obtain prolonged survival besides the presence of metastatic disease; however, a subset (poorly differentiated NENs) may display a truly aggressive behaviour exhibiting a poor prognosis. The recently developed classification systems along with advances in functional imaging have helped stratify patients to the administration of appropriate therapeutic options. Surgery is the mainstay of treatment of NENs, but in recent decades there has been a considerable evolution of medical treatments that are used for locally advanced or metastatic disease not amenable to surgical resection. Long acting somatostatin analogues are the main therapeutic modality for patients with functioning and well-differentiated low grade NENs exhibiting symptomatic control and mainly stabilisation of tumour growth. Other systemic treatments include chemotherapy, molecular targeted agents, interferon-α, peptide receptor radionuclide therapy (PRRT), and immunotherapy. In addition, new agents such as telotristat may be used for the control of symptoms of carcinoid syndrome. The choice and/or sequence of therapeutic agents should be individualized according to tumour origin and differentiation, disease burden, presence of clinical symptoms and patients’ performance status in the context of a multidisciplinary approach. Recent advances in the molecular pathogenesis of NENs set the field for a more personalised treatment approach.

Key words

neuroendocrine tumour - somatostatin analogues - radionuclides - targeted treatment - carcinoid syndrome


Publication History

Received: 01 July 2019

Accepted: 22 January 2020

Article published online:
27 February 2020

© Georg Thieme Verlag KG
Stuttgart · New York

 

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