Systemic treatment for advanced neuroendocrine tumours

Gastroenteropancreatic and bronchopulmonary neuroendocrine tumours are diagnosed with in creasing incidence and have a high prevalence, explained by their relatively indolent nature. The pathological and clinical heterogeneity, the ability of these tumours to produce hormones, and the variability in clinical outcomes pose special challenges in the management of these patients. These tumours are featured by the common expression of somatostatin receptors, providing molecular targets useful both for diagnosis and treatment.

In this review, we will briefly present the various drug classes used in the state-of-the-art systemic therapies with attention to the importance of site-specific characteristics in treatment decisions. We will present cabozantinib, the recently approved drug for NET treatment and also discuss the practical aspects of peptide receptor radio nuclide therapy using 177Lu-DOTATATE. Molecular profiling, both by pathological methods and by comprehensive genomic analysis, is encouraged to identify therapeutic targets. Multidisciplinary evaluation remains crucial to tailoring and sequencing systemic treatment options based on tumour characteristics and patient factors.