Neuroendocrine Tumors Clinical Practice Guidelines (NCCN, 2021)

National Comprehensive Cancer Network

These are some of the highlights of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

September 30, 2021

Updated clinical guidelines on the diagnosis and management of neuroendocrine and adrenal tumors were released in 2021 by the National Comprehensive Cancer Network (NCCN). The new version of the guidelines, published in the Journal of the National Comprehensive Cancer Network, provides recommendations on genetic risk evaluation and well-differentiated grade 3 neuroendocrine tumors.[1]

Genetic risk evaluation

It is recommended that patients with any of the following undergo genetic risk evaluation: (1) adrenocortical carcinoma (ACC); (2) paraganglioma/pheochromocytoma; (3) gastrinoma (duodenal/pancreatic or type 2 gastric neuroendocrine tumor [NET]); (4) multifocal pancreatic NETs; (5) parathyroid adenoma or primary hyperparathyroidism before age 30 years or multiple parathyroid adenomas, multigland hyperplasia (with no obvious secondary causes), or recurrent primary hyperparathyroidism; (6) clinical suspicion for multiple endocrine neoplasia type 2 (MEN2) owing to the existence of MEN2-related features; (7) a mutation identified on tumor genomic testing that, if also found in the germline, could have a clinical impact; (8) a close blood relative in whom a cancer susceptibility gene carries a known or likely pathogenic variant; (9) a first-degree relative in whom one of the above criteria exists but who is unavailable for testing; and (10) clinical suspicion for MEN1 due to the presence of two or more of the following or the existence of one of the following along with a family history of one or more of the following: primary hyperparathyroidism, duodenal/pancreatic NET, pituitary adenoma, or foregut carcinoid (bronchial, thymic, or gastric). Patients of any age with duodenal/pancreatic NET should also be considered for genetic risk evaluation.

Well-differentiated grade 3 neuroendocrine tumors

It is recommended that patients undergo multiphasic abdominal/pelvic computed tomography (CT) or magnetic resonance imaging (MRI) scans with contrast (with or without chest CT scans [if clinically indicated]), as well as somatostatin-receptor (SSR)–based positron emission tomography (PET) imaging (SSR-PET).

In SSR-based PET scanning, PET-CT imaging or PET-MRI, from the skull base to the midthigh, should be included, when feasible, with intravenous contrast (both arterial and portal venous phase) employed.

Regardless of tumor biology, resection is recommended for locoregional (resectable) disease, along with, if possible, regional lymphadenectomy.

A clinical trial is preferred in resectable locoregional disease with unfavorable biology (ie, typically having a Ki-67 level at or above 55%, being faster growing, and possibly yielding a negative SSR-based PET scan result).

Among asymptomatic patients with unresectable locally advanced or metastatic tumors and a low tumor burden, select individuals can, as an option, undergo observation with a short-interval follow-up scan. Otherwise, it is recommended that patients who are SSR-positive and/or have hormonal symptoms be treated with octreotide or lanreotide.

The preferred option for locally advanced or metastatic disease with unfavorable biology is a clinical trial. Chemotherapy and the combination of nivolumab and ipilimumab are also options.

In patients with locally advanced or metastatic disease with unfavorable biology, pembrolizumab is an option for those in whom a test approved by the US Food and Drug Administration (FDA) has determined the presence of advanced tumor mutational burden-high (TMB-H) tumors, with post-treatment progression having occurred and no satisfactory alternative treatments existing.

Irinotecan-based therapies are a chemotherapeutic option in patients with locally advanced or metastatic disease with unfavorable biology.

In surveillance for resectable locoregional, locally advanced, or metastatic disease, the patient is assessed through a routine history and physical examination as well as via appropriate imaging studies (abdominal/pelvic MRI scans with contrast or abdominal/pelvic multiphasic CT scans, and chest CT scans as clinically indicated), with surveillance being carried out every 12-24 weeks for the first 2 years and every 6-12 months after that, for up to 10 years.

For more information, please go to Neuroendocrine Tumors Guidelines.

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