7 months and the 1 year survival rate was 62%. Studies describing children with thymic carcinoma are scarce. Yaris reviewed the English literature and described 15 cases under 18 years of age, most of whom presented selleck bio with an anterior mediastinal mass and suf fered from chest pain, cough, fever, weight loss, and respiratory distress. Radiologically, these tumours were often associated with pleural effusions and Inhibitors,Modulators,Libraries or the invol vement of neighbouring structures such as the pleura and pericardium. Nine patients died within 1. 5 to 15 months of their diagnosis, 4 were alive from 1 to 12 years Inhibitors,Modulators,Libraries after their diagnosis. A recent report from the Polish Rare Tumour group described 9 children with thymic carcinomas 2 were classified as Masaoka stage II, 5 as stage III, and 2 as stage IV.

Only 1 patient underwent complete tumour resection at diagnosis, six received multidrug che motherapy and 4 had radiotherapy. The outcome was dismal, and only 2 children were long term survivors. The cases of thymic carcinoma in our series all had unfavourable Inhibitors,Modulators,Libraries features they all presented with large masses and evidence of local or distant spread. Only a diagnostic biopsy was performed in 3 patients and an attempt at tumour resection in 2 left macroscopic residuals. Despite the administration of chemotherapy and radiotherapy, these tumours remained unresectable and all patients died. Four patients in our series received radiotherapy, but it failed to reduce the tumour bulk. In the series pub lished by the Polish group, 4 children were irradiated and 2 of them were still alive, though one of them unfortunately developed severe neurological sequelae due to radiation induced spinal damage.

Although thymic tumours seem to be sensitive Inhibitors,Modulators,Libraries to che motherapy, the most effective regimen remains to be established. Different drug combinations have been used, generally based on cisplatin, doxorubicin, cyclo phosphamide and prednisone. In our experience, we only found evidence of a short lived tumour response in 3 children treated with cisplatin based chemotherapy. Similar results were obtained by the Polish group, with Inhibitors,Modulators,Libraries 4 out of 5 assessable children showing a tumour response after initial chemotherapy. Different regimens were administered, however, making it hard to say which is the most effective combination.

More effective drugs are therefore needed and tar geted molecular therapies might pave the way to new therapeutic options sellekchem in patients with thymic carcinoma in advanced stages. Strobel et al. described the first case of a carcinoma with an activating KIT mutation and suggested that screening for activating KIT muta tions may identify KIT expressing carcinomas that could benefit from imatinib. The same authors described 4 patients of metastatic thymic carcinoma refractory to conventional therapies who were treated with sunitinib, a multi targeted tyrosine kinase inhibitor.