Liver Cancers pp 245-258 | Cite as

Making the Diagnosis of Neuroendocrine Tumour Disease

  • Vandana M. Sagar
  • Mona Elshafie
  • Tahir Shah


Neuroendocrine tumours (NETs) are generally slow-growing cancers with a rising incidence. Public Health England recorded 8726 neuroendocrine neoplasms (3978 NETs) over 2 years (2013–2014), yielding an overall incidence rate of 8 per 100,000 persons. NETs are often diagnosed incidentally, but most are discovered following investigation of symptoms. NETs commonly arise in the gastrointestinal tract, pancreas or lung, although primary site could be anywhere in the body. Local and distal metastases are common at diagnosis. Gastrointestinal NETs can cause life-threatening peritoneal and cardiac valve fibrosis.

Diagnosis of NETs relies on clinical features, biochemical assessment, imaging and histopathology. They are histologically graded from 1 to 3 according to the WHO 2010 classification system. Radiological assessment relies on anatomic and functional imaging.

Early diagnosis of NETs is challenging due to the rare nature of the disease as well as their presentation with non-specific symptoms. Patients are commonly diagnosed late, when metastatic disease is present and cure is not possible. There is a need for earlier diagnosis to increase treatment options and improve prognosis.


Neuroendocrine tumours Carcinoid Functional imaging Carcinoid heart disease Somatostatin receptors 



We would like to thank Dr. Salil Karkhanis for his contribution in writing this chapter.


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Vandana M. Sagar
    • 1
  • Mona Elshafie
    • 1
  • Tahir Shah
    • 1
  1. 1.Birmingham Neuroendocrine Tumour CentreQueen Elizabeth HospitalBirminghamUK

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