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Neuroendocrine Tumors Guidelines

Initial Evaluation

For evaluation of duodenal, jejunal, ileal, and colonic neuroendocrine tumors (NETs), the National Comprehensive Cancer Network (NCCN) recommends abdominal/pelvic multiphasic CT or MRI and any of the following as appropriate
:

Somatostatin receptor scintigraphy

Colonoscopy

Small-bowel imaging (CT enterography or capsule endoscopy)

Chest CT

Biochemical evaluation if clinically indicated by symptoms of hormone secretion

For evaluation of T1 rectal NETs, the NCCN recommends endorectal MRI or endoscopic ultrasound (EUS); for T2-T4 rectal NETs, recommended studies are as follows:

Colonoscopy

Abdominal/pelvic multi-phasic CT or MRI

Endorectal MRI or EUS

In addition, the following are recommended to be done, as appropriate:

Somatostatin receptor scintigraphy

Chest CT

Biochemical evaluation if clinically indicated

For evaluation of gastric NETs, NCCN recommendations are as follows:

Esophagogastroduodenoscopy (EGD)

Gastric biopsy

Serum gastrin level

Consider gastric pH, as appropriate

For evaluation of bronchopulmonary NETs, the NCCN recommends chest CT and abdominal multiphasic CT or MRI, with the following as appropriate:

Somatostatin receptor scintigraphy

Bronchoscopy

Biochemical workup for Cushing syndrome, if clinically indicated

Other biochemical evaluation, as clinically indicated

For evaluation of thymus NETs, the NCCN recommends chest/mediastinal multiphasic CT and abdominal multiphasic CT or MRI, the following as appropriate:

Somatostatin receptor scintigraphy

Bronchoscopy

Biochemical workup for Cushing syndrome, if clinically indicated

Other biochemical evaluation, as clinically indicated

European Neuroendocrine Tumor Society (ENETS) consensus guidelines for high-grade gastroenteropancreatic neuroendocrine tumors, issued in 2016, include the following minimal consensus recommendations on diagnosis
:

Clinical signs and symptoms should guide the appropriate diagnostic procedures

Chromogranin A and neuron-specific enolase (NSE) testing is not mandatory, but may be useful if levels are elevated at diagnosis; other hormone tests are not routinely recommended

A minimal diagnostic workup should include site-specific endoscopic assessment with tumor biopsy, and whole-body CT scan (and/or MRI) for tumor staging

In patients with metastatic disease, an ultrasound-guided percutaneous biopsy may be performed if feasible

Somatostatin receptor scintigraphy is not routinely indicated but may be considered in tumors with proliferative indexes in the low range of G3 (Ki-67 < 55%)

Bone scans or brain imaging (CT or MRI) should not be performed in the absence of site-specific symptoms

Fluorodeoxyglucose positron emission tomography (FDG-PET) may be considered in patients in whom radical surgery is being pursued or if clarification of equivocal findings on conventional imaging may change the therapeutic approach; FDG-PET may be useful in resectable cases for whole-body assessment

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