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.
| Original language | English |
|---|---|
| Article number | 49 |
| Journal | Journal of Translational Medicine |
| Volume | 12 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - Feb 21 2014 |
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Keywords
- PDGF
- Peripheral medulloepithelioma
- Target therapy
- Treatment
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)
- Medicine(all)
Cite this
Peripheral medulloepithelioma : A rare tumor with a potential target therapy. / De Pasquale, Maria D.; De Ioris, Maria A.; Gallo, Angela; Mastronuzzi, Angela; Crocoli, Alessandro; Cozza, Raffaele; Boldrini, Renata.
In: Journal of Translational Medicine, Vol. 12, No. 1, 49, 21.02.2014.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Peripheral medulloepithelioma
T2 - A rare tumor with a potential target therapy
AU - De Pasquale, Maria D.
AU - De Ioris, Maria A.
AU - Gallo, Angela
AU - Mastronuzzi, Angela
AU - Crocoli, Alessandro
AU - Cozza, Raffaele
AU - Boldrini, Renata
PY - 2014/2/21
Y1 - 2014/2/21
N2 - 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.
AB - 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.
KW - PDGF
KW - Peripheral medulloepithelioma
KW - Target therapy
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=84904243400&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84904243400&partnerID=8YFLogxK
U2 - 10.1186/1479-5876-12-49
DO - 10.1186/1479-5876-12-49
M3 - Article
VL - 12
JO - Journal of Translational Medicine
JF - Journal of Translational Medicine
SN - 1479-5876
IS - 1
M1 - 49
ER -