Gastroenteropancreatic neuroendocrine tumor

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Classification according to ICD-10
C15-C26 Malignant neoplasms of the digestive organs
D13 Benign new formation of other and imprecisely designated parts of the digestive system
ICD-10 online (WHO version 2019)

As Gastroenteropancreatic neuroendocrine tumors (GEP-NET) , a heterogeneous group of hormone-producing tumors of the rule is the digestive system (gastrointestinal tract and pancreas), respectively. They represent the largest subgroup of neuroendocrine tumors (NET). Their dignity can only be determined with certainty when metastases have already occurred; the five-year survival rate for this inhomogeneous group averages 50%.

introduction

GEP-NET represent an extensive range of endocrine and neuroendocrine tumors of the gastroenteropancreatic system (gastrointestinal tract and pancreas). They behave in terms of growth pattern, origin (from the sections of the primitive embryonic intestinal canal), differentiation, functionality (including expression of neuroendocrine marker molecules ) and localization as well as their prognosis are completely different. Overall, they are very rare ( incidence <1: 100,000). GEP-NETs occur more frequently in multiple endocrine neoplasia type 1 and Hippel-Lindau syndrome .

Systematics

GEP-NETs represent the main group of neuroendocrine tumors . 75% of all NETs are GEP-NETs.

Since the GEP-NET appear completely different, different forms of classifications and terminologies have emerged. These include localization-related concepts islet cell tumor, function-related insulinoma, gastrinoma and glucagonoma and dignitätsbezogen Inselzelladenom or -carzinom and carcinoid .

The current WHO classification (from the year 2000) gives priority to the dignity:

  • 1a - highly differentiated neuroendocrine tumor
  • 1b - highly differentiated neuroendocrine carcinoma
  • 2 - low-differentiated neuroendocrine carcinoma

Web links

Individual evidence

  1. ↑ a b c U.-F. Pape: Diagnosis and therapy of gastroenteropancreatic neuroendocrine tumors from an internal perspective. In: The oncologist. Springer-Verlag, 6/7/2000, ISSN  0947-8965 , pp. 624-633, (online at: books.google.de )
  2. ↑ C. Thomas: Histopathology. Schattauer Verlag, 2006, ISBN 3-7945-2429-2 , p. 279, (online at: books.google.de )
  3. ↑ G. Feichter, among other things: Zytopathologie. Springer, 2000, ISBN 3-540-66502-1 , p. 278, (online at: books.google.de )
  4. ↑ a b c A. Schmitt-Gräff et al: Gastroenteropancreatic neuroendocrine / endocrine tumors Current pathological-diagnostic perspective. In: The Pathologist. 22/2/2001, pp. 105–113, ISSN  0172-8113 , (online at: springerlink.com )
  5. ↑ A. Brochert: Surgery on a case-by-case basis. Urban & Fischer-Verlag, 2005, ISBN 3-437-43971-5 , p. 28. (online at: books.google.de )
  6. ↑ G. Klöppel: From carcinoid to neuroendocrine tumor: classification in the gastrointestinal tract and pancreas. In: Dtsch Arztebl. 2003; 100 (28-29), p. A-1932, (online at: aerzteblatt.de )