About
Although neuroendocrine tumors (NET) of the appendix are rare, they are one of the most common cancers of the gastrointestinal system in children. These tumors cause symptoms similar to those associated with appendicitis, which is why these tumors are often discovered by chance after surgery for appendicitis.
The follow-up treatment of children in whom a NET of the appendix has been found is largely based on research done in adults. As a result, there are currently no specific guidelines for the treatment of children. As a result, there are currently several standard treatments for children with these tumors, while it is still unclear what the best treatment is. These tumors can be divided into low-risk and high-risk groups. Current treatment guidelines recommend a second extensive colon surgery in the high-risk patients. Unlike adults, the disease course in children appears to be much more favorable. However, large-scale research on the treatment of this type of cancer in children is lacking. Therefore, the purpose of this project is to investigate whether the comprehensive second operation on the colon is necessary in children in the high-risk group.
To examine the outcomes of as many children as possible, this project is collaborating with hospitals around the world. First, the outcomes of children treated for NET of the appendix over the past 30 years will be examined. Then, these outcomes will be used by an international expert group (consisting of pediatric surgeons, pediatric oncologists, quality of life experts, and patient representatives) to make recommendations for a pediatric guideline. Ultimately, we expect this guideline to provide a new classification of low-risk and high-risk tumors in children, which will ensure fewer additional surgeries, fewer complications, and a better quality of life for children with this type of cancer. In addition, with these results, physicians will be able to better educate children and parents about the treatment and likely course of this cancer