Carcinoid Tumors

In: Endotext [Internet]. South Dartmouth (MA):, Inc.; 2000–.


The incidence of carcinoid tumors is rising. Their expression of hormones or amines is variable and rate of growth slow, which may make diagnosis erroneous or late. Major clinical features remain diarrhea, flushing, bronchospasm, and carcinoid heart disease. There are new and emerging syndromes such as pigmentation, myopathy, neuropathy and other paraneoplastic syndromes. Important biomarkers include 5HIAA, Chromogranin A(CgA), Neurokinin A(NKA), and pancreastatin. Targeted therapies and aggressive debulking are effective methods of controlling tumor burden. Octreotide and Lanreotide are effective at controlling symptoms. Escape can occur but is minimized by targeting drug plasma levels known to saturate the somatostatin receptors. Addition of telotristat, which works by a different mechanism, is effective in patients with refractory diarrhea. Newer biologics, including sunitinib and mTOR inhibitors, are effective based upon solid clinical trial data. Other advances including Gallium DOTATOC scanning and peptide receptor radiation therapy (PRRT) have now become a reality in the USA. For complete coverage of this and all related areas of Endocrinology, please visit our FREE on-line web-textbook,

Publication types

  • Review