Appendix carcinoid associated with the XXXX .

@entity1 @entity390 @entity1127 @entity5 @entity2838 @entity1709 @entity74 @entity490 @entity1412 @entity105 @entity2839
INTRODUCTION: The @entity2838 ( @entity2838 ) is a rare hereditary, @entity74 . It is characterized by a gastrointestinal @entity1412 and mucocutaneous @entity1709 . It has also been reported as a precondition for @entity5 with a life-time-hazard for @entity5 up to 93%, caused by a germline mutation in the @entity2839 gene. PRESENTATION OF CASE: A 21-year-old @entity1 presented with @entity390 and @entity490 . He had a known history of @entity2838 since the age of 13 when he was treated for intussusception due to a @entity1127 . Preoperative diagnostics revealed a second intussusception and an extensive @entity1412 . Intraoperative findings confirmed the suspected diagnoses and desvagination was performed. Nearly 50 @entity1127 were removed from the small intestinum over several longitudinal sections. As the appendix appeared thickened an appendectomy was performed simultaneously. Histology showed @entity1127 and the incidental finding of a pT1 carcinoid of the appendix. The @entity1 recovered well and needed no further treatment for his @entity5 . DISCUSSION: The mechanism of @entity105 in @entity2838 still remains debatable, although the @entity74 underlying the syndrome is known. A predisposition for @entity5 also stays questionable. To our knowledge there is no description of an association between @entity5 of the appendix and @entity2838 to date. CONCLUSION: Life-expectancy in @entity1 with @entity2838 is reduced. Causes are the development of @entity5 and complications from the @entity1127 such as intussusception. Since there is no treatment possible main focus must be aimed at early recognition of @entity5 and the prevention of complications.
