First Case of Primary Sellar/Suprasellar-Intraventricular Ewing Sarcoma: Case Report and Review of the Literature
| Autor: | Antonio Romano, Corrado Iaccarino, Davide Nasi, Pier Paolo Mattogno, Luisa Santoro, Gabriele Oretti |
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| Rok vydání: | 2016 |
| Předmět: |
Natural Orifice Endoscopic Surgery
Pathology medicine.medical_specialty Adjuvant chemotherapy Skull Neoplasms Sarcoma Ewing Sellar region tumors Lesion 03 medical and health sciences Therapeutic approach 0302 clinical medicine medicine Humans Neuroectodermal Tumors Primitive Child Peripheral Primitive Neuroectodermal Tumor business.industry Brain Neoplasms Peripheral primitive neuroectodermal tumors medicine.disease Endoscopic endonasal transtuberculum approach Ewing sarcoma Magnetic Resonance Imaging Craniopharyngioma Molecular analysis Treatment Outcome 030220 oncology & carcinogenesis Immunohistochemistry Surgery Neurology (clinical) Sarcoma medicine.symptom business Perforator Flap 030217 neurology & neurosurgery |
| Zdroj: | World neurosurgery. 98 |
| ISSN: | 1878-8769 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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