Neuroendocrine neoplasms (NEN) originating from the small bowel comprise, after pancreatic NEN, probably the largest subgroup of NEN within the gastroenteropancreatic system, and reflect the typical distribution pattern of the endocrine cells of the diffuse endocrine system within the digestive system. Previous ENETS guidelines have discussed NEN of the jejuno-ileum and of the appendix including goblet cell carcinoids/carcinomas (GCC) in two separate papers. This update summarises these entities together in one chapter, while excluding the consideration of metastatic disease in a separate chapter. The chapter on metastatic NEN by Pavel et al. also includes guidelines for medical and other treatment options of the carcinoid syndrome since this occurs almost exclusively only when the tumour is metastatic. Carcinoid heart syndrome (Hedinger’s syndrome),however, is covered in this chapter, since early recognition and treatment is important for this complication of carcinoid syndrome.
Since highly proliferative carcinomas with a neuroendocrine phenotype but poor histological differentiation grade are extremely rare in the jejuno-ileum, management of such cases remains highly individual and can currently not be considered standardised according to organ-oriented guidelines. For the management of such cases, the reader is referred to previous guidelines.
This chapter, therefore, deals with non-metastatic NEN originating from the small bowel and the appendix as originally described as ‘carcinoid’ tumours by Oberndorfer in 1907. For greater ease of use, jejuno-ileal NEN (part 1), appendiceal NEN (part 2) and GCC (part 3) are discussed in separate sections.
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