First case of primary sellar/suprasellar intraventricular Ewing Sarcoma: case report and review of literature.

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First case of primary sellar/suprasellar intraventricular Ewing Sarcoma: case report and review of literature.

World Neurosurg. 2016 Dec 22;:

Authors: Mattogno PP, Nasi D, Iaccarino C, Oretti G, Santoro L, Romano A

Abstract
BACKGROUND: Intracranial Ewing Sarcoma (ES) and peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare and poorly differentiated neoplasms. Recent immunohistochemical and cytogenetic findings support the possibility of a unique nosological entity. Primary intracranial localization of this tumor is extremely rare: a small number of cases are reported in the literature, with only a part of them confirmed by genetic studies.
CASE DESCRIPTION: We report the case of 12 year-old-patient affected by sellar-suprasellar mass with intraventricular extension that in all its features mimes a transinfundibular craniopharyngioma. The patient underwent to endoscopic endonasal transtuberculum approach with complete resection of the lesion six days after ventriculo-peritoneal shunt for acute obstructive hydrocephalus. Histopathological and genetic examinations resulted for ES/pPNET. In fact, the diagnosis was confirmed by detection of a rearrangement of the EWSR1 gene by fluorescent in situ hybridization (FISH) and identification of the diagnostic t(11;22) translocation by reverse transcription-polymerase chain reaction RT-PCR. Twelve months after tumor resection followed by adjuvant chemotherapy, the patient remained in complete clinical remission with no radiological evidence of tumor recurrence. This case was illustrated by surgical video.
CONCLUSIONS: To our knowledge, this is the first case of primary intra/suprasellar intraventricular ES/pPNET, confirmed by molecular genetic analysis. Extensive investigations, including pathological, immunohistochemical and genetic studies, are needed for the differentiation of these tumors from other more common sellar/suprasellar tumors. Finally, our case highlights that an interdisciplinary therapeutic approach is mandatory to guarantee a favorable outcome.

PMID: 28017744 [PubMed - as supplied by publisher]

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