Abstract
Neuroendocrine neoplasms (NENs) are heterogeneous malignancies which are becoming
more common. Systemic treatment is considered for patients with advanced disease,
and treatment decisions are often driven by the histological grade of the tumor and
the site of primary. Somatostatin analogues are the first-line option of choice for
gastroenteropancreatic NENs but subsequent options may include the targeted agents
everolimus and sunitinib as well as peptide receptor radionuclide therapy. Telotristat
is a new option for the treatment of refractory carcinoid syndrome diarrhea. Chemotherapy
is infrequently used for Grade 1 to 2 NENs (except for the combination of capecitabine
and temozolomide) but is the mainstay of therapy for Grade 3 neuroendocrine carcinomas.
Bronchial NENs are graded differently and there are few proven options for systemic
treatment. Optimal integration of available systemic therapies, the timely recognition
of tumor heterogeneity, and the use of nuclear medicine are areas of ongoing research.
Finally, the patient experience is impacted by factors such as delayed diagnosis and
symptoms of carcinoid syndrome. Clinicians need to account for patient priorities
and disease characteristics to individualize therapy choices for patients with advanced
NEN.
Keywords
neuroendocrine tumors - somatostatin - everolimus - PRRT - neuroendocrine carcinomas
