First Case of Primary Sellar/Suprasellar-Intraventricular Ewing Sarcoma: Case Report and Review of the Literature

被引:11
作者
Mattogno, Pier Paolo [1 ]
Nasi, Davide [2 ]
Iaccarino, Corrado [2 ,3 ]
Oretti, Gabriele [4 ]
Santoro, Luisa [5 ]
Romano, Antonio [2 ,3 ]
机构
[1] Inst Neurosurg, Cathol Sch Med, Rome, Italy
[2] Inst Sci & Care Res ASMN Reggio Emilia, Dept Neurosurg, Reggio Emilia, Italy
[3] Univ Hosp Parma, Neurosurg Neurotraumatol Unit, Parma, Italy
[4] Univ Hosp Parma, Otolaryngol Unit, Parma, Italy
[5] Univ Hosp Padova, Inst Pathol, Padua, Italy
关键词
Endoscopic endonasal transtuberculum approach; Ewing sarcoma; Peripheral primitive neuroectodermal tumors; Sellar region tumors; PRIMITIVE NEUROECTODERMAL TUMOR; PERIPHERAL PNET/EWINGS SARCOMA; VAULT; DURA;
D O I
10.1016/j.wneu.2016.12.045
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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页码:869.e1 / 869.e5
页数:5
相关论文
共 28 条
[1]   Intracranial Ewings Sarcoma/peripheral primitive neuroectodermal tumor [J].
Amita, R. ;
Sandhyamani, S. ;
Nair, Suresh ;
Kapilamoorthy, T. R. .
NEUROLOGY INDIA, 2014, 62 (04) :426-427
[2]   Primary peripheral PNET/Ewing's sarcoma arising in the meninges, confirmed by the presence of the rare translocation t(21;22) (q22;q12) [J].
Antonelli, Manila ;
Caltabiano, Rosario ;
Chiappetta, Caterina ;
Oliva, Maria A. ;
Giangaspero, Felice ;
Lanzafame, Salvatore .
NEUROPATHOLOGY, 2011, 31 (05) :549-555
[3]   Intracranial Ewing sarcoma/'peripheral' primitive neuroectodermal tumor of dural origin with molecular genetic confirmation [J].
Antunes, NL ;
Lellouch-Tubiana, A ;
Kalifa, C ;
Delattre, O ;
Pierre-Kahn, A ;
Rosenblum, MK .
JOURNAL OF NEURO-ONCOLOGY, 2001, 51 (01) :51-56
[4]  
Attabib Najmedden A, 2006, Neurosurgery, V58, pE992, DOI 10.1227/01.NEU.0000210215.73374.EE
[5]  
Bano Shahina, 2009, Indian J Radiol Imaging, V19, P305, DOI 10.4103/0971-3026.57215
[6]   Primary peripheral PNET/Ewing's sarcoma of the dura with FISH analysis [J].
D'Antonio, A ;
Caleo, A ;
Garcia, JF ;
Marsilia, GM ;
De Dominicis, G ;
Boscaino, A .
HISTOPATHOLOGY, 2004, 45 (06) :651-654
[7]   EWS-FL11 fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma [J].
de Alava, E ;
Kawai, A ;
Healey, JH ;
Fligman, I ;
Meyers, PA ;
Huvos, AG ;
Gerald, WL ;
Jhanwar, SC ;
Argani, P ;
Antonescu, CR ;
Pardo-Mindan, FJ ;
Ginsberg, J ;
Womer, R ;
Lawlor, ER ;
Wunder, J ;
Andrulis, I ;
Sorensen, PHB ;
Barr, FG ;
Ladanyi, M .
JOURNAL OF CLINICAL ONCOLOGY, 1998, 16 (04) :1248-1255
[8]   Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET [J].
Dedeurwaerdere, F ;
Giannini, C ;
Sciot, R ;
Rubin, BP ;
Perilongo, G ;
Borghi, L ;
Ballotta, ML ;
Cornips, E ;
Demunter, A ;
Maes, B ;
Tos, APD .
MODERN PATHOLOGY, 2002, 15 (06) :673-678
[9]  
Dehner LP, 1993, J SURG PATHOL, V17, P1
[10]  
Folpe AL, 2005, AM J SURG PATHOL, V29, P1025