Peripheral medulloepithelioma

A rare tumor with a potential target therapy

Research output: Contribution to journalArticle

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 languageEnglish
Article number49
JournalJournal of Translational Medicine
Volume12
Issue number1
DOIs
Publication statusPublished - Feb 21 2014

Fingerprint

Primitive Neuroectodermal Tumors
Tumors
Platelet-Derived Growth Factor Receptors
irinotecan
temozolomide
Neoplasms
Chemotherapy
Radiotherapy
Neurology
Therapeutics
Complementary Therapies
Surgery
Disease-Free Survival
Dosimetry
Central Nervous System
Tissue
Recurrence
Drug Therapy
sorafenib

Keywords

  • PDGF
  • Peripheral medulloepithelioma
  • Target therapy
  • Treatment

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Medicine(all)

Cite this

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title = "Peripheral medulloepithelioma: A rare tumor with a potential target therapy",
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.",
keywords = "PDGF, Peripheral medulloepithelioma, Target therapy, Treatment",
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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.

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KW - Treatment

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