Table 2.

ENETS 2016 recommendations [15]

Drug	Functionality	Grading	Primary site	SSTR status	Special consideration
Octreotide	+/–	G1	Midgut	+	Low tumor burden
Lanreotide	+/–	G1 and G2 (–10%)	Midgut, pancreas	+	Low and high (> 25%) liver tumor burden
Interferon-alpha 2b	+/–	G1/G2	Midgut		If SSTR negative
STZ/5-FU	+/–	G1/G2	Pancreas		Progressive in short term* or high tumor burden or symptomatic
TEM/CAP	+/–	G2	Pancreas		Progressive in short term* or high tumor burden or symptomatic if STZ is contraindicated or not available
Everolimus	+/–	G1/G2	Lung		Atypical carcinoid and/or SSTR negative
			Pancreas		Insulinoma or contraindication for CTX
			Midgut		If SSTR negative
Sunitinib	+/–	G1/G2	Pancreas		Contraindication for CTX
PRRT	+/–	G1/G2	Midgut	+ (required)	Extended disease; extrahepatic disease (e.g. bone metastasis)
Cisplatin#/etoposide	+/–	G3	Any		All poorly differentiated NET

CAP – capecitabine; CTX – chemotherapy; 5-FU – 5-fluorouracil; STZ – streptozotocin; TEM – temozolomide.

^*≤ 6–12 months

^#Cisplatin can be replaced by carboplatin