All (neuro) endocrine tumors of the stomach, intestines and pancreas (carcinoid and islet cell tumors) occur today much more frequently than they did 30 or 40 years ago. In the U.S.A. these tumors increased in the past 30 years by about 700%. Gastric and rectal carcinoids have increased in frequency by about 1000%. Currently in the U.S.A. there are more than five new cases of neuroendocrine tumors per 100,000 inhabitants every year. The general frequency i.e. the number of people who currently suffer or had suffered from this type of tumor is distinctly higher: it is 35 per 100,000. Similar epidemiological observations have been reported from Scandinavia, England and Germany.

Figure 2:
Drastic increase of neuroendocrine tumor disease of the small intestines, rectum,
stomach, and pancreas

figure 2

Modified according to Modlin et al., Lancet Oncology 2008 ; 9 : 61-72

Neuroendocrine tumors of the pancreas are observed 5 to 10 times less often than carcinoids (of the stomach, small intestines, rectum or appendix). Neuroendocrine pancreatic tumors include insulinomas, VIPomas, glucagonomas and, in part, gastrinomas. The reason for the drastic epidemiological increase in neuroendocrine tumors has not been studied and is not well understood; the drastic increase of asymptomatic (often incidentally detected) early neuroendocrine tumors may well be the result of the nowadays general availability of comprehensive imaging and endoscopic techniques.

Insulinoma: Patients with insulinoma usually suffer from low blood sugar levels (hypoglycemia). This hypoglycemia can manifest itself in outbreaks of sweating, hunger attacks, weight gain, increased pulse rate, confusion or even a loss of consciousness.

Glucagonoma: These tumors (of the pancreas) are extremely rare. Patients typically complain of high blood sugar levels and skin rashes.

VIPoma: VIPoma of the pancreas releases the protein vasoactive intestinal peptide. This substance causes massive amounts of watery diarrhea (> 6l/day).

Gastrinoma: Patients with gastrinomas typically suffer from chronic heartburn, chronic diarrhea, and gastric or duodenal ulcers when left untreated. Gastrinomas mainly originate in the duodenum and in the nearby lymph nodes; 25-30% of the gastrinomas derive from the pancreas. There is good and effective treatment of gastrinomas. Thanks to the widespread use of very effective acid inhibiting drugs - namely proton pump inhibitors – gastrinomas are nowadays quite often missed as the underlying cause of ulcer disease.

Carcinoids originate in different locations as follows:

  •       3-9%                        Stomach
  •       2-6%                        Pancreas
  •        ~1%                        Liver and gall bladder
  •       5-42%                      Small intestines
  •       0,4-26%                  Appendix
  •       22-62%                   Rectum and sigma
  •       5-7%                        Remaining colon
  •       20-27%                   Lungs and thymus
  •       10-20%                   Unknown origin

Strikingly, this percentage breakdown varies considerably among different countries (e.g. Taiwan versus Western countries) and among various tumor registries.

Prof. Dr. med. Hans Scherübl


Center of Neuroendocrine Tumors
Prof. Dr. med. Hans Scherübl
Vivantes Klinikum Am Urban
Academic Teaching Hospital of Charité-University Medicine, Berlin
Dieffenbachstraße 1
10967 Berlin, Germany
Tel: + 49 30 130 225201
Fax: + 49 30 130 225205
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