ABSTRACT
Neuroendocrine neoplasms take origin from neuroendocrine cells and generate a heterogenous group of tumors. The annual incidence is 3-5/100.000. They are most common in the gastrointestinal system and lungs but may develop in almost any organ. Some neuroendocrine tumors are able to secrete metabolically active or inactive mediators that can be used as tumor markers in the diagnosis and follow-up. The markers are classified as general and specific. Chromogranin A is the most frequently used general marker in clinical practice. However high rates of false positivity and false negativity limit its use. Pancreastatin, neuron specific enolase, pancreatic polypeptide are other general markers not as frequently used. Insulin, gastrin, glucagon, vasoactive intestinal peptide are among the hormonal specific markers generally secreted from functional pancreatic neuroendocrine tumors. 5-HIAA is another marker with high sensitivity and specificity for neuroendocrine tumors that cause carcinoid syndrome. The clinical picture of the patient determines which marker will be used. Neuroendocrine tumor related transcript based evaluation test is a novel test that measures tumor transcripts in circulation by polymerase chain reaction. It has high sensitivity and specificity however further studies are needed to integrate its use into clinical practice. Carcinoid crisis is a life threatening state that is caused by sudden release of vasoactive mediators from tumor in systemic circulation. It is generally triggered by surgery and anaesthesia. Carcinoid syndrome, carcinoid heart disease, presence of liver metastasis, high 5-HIAA levels are among risk factors. Octreotide, steroids, anti-histamine drugs are used in the treatment of crisis. There is no consensus in the literature about prophylactic use and treatment protocols of octreotide during crisis and there is need for further studies.
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