title: Available evidence and new biological perspectives on medical treatment of advanced thymic epithelial tumors.
abstract: Thymic epithelial tumors (TETs) are rare primary mediastinal tumors arising from thymic epithelium. Their rarity and complexity hinder investigations of their causes and therapy development. Here, we summarize the existing knowledge regarding medical treatment of these tumors, and thoroughly review the known genetic aberrations associated with TETs and the present status of potential biological treatments. Epidermal growth factor receptor (EGFR), stem-cell factor receptor, insulin-like growth factor-1 receptor (IGF1R), and vascular endothelial growth factors (VEGF-A, VEGF-B, and VEGF-2) are overexpressed in TETs. EGFR overexpression in TETs is associated with higher stage, and IGF1R overexpression has poor prognostic value. Data indicate that anti-IGF1R monoclonal antibodies, and inhibitors of angiogenesis, somatostatin receptors, histone deacetylase, mammalian target of rapamycin, and cyclin-dependent kinases may be active against TETs. Continued investigations in this field could lead to advancement of targeted and biological therapies for TETs.
authors.affiliation: Thoracic Oncology Unit, Department of Medical Oncology.
authors.forename: D
authors.initials: D
authors.lastname: Serpico
authors.affiliation: Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan.
authors.forename: A
authors.initials: A
authors.lastname: Trama
authors.affiliation: Thoracic Oncology Unit, Department of Medical Oncology.
authors.forename: E R
authors.initials: ER
authors.lastname: Haspinger
authors.affiliation: Thoracic Oncology Unit, Department of Medical Oncology.
authors.forename: F
authors.initials: F
authors.lastname: Agustoni
authors.affiliation: Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan.
authors.forename: L
authors.initials: L
authors.lastname: Botta
authors.affiliation: Department of Medical Oncology, Universita Politecnica delle Marche, Ancona.
authors.forename: R
authors.initials: R
authors.lastname: Berardi
authors.affiliation: Department of Molecular and Clinical Endocrinology and Oncology, 'Federico II', University, Naples.
authors.forename: G
authors.initials: G
authors.lastname: Palmieri
authors.affiliation: Department of Oncology and Hematology, Humanitas Cancer Center, Rozzano.
authors.forename: P
authors.initials: P
authors.lastname: Zucali
authors.affiliation: Thoracic Oncology Unit, Department of Medical Oncology.
authors.forename: R
authors.initials: R
authors.lastname: Gallucci
authors.affiliation: Laboratory of Molecular Pharmacology, IRCCS 'Mario Negri', Milan.
authors.forename: M
authors.initials: M
authors.lastname: Broggini
authors.affiliation: Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan.
authors.forename: G
authors.initials: G
authors.lastname: Gatta
authors.affiliation: Departments of Thoracic Surgery.
authors.forename: U
authors.initials: U
authors.lastname: Pastorino
authors.affiliation: Pathology.
authors.forename: G
authors.initials: G
authors.lastname: Pelosi
authors.affiliation: Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: F
authors.initials: F
authors.lastname: de Braud
authors.affiliation: Thoracic Oncology Unit, Department of Medical Oncology. Electronic address: marina.garassino@istitutotumori.mi.it.
authors.forename: M C
authors.initials: MC
authors.lastname: Garassino
mesh_terms: D000818:Animals; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D001688:Biological Products / Q000009:adverse effects / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000037:antagonists & inhibitors* / Q000235:genetics / Q000378:metabolism; D006801:Humans; D058990:Molecular Targeted Therapy / Q000009:adverse effects; D009367:Neoplasm Staging; D009375:Neoplasms, Glandular and Epithelial / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D015398:Signal Transduction / Q000187:drug effects*; D013953:Thymus Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D001688:Biological Products; D014408:Biomarkers, Tumor
keywords: biological agents; chemotherapy; targeted therapy; thymic carcinoma; thymic epithelial tumors; thymoma