Klinik und Prognose von 276 Patienten mit einem Karzinoidsyndrom – eine retrospektive multizentrische Studie

Introduction Carcinoid syndrome is the most frequent functional syndrome of neuroendocrine neoplasia. It is characterized by flushing, diarrhea, wheezing, hypotension and exanthema and may cause carcinoid heart disease. Methods We assessed clinical characteristics and prognosis of patients with carcinoid syndrome and carcinoid heart disease in 276 patients from three referral centers. Results Carcinoid syndrome patients had a mean age of 57 years (range 21-84) and normal BMI of 24.9 (SD 4.5; range 13.8 - 39.6). Most primaries were of small bowel or unknown primaries with distant metastasis in 94.6%. Flushing was most frequent symptom in 74.3% of patients followed by diarrhea in 68.8% and wheezing in 40.9%. Pain was described by 45.3%, weakness by 23.5% and weight loss of more than 10% in 6 months by 30.1% of patients. Carcinoid heart disease was diagnosed in 37.3% of patients (n=104) by echocardiography and involved predominantly tricuspid valve. Combinations with other valve defects were common. Somatostatin analogues were taken by 80.4% of patients and 17% needed additional loperamide/opium tincture. Surgery and peptide receptor radiotherapy were most frequent treatments. Median survival of patients with carcinoid syndrome after diagnosis was 9 years. Prognosis was significantly impaired by male sex and diagnosis of carcinoid heart disease, but surprisingly significantly increased by presence of symptoms flushing and weakness. Discussion/Conclusion Carcinoid syndrome is associated with extensive disease and primaries in small bowels or of unknown primary. Weight loss, weakness and pain are frequent and carcinoid heart disease are diagnosed in more than one third of patients.