Neuroendocrine Tumors Disease Summary

Overview

Neuroendocrine tumors are rare, small, slow-growing epithelial neoplasms with predominant neuroendocrine differentiation found in most organs of the body and arise from cells throughout the diffuse endocrine system, as stated in the Introduction section.

There is an increased incidence of neuroendocrine tumors seen in Western countries compared to Asian and European countries. A detailed discussion about the prevalence of neuroendocrine tumors is in the Epidemiology section.

The Pathophysiology section states that neuroendocrine tumors have cytologic features of an endodermal origin, and NETs have the ability to store and secrete various peptides and neuroamines. 

Neuroendocrine Tumors_Disease Summary

History and Physical Examination

The Clinical Presentation section describes the clinical features suggestive of neuroendocrine tumors depending on the type.

Diagnosis

The Diagnosis or Diagnostic Criteria section features the types of neuroendocrine neoplasm according to differentiation, grade, and proliferative rate.

Patients with neuroendocrine tumors are recommended to have a genetic risk assessment, which is further discussed in the Screening section.

Discussions on biochemical markers such as chromogranin A, 5-hydroxyindoleacetic acid, and neuron-specific enolase in the evaluation of neuroendocrine tumors are in the Laboratory Tests and Ancillaries section.

Imaging studies such as computed tomography scan, magnetic resonance imaging, ultrasonography, somatostatin receptor-positron emission tomography scan and scintigraphy are discussed in detail in the Imaging section. Invasive procedures such as bronchoscopy, endoscopic ultrasonography, mediastinoscopy, endoscopy, colonoscopy, and echocardiogram are also discussed in this section.

Management

The American Joint Committee on Cancer (AJCC) and European Neuroendocrine Tumor Society (ENETS) developed the TNM System for NETs of all anatomical sites. The AJCC prognostic staging of neuroendocrine tumors depending on the site is presented in the Evaluation section.

General therapy principles and goals of therapy for neuroendocrine tumors are discussed in the Principles of Therapy section.

The Pharmacological Therapy section discusses in detail treatment options of neuroendocrine tumors, such as somatostatin analogs, mammalian target of rapamycin inhibitors, kinase inhibitors, interferon alpha and immunotherapy. Chemotherapy as an option for the treatment of neuroendocrine tumors is also discussed in this section.

Surgical management of neuroendocrine tumors is considered after a full multidisciplinary evaluation. Curative and palliative surgery options are explained in the Surgery section.

The Radiotherapy section discusses radioisotope therapy and radiotherapy as a treatment option in neuroendocrine tumors depending on patient factors, disease site and stage.

The Monitoring section identifies follow-up recommendations for patients with neuroendocrine tumors, depending on the duration and status of the disease.