Neuroendocrine tumors of the thymus and mediastinum

Neuroendocrine tumors of the thymus (tNET) and mediastinum are very rare neoplasms with scarce available data. All subtypes [typical and atypical carcinoid tumors (TC and AC), large cell neuroendocrine and small cell carcinoma (SCC)] observed elsewhere in the body occur also in the mediastinum and show only few if any organ-specific morphological differences. Although all available data suggest that the broad principles that govern the biology (and hence) the classification of these tumors in general apply also to tNET, there are a few noteworthy peculiarities related e.g., to risk factors, relative frequency and also to molecular genetic features. In this review, we will briefly summarize current knowledge on tNET with a special emphasis on shared and private features in comparison e.g., with pulmonary NET, which have traditionally been regarded the next closely related NET group.