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Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the upper eyelid in an adult patient

Rubino, Corrado and Mulas, Pietro and Contu, Antonio and Farace, Francesco and Tanda, Francesco and Bella, Michelangelo and Pazzola, Antonio (2014) Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the upper eyelid in an adult patient. European Journal of Plastic Surgery, Vol. 37 (10), p. 559-562. ISSN 0930-343X. eISSN 1435-0130. Article.

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DOI: 10.1007/s00238-014-0979-8

Abstract

Peripheral primitive neuroectodermal tumor (PPNET) and Ewing’s sarcoma (ES) are two variants of a highly malignant bone tumor usually affecting children and young adults with a tendency for early metastasis. Cutaneous localization of PPNET/ES is very uncommon in adult age and is normally confused with other tumors. We report the first recorded case of PPNET of the eyelid in an adult patient. The patient underwent one biopsy, two surgical wide excisions, and two series of 4 cycles of chemotherapy without radiotherapy. At 8 years of follow-up from the first wide excision, the patient is tumor free. Cutaneous PPNET/ES is rare and not considered in the usual differential diagnosis of skin lesions, especially those involving the eyelid. Once diagnosed, a multidisciplinary approach to treatment offers the highest survival rates.

Item Type:Article
ID Code:10173
Status:Published
Refereed:Yes
Uncontrolled Keywords:PPNET, peripheral primitive neuroectodermal tumor, Ewing’s sarcoma, eyelid, adult
Subjects:Area 06 - Scienze mediche > MED/19 Chirurgia plastica
Area 06 - Scienze mediche > MED/08 Anatomia patologica
Divisions:001 Università di Sassari > 01-a Nuovi Dipartimenti dal 2012 > Scienze Chirurgiche, Microchirurgiche e Mediche
002 Altri enti e centri di ricerca del Nord Sardegna > Azienda ASL1, Sassari > Ospedale civile SS. Annunziata > Ambulatorio oncologia medica
Publisher:Springer Berlin/Heidelberg
ISSN:0930-343X
eISSN:1435-0130
Deposited On:13 Oct 2014 15:24

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