First Case of Primary Sellar/Suprasellar-Intraventricular Ewing Sarcoma: Case Report and Review of the Literature.
World Neurosurg
; 98: 869.e1-869.e5, 2017 Feb.
Article
em En
| MEDLINE
| ID: mdl-28017744
ABSTRACT
BACKGROUND:
Intracranial Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare and poorly differentiated neoplasms. Immunohistochemical and cytogenetic findings support the possibility of a unique nosologic entity. Primary intracranial localization of this tumor is extremely rare; a few cases are reported in the literature, with only some confirmed by genetic studies. CASE DESCRIPTION We report a 12-year-old patient with a sellar/suprasellar mass with intraventricular extension that in all its features mimicked a transinfundibular craniopharyngioma. The patient underwent complete resection of the lesion via an endoscopic endonasal transtuberculum approach 6 days after ventriculoperitoneal shunt for acute obstructive hydrocephalus. Histopathologic and genetic examination demonstrated ES/pPNET. The diagnosis was confirmed by detection of a rearrangement of the EWSR1 gene by fluorescent in situ hybridization and identification of the diagnostic t(11;22) translocation by reverse transcriptase polymerase chain reaction. The patient remained in complete clinical remission 12 months after tumor resection followed by adjuvant chemotherapy with no radiologic evidence of tumor recurrence.CONCLUSIONS:
To our knowledge, this is the first case of primary intrasellar/suprasellar-intraventricular ES/pPNET confirmed by molecular genetic analysis. Extensive investigations, including pathologic, immunohistochemical, and genetic studies, are needed for differentiation of these tumors from other, more common sellar/suprasellar tumors. Our case highlights that an interdisciplinary therapeutic approach is mandatory to guarantee a favorable outcome.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sarcoma de Ewing
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Neoplasias Cranianas
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Neoplasias Encefálicas
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Tumores Neuroectodérmicos Primitivos
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Cirurgia Endoscópica por Orifício Natural
Tipo de estudo:
Prognostic_studies
Limite:
Child
/
Humans
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article