ABSTRACT

Neuroendocrine tumors (NETs) are a heterogeneous group of malignant diseases with various clinical presentations and clinical courses. In general, they are slow-growing tumors, but in some instances, they behave in a highly aggressive fashion (neuroendocrine carcinoma [NEC]). Due to their diverse symptoms (sweating, flushing, diarrhea, bronchospasm, and anxiety), diagnosis is often significantly delayed, and lesions therefore are only identified when metastatic spread has occurred [1, 2]. Still, the delay from the first symptom until correct clinical diagnosis is about 4–5 years. Metastases can occur locally, in the mesentery and the adjacent lymph nodes, and by hematogenous spread to the liver, lungs, and bone. In most patients, the liver is the dominating site of metastatic spread, but lung, bone, and brain may also be affected. As a consequence of the substantial percentage of individuals with metastatic disease, most therapeutic strategies are directed at the management of hepatic secondaries or local recurrence [3].