title: Thymic malignancies: from clinical management to targeted therapies.
abstract: OBJECTIVE
  A key challenge in the treatment of thymoma and thymic carcinoma (TC) is in improving our understanding of the molecular biology of these relatively rare tumors. In recent years, significant efforts have been made to dissect the molecular pathways involved in their carcinogenesis. Here we discuss the results of large-scale genomic analyses conducted to date and review the most active chemotherapies and targeted treatments.
  
  
  METHODS
  We reviewed the literature for chemotherapeutic trials in the last 20 years and trials involving targeted therapies between 1999 and 2010. The search was supplemented by a review of abstracts presented at the annual meetings of the American Society of Clinical Oncology (from 1999 to 2010), at the first International Conference on Thymic Malignancies in 2009, and at a follow-up meeting of the newly formed International Thymic Malignancies Interest Group in 2010.
  
  
  RESULTS
  Surgery remains the treatment of choice for operable tumors, whereas chemotherapy is standard in locally advanced and metastatic disease. Thus far, targeted therapies have been developed empirically. Histone deacetylase inhibitors have shown some activity in thymoma whereas sunitinib may be active in TC. There are no data to support the use of HER2- or EGFR-targeted therapies in thymic malignancies.
  
  
  CONCLUSIONS
  Drug development for the treatment of thymic malignancies is difficult because of the rarity of these tumors. Ethnic differences are becoming apparent, with aggressive subtypes being observed in Asians and African Americans. Incremental improvements in our understanding of tumor biology suggest that molecular profiling-directed therapies may be the preferred route of investigation in the future.
authors: National Cancer Institute, National Institutes of Health, Bethesda, MD 20892-1457, USA.
authors: Ronan J
authors: RJ
authors: Kelly
authors: Iacopo
authors: I
authors: Petrini
authors: Arun
authors: A
authors: Rajan
authors: Yisong
authors: Y
authors: Wang
authors: Giuseppe
authors: G
authors: Giaccone
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000818:Animals; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D066246:ErbB Receptors / Q000037:antagonists & inhibitors; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D009367:Neoplasm Staging; D015282:Octreotide / Q000627:therapeutic use; D019009:Proto-Oncogene Proteins c-kit / Q000037:antagonists & inhibitors; D020917:Receptor, trkA / Q000037:antagonists & inhibitors; D013953:Thymus Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology
chemical_list: D020533:Angiogenesis Inhibitors; D056572:Histone Deacetylase Inhibitors; D066246:ErbB Receptors; D019009:Proto-Oncogene Proteins c-kit; D020917:Receptor, trkA; D015282:Octreotide