Background: Medulloepithelioma (ME) is a rare embryonal tumor predominantly located in the eye or in the central nervous system without an established treatment.Case presentation: We report of a case of a localized peripheral ME treated with conventional and high dose chemotherapy, surgery and local radiotherapy. At relapse, the tumor tissue revealed a different molecular signature compared to the initial tumor mass. This molecular signature revealed a high expression of platelet derived growth factor receptor (PDGFR). Sorafenib plus irinotecan and temozolomide was started with a 5 month progression free survival.Conclusion: Our experience suggests a possible role of sorafenib or different PDGFR inhibitors in ME. Targeting treatment could represent an adjuvant and/or alternative therapy for ME and other rare tumors. © 2014 De Pasquale et al.; licensee BioMed Central Ltd.
De Pasquale, M. D., De Ioris, M. A., Gallo, A., Mastronuzzi, A., Crocoli, A., Cozza, R., Boldrini, R., Peripheral medulloepithelioma: A rare tumor with a potential target therapy, <<JOURNAL OF TRANSLATIONAL MEDICINE>>, 2014; 12 (1): 1-6. [doi:10.1186/1479-5876-12-49] [https://hdl.handle.net/10807/328041]
Peripheral medulloepithelioma: A rare tumor with a potential target therapy
Mastronuzzi, AngelaWriting – Original Draft Preparation
;
2014
Abstract
Background: Medulloepithelioma (ME) is a rare embryonal tumor predominantly located in the eye or in the central nervous system without an established treatment.Case presentation: We report of a case of a localized peripheral ME treated with conventional and high dose chemotherapy, surgery and local radiotherapy. At relapse, the tumor tissue revealed a different molecular signature compared to the initial tumor mass. This molecular signature revealed a high expression of platelet derived growth factor receptor (PDGFR). Sorafenib plus irinotecan and temozolomide was started with a 5 month progression free survival.Conclusion: Our experience suggests a possible role of sorafenib or different PDGFR inhibitors in ME. Targeting treatment could represent an adjuvant and/or alternative therapy for ME and other rare tumors. © 2014 De Pasquale et al.; licensee BioMed Central Ltd.
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