Current updates and future directions in diagnosis and management of gastroenteropancreatic neuroendocrine neoplasms

Table 3 Small intestinal neuroendocrine tumors[96,97,101,102,104,108,109]

	Duodenal	Ampullary	Jejuno-ileal
Epidemiology	2%-3% GEP-NETs	0.3%-1% GEP-NETs	1.2 cases/100000 incidence quadrupled over past 30 yr
Evaluation	> 2 cm: CT and EUS	CT, EUS	Chromogranin A, urine 5-HIAA, CT/MRI, gallium-DOTATATE PET CT, colonoscopy into terminal ileum
5-yr survival	No metastases: 80%-95%; Regional metastases: 65%-75%; Zollinger-Ellison or MEN-1: > 90%	59%	Local disease: 80%-100%; Regional disease: 70%-80%; Distant metastases: 35%-80%
Treatment	< 1 cm: Endoscopic resection; 1-2 cm: Endoscopic or surgical resection; > 2 cm: EMR or ESD, surgical resection for regional disease	< 2 cm superficial without metastases: Pancreaticoduodenectomy or consider endoscopic ampullectomy; > 2 cm: Pancreaticoduodenectomy	Surgery; Carcinoid syndrome: Long-acting SSA (octreotide LAR 20-30 mg IM)
Surveillance	EGD at least every 2 yr	EGD at 1-2 yr interval	NANETS: Curative surgery-CT every 3-6 mo then 6-12 mo for 7 yr; Advanced disease- CT every 6 mo; ENETS: Curative surgery: Chromogranin A, urine 5-HIAA, CT every 6-12 mo; Slow-growing treated without curative intent: every 3-6 mo

EUS: Endoscopic ultrasound; EGD: Esophagogastroduodenoscopy; GEP-NETs: Gastroenteropancreatic neuroendocrine tumors; PET: Positron emission tomography; CT: Computed tomography; MRI: Magnetic resonance imaging; SSAs: Somatostatin analogues; LAR: Long-acting release; HIAA: Hydroxyindoleacetic acid.