Clinical Features and Long-Term Outcome of Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors: 14 Cases From a Single Institution

Jiang, Qian; Zhang, Yu; Yu, Yufei; Zheng, Yifeng; Chen, Juan; Zhao, Yiqing; Zhang, Miao; Fan, Fanfan; Wang, Yu

doi:10.1016/j.wneu.2018.11.151

Cited by 31 publications

(80 citation statements)

References 20 publications

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“…Intracranial ES/pPNETs are very rare malignant tumors, accounting for 0.03% of the total number of intracranial tumors ( 3 , 4 ), with only 57 cases reported until now. ES/pPNETs mostly occur in children and adolescents with a median age at first onset of disease being 15 years of age, and the peak of disease is prominent in the second decade, ranging from 5 months to 67 years of age, with a slight male predisposition ( 14 , 16 – 18 ).…”

Section: Discussionmentioning

confidence: 99%

“…We extensively reviewed previous reports and found that primary intracranial ES/pPNETs mostly showed mixed isointense-to-hypointense signals on T1WI, and isointense-to-hyperintense signals on T2WI ( 4 ). Our case was consistent with these findings.…”

Section: Discussionmentioning

confidence: 99%

“…Primary intracranial ES/pPNETs are a group of poorly differentiated, highly malignant, and aggressive small round cell neoplasms, generally originating from bone and soft tissue, which are common among children and adolescents ( 1 – 4 ). ES/pPNETs that occur intracranially are extremely rare, with only 57 cases reported so far.…”

Section: Introductionmentioning

confidence: 99%

“…ES/pPNETs that occur intracranially are extremely rare, with only 57 cases reported so far. For patients over 30 years of age, only 9 cases have been reported ( 4 – 12 ). Fusion of the EWSR1 gene with a member of the ETS gene family is thought to be the primary cause of the ES/pPNETs ( 3 , 4 , 13 – 15 ).…”

Section: Introductionmentioning

confidence: 99%

“…For patients over 30 years of age, only 9 cases have been reported ( 4 – 12 ). Fusion of the EWSR1 gene with a member of the ETS gene family is thought to be the primary cause of the ES/pPNETs ( 3 , 4 , 13 – 15 ). Herein, we present a case of ES/pPNET located in the left frontal lobe of a 55-year-old female, who was tested through histological examination, immunohistochemical analysis and fluorescence in situ hybridization (FISH).…”

Section: Introductionmentioning

confidence: 99%

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Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review

et al. 2020

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Background: Primary intracranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies, which arise in children and adolescents, with only 9 cases reported in patients over 30 years of age. Due to its rarity, MRI features and treatment strategies for primary intracranial ES/pPNETs remain unclear. The purpose of this study was to explore the clinical features, imaging findings, pathological characteristics, different diagnoses, treatment, and prognosis of cerebellar liponeurocytoma in adults. Case Description: A 55-year-old female was admitted to the hospital with memory decline over 1 month, which aggravated in the last 2 weeks. MRI showed a 4.3 × 6.5 × 3.5 cm heterogeneous large mass in the left frontal lobe with mild peritumoral edema. The mass was successfully removed under neuronavigation and electrophysiological monitoring. The entire mass was removed, and postoperative pathology indicated an ES pPNET diagnosis, with an EWSR1 gene rearrangement. Subsequently, the patient underwent disciplinary radiotherapy. Conclusion: The diagnosis of primary intracranial ES/pPNETs depends on the comprehensive consideration of histological examination, immunohistochemical analysis, and genetic detection. Gross tumor resection combined with radiotherapy and chemotherapy might be the most beneficial treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review

et al. 2020

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Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: A rare case report

Ghaemi

Rajabi-Moghaddam

Abbaszadeh

2022

Clinical Case Reports

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Primary intracranial peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies that commonly affect children and adolescents. Only 10 cases over the age of 33 have been reported. pPNETs have an aggressive behavior and a high tendency for local recurrence and distant metastasis. Here, we present a case of supratentorial pPNET that affected the left frontoparietal lobe of a 36‐year‐old female patient. The patient complained of aphasia during the last 2 months. Aphasia is reported for the first time as a result of a pPNET. In T1‐weighted MRI, a large mass with mixed isointense to hypointense signals was observed. The tumor was completely removed. Histopathologic examination was indicative of a small round cell tumor. Immunohistochemical analysis showed positivity for CD99. Presence of EWSR1 gene rearrangement confirmed the diagnosis. The patient's aphasia was gradually resolved post‐surgery. Six months follow‐up showed no evidence of local recurrence or metastasis.

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Sarcoma and the nervous system

Dixit

Wolinsky

Kumthekar

et al. 2022

Neurological Complications of Systemic Cancer and Antineoplastic Therapy

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Clinical Features and Long-Term Outcome of Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors: 14 Cases From a Single Institution

Cited by 31 publications

References 20 publications

Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review

Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review

Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: A rare case report

Sarcoma and the nervous system

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