Systemic therapy for unresectable advanced or metastatic pancreatic and midgut neuroendocrine tumours

Guideline Objective

To make recommendations with respect to systemic therapy for the treatment of patients with pancreatic neuroendocrine tumours (pNETs) and midgut neuroendocrine tumours (midgut NETs).

Patient Population

Adults with a diagnosis of advanced and metastatic pNETs and midgut NETs that have been deemed unresectable after assessment by a neuroendocrine specialist in a multidisciplinary setting.

Patients with neuroendocrine carcinomas (NECs) (i.e., poorly differentiated), malignant neuroblastoma, pituitary tumours, thymic tumours, goblet cell carcinoma, bronchial NETs, paragangliomas, mixed NETs, pheochromocytoma, small cell lung cancer, and thyroid cancer are excluded. 

Intended Guideline Users

All clinicians involved in the treatment of patients with pNETs and midgut NETs.

Research Questions

1. Which of the anti-neoplastic systemic therapies (Table 4-1) is the most effective in improving clinical outcomes (i.e., PFS, OS) and quality of life while minimizing adverse events in patients with unresectable advanced or metastatic pNETs? 
2. Which of the anti-neoplastic systemic therapies (Table 4-1) is the most effective in improving clinical outcomes (i.e., PFS, OS) and quality of life while minimizing adverse events in patients with unresectable advanced or metastatic midgut NETs?