Thymomas and
thymic carcinomas (TC) are rare epithelial
tumors of the thymus. Although most
thymomas have organotypic features (i.e., resemble the normal thymus), TC are morphologically undistinguishable from
carcinomas in other organs. Apart from their different morphology, TC and
thymomas differ also in functional terms (TC, in contrast to
thymomas, have lost the capacity to promote the maturation of intratumorous lymphocytes), have different genetic features (discussed in this review), a different immunoprofile (most TC overexpress c-KIT, whereas
thymomas are consistently negative), and different clinical features (TC, in contrast to
thymomas, are not associated with paraneoplastic
myasthenia gravis). Thus, although all the data suggest that the biology of
thymomas and TC is different, in clinical practice, their therapeutic management up to now is identical. In the age of
personalized medicine, the time may have come to think this over. We will briefly review the molecular genetics of malignant
thymic tumors, summarize the current status of targeted
therapies with an emphasis on the multitargeted
kinase inhibitors
sunitinib and
sorafenib, and try to outline some future directions.