Primary intracranial extraosseous Ewing’s sarcoma

Sohail, Amir; Sachal, Mohammed; Maan, Muhammad Arslan Arif; Soban, Muhammad; Khan, Muhammad Salman; Bari, Muhammad Ehsan

doi:10.1007/s00381-018-3991-7

Cited by 6 publications

(3 citation statements)

References 15 publications

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“…Because pPNET has a meningeal origin and displays a dural base on brain MRI, 18 31% of cases mimicked the presence of a meningioma or hemangiopericytoma. 7 , 18 , 27 , 28 , 30 This finding resonates with our clinical scenario, given that an absent acknowledgment of pPNET in the differential diagnosis can impact the precision of treatment and the patient outcome.…”

Section: Discussionsupporting

confidence: 82%

“…We performed a MEDLINE review of articles reporting durabased pPNET in adults over the last decade (Table 1). 2,7,18,19,[23][24][25][26][27][28][29][30][31][32][33] Approximately 16 patients (50% female), with a median age of 33 years (range 18-56 years), were diagnosed with pPNET and followed up for a median of 12 months (range 0.2-60 months). Frequent presenting symptoms included headaches, vomiting, vision impairment, and neurological deficits.…”

Section: Discussionmentioning

confidence: 99%

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Primary intracranial peripheral primitive neuroectodermal tumor: lessons from an exceptionally rare neoplasm. Illustrative case

Bocanegra-Becerra,

Novoa-Ramírez,

Latorre-Zúñiga

et al. 2024

Journal of Neurosurgery: Case Lessons

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BACKGROUND The primary intracranial peripheral primitive neuroectodermal tumor (pPNET) is a lesion subtype within the Ewing sarcoma family of tumors. pPNETs are extremely uncommon pathologies, accounting for 0.03% of intracranial tumors and 1% to 2% of Ewing sarcoma cases. Given its histological aspect similar to other highly proliferative malignant neuroectodermal neoplasms, pPNET merits extensive workup for accurate diagnosis and treatment. OBSERVATIONS A 36-year-old male presented to the emergency department with a 1-year history of headaches in the right frontoparietal area, generalized tonic-clonic seizures, and a history of the resection of a tumor labeled as a meningioma 5 years before admission. He was neurologically intact. Brain magnetic resonance imaging revealed a heterogeneous focal lesion of 25 × 35 × 23 mm with a necrotic center and neoformative appearance in the right frontal cortex. The patient underwent multimodal treatment with gross-total resection, radiotherapy, and chemotherapy. Histopathological examination results supported the diagnosis of pPNET. At the 2-year follow-up, the patient had no new-onset symptoms, and brain imaging revealed absent signs of tumor recurrence. LESSONS The present case describes an extraordinary pPNET case, initially confounded as a clear cell meningioma. Managing pPNET requires thorough investigation, careful differentiation from similar neuroectodermal lesions, and multimodal treatment to improve the patient’s prognosis.

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Section: Discussionsupporting

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Primary intracranial peripheral primitive neuroectodermal tumor: lessons from an exceptionally rare neoplasm. Illustrative case

Bocanegra-Becerra,

Novoa-Ramírez,

Latorre-Zúñiga

et al. 2024

Journal of Neurosurgery: Case Lessons

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“…Primary intracranial Ewing sarcoma (ES)/Primitive neuroectodermal tumour (pPNET) is malignant small round cell neoplasms, that arise from bone and soft tissue, commonly seen in children and adolescents [1,2]. Primary intracranial extraosseous ES is extremely rare, and only a few cases have been described [3][4][5][6][7]. Diagnosis of primary intracranial ES is made by the inclusion of radiological findings, histological examinations, and immunohistochemical and molecular genetic analysis.…”

Section: Introductionmentioning

confidence: 99%

Posterior fossa primary intracranial extraosseous Ewing’s sarcoma: case report

Shah,

Charan,

Agarwal

et al. 2024

Egypt J Radiol Nucl Med

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Background Primary intracranial Ewing’s sarcoma (ES) is a type of primitive neuroectodermal tumour and is a rare malignant tumour in children and adolescents. The imaging features of ES overlap with other central nervous system embryonal tumours, making it difficult to pinpoint a specific diagnosis. We aim to explore the clinical, neuroimaging and differential diagnoses of this entity. Case presentation We describe a 6-month-old infant who presented with complaints of enlarging the head size and poor feeding. Imaging revealed a contrast-enhancing large solid-cystic mass lesion with internal calcification, focal bone erosion and haemorrhage in the posterior fossa. Histopathological examinations, immunohistochemistry, and molecular analysis confirmed ES. Conclusions The confirmative diagnosis of primary intracranial ES requires histological examination, immunohistochemical analysis, and genetic detection, along with radiological findings. Surgical excision followed by combined radiotherapy and chemotherapy is the treatment of choice.

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