From the Guidelines

Diagnosis and treatment of neuroendocrine tumors (NETs) should be individualized and based on a multidisciplinary approach.

The diagnosis of NETs involves histopathologic assessment to determine prognosis and individualize therapy, with consideration of tumor grade, differentiation, and molecular markers 1.
The management of NETs depends on the primary site, tumor grade, and extent of disease, with options including somatostatin analogs, hepatic-directed therapies, molecularly targeted agents, and cytotoxic chemotherapy 1.
Somatostatin analogs are a key therapeutic option for regulating hormone secretion and tumor growth in NETs, particularly for well-differentiated and low- or intermediate-grade tumors 1.
Molecularly targeted agents, such as everolimus, may be used for antitumor effects in advanced NETs 1.
Hepatic-directed therapies may be considered for liver-predominant disease 1.
Cytotoxic chemotherapy may be used in certain cases, particularly for high-grade NETs or those with distant metastasis 1.

The NCCN Guidelines provide a framework for the diagnosis and management of NETs, with updates annually and interim updates as required 1.
The ESMO Clinical Practice Guidelines focus on the diagnosis, treatment, and follow-up of gastroenteropancreatic neuroendocrine neoplasms, with consideration of key features such as proliferative activity, somatostatin receptor expression, tumor growth rate, and extent of disease 1.
International collaboration and endorsement of guidelines, such as the Commonwealth Neuroendocrine Tumour Research Collaboration and the North American Neuroendocrine Tumor Society, aim to harmonize care and reduce variation in practice 1.

From the Research

Diagnosis of NETs is based on clinical features, hormonal or non-hormonal biomarkers, and histology of a suspected NET 2
Functional imaging using Ga-radiolabeled DOTATATE positron emission tomography/computed tomography can help identify the anatomic primary site or NET subtype 3
Molecular tumor classification based on differential gene expression has demonstrated high diagnostic accuracy in blinded validation studies of different NET types and subtypes 3

Surgical management remains the mainstay of therapy and can be curative for well-differentiated pulmonary carcinoid tumors 4
Medical treatments for NETs include somatostatin analog (SSA) therapy, targeted therapy, chemotherapy, or peptide receptor radionuclide therapy 4, 5
Somatostatin analogues are widely used in patients with symptoms and with carcinoids of low differentiation grade 5
Chemotherapy is active in patients with poorly differentiated neuroendocrine tumors, with the combination of cisplatinum and etoposide being a commonly used therapeutic regimen 5
Peptide receptor radionuclide therapy may be used and has been shown to increase survival and reduce symptoms in some studies 4

A multidisciplinary approach to workup and treatment should be utilized for each patient 4
Treatment plans should be individualized based on patient and tumor characteristics, including extent of disease, grade, pace of growth, functional status, primary site, somatostatin receptor status, performance status, and comorbidities 6
Familiarity with ongoing clinical trials can guide therapeutic decision making 6