A rare malignant disease with incidence about 0.02 per hundred thousand person-years.
Fewer than 100 cases are diagnosed annually in the US.
Patients are typically 50 to 70 years of age at presentation.
Categorized as type C thymomas.
The majority of these lesions are a squamous cell carcinoma that penetrate the thin thymic epithelial capsule, invade mediastinal structures, and disseminate to the pleura.
PET scan: standardized uptake value (SUV) for thymic carcinoma is considered to be significantly greater than that for invasive or noninvasive thymoma, often with an SUV cutoff point of 5.0, thymic carcinoma can be differentiated from thymoma with reasonably high sensitivity (84.6%), specificity (92.3%), and accuracy (88.5%).
Differential diagnosis: aggressive mediastinal germ cell tumor
primary mediastinal lymphoma with invasive spread: lack infiltration
lung cancer with mediastinal invasion
Maximal surgical resection is typically recommended for localized phonic carcinoma.
Radiotherapy is supported by large retrospective studies associated with improved outcomes.
Cytotoxic chemotherapy program, death ligand checkpoint immunotherapy are other measures.
Durable responses with the above treatments have been observed in 15 to 20% of patients.