Intracranial Peripheral Primitive Neuroectodermal Tumor Arising from the Clivus with Intracranial Metastasis in an Elderly Woman: Case Report and Review of the Literature

Gupta, Shruti; Kumar, Arushi; Rangari, Kamlesh; Mehrotra, Anant; Pal, Lily; Kumar, Raj

doi:10.1016/j.wneu.2018.08.066

Cited by 5 publications

(6 citation statements)

References 18 publications

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“…Primary intracranial ES appear as hypointense to heterointense on T1WI and heterogeneously hyperintense on T2WI with diverse patterns of enhancement, which is consistent with our case. Primary intracranial ES is predominantly identified as a dura-based tumor [ 7 ]. This characteristic makes preoperative differential diagnosis from other extra-axial tumors, such as meningioma, hemangiopericytoma, solitary fibrous tumors, and smooth muscle tumors, challenging [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

Primary Intracranial Ewing Sarcoma With an Unusual Presentation: A Case Report

Kim,

Park

et al. 2024

Brain Tumor Res Treat

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Primary extraosseous intracranial Ewing sarcoma (ES) is an extremely rare disease, limited to the pediatric population, that primarily originates in the skull. Here, we present an unusual case of adult Ewing’s sarcoma originating from the brain parenchyma. The 50-year-old male patient visited our hospital with severe headache lasting 3 weeks. MRI presented 6.1×6.2×5.2 cm sized heterogeneously enhanced mass containing peritumoral edema in the right frontal lobe. The patient underwent right frontal craniotomy, at which time the gray and red masses adhered to the surrounding brain parenchyma. The mass was completely resected using neuronavigation and electrophysiological monitoring. Histopathological examination revealed ES-compatible findings of small round cell tumor and CD-99 positive membranous immunostaining. Next generation sequencing revealed translocation and fusion of EWSR1 and FLI1 , consistent with a confirmed diagnosis of ES. Consequently, the patient underwent postoperative radiotherapy. The present case revealed adult primary intracranial ES arising from the frontal lobe. Although its etiology remains poorly understood, intraparenchymal ES should be included in the differential diagnosis of parenchymal brain tumors.

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Section: Discussionmentioning

confidence: 99%

Primary Intracranial Ewing Sarcoma With an Unusual Presentation: A Case Report

Kim,

Park

et al. 2024

Brain Tumor Res Treat

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“… 10 Intracranial Ewing’s sarcomas may be intra- or extraaxial with or without bone involvement and are typically dura-based. 5 , 8 The most common sites of intracranial involvement are convexity, parafalcine, falcine, skull base, and tentorium with nearly 80% of the tumors being supratentorial leaving the posterior fossa as an uncommon location of this malignancy. 1 Ewing’s sarcoma has a reported peak incidence in men between the ages of 5 and 13 years, with one database query finding only 6.3% of tumors discovered in patients over the age of 50.…”

Section: Discussionmentioning

confidence: 99%

“… 15 Given the nonspecific presentation, initial differential diagnosis can be wide including a variety of primary or secondary intracranial tumors such as meningioma, chordoma, lymphoma, eosinophilic granuloma, neuroblastoma or metastasis. 5 , 8 While imaging findings help narrow the differential diagnoses, molecular confirmation of EWS gene rearrangement is required for confirmation of peripheral primitive neuroectodermal tumor (pPNET) as opposed to central primitive neuroectodermal tumor (PNET; e.g. medulloblastoma, pinealoblastoma and supratentorial PNET) that can also present with similar morphological findings.…”

Section: Discussionmentioning

confidence: 99%

“…4,5 Only 1%-2% of reported Ewing's sarcomas are intracranial and are commonly referred to as the tumors belonging to the "small round blue cell" central nervous system tumor group. [6][7][8][9] Less than 60 cases of intracranial Ewing's sarcoma have been reported to date. 10 Intracranial Ewing's sarcomas may be intra-or extraaxial with or without bone involvement and are typically dura-based.…”

Section: Discussion Observationsmentioning

confidence: 99%

“…1 Our English MEDLINE online literature review identified only 13 cases of primary intracranial Ewing's sarcoma in patients of age 50 years or older (Table 1). 5,8,10,[16][17][18][19][20][21][22][23][24] The mean (standard deviation) age of these patients was 59.2 (8.2) years. Male to female ratio was 8:5 (61.6%:38.4%) Of these patients, only 3 (23%) were of age similar to or older than our patient.…”

Section: Discussion Observationsmentioning

confidence: 99%

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Primary intracranial extraosseous Ewing’s sarcoma of the skull base in an elderly adult: illustrative case

Rāviņa¹,

Windermere²,

Zhao³

et al. 2022

Journal of Neurosurgery: Case Lessons

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BACKGROUND Primary extraosseous intracranial Ewing’s sarcoma, also known as a peripheral primitive neuroectodermal tumor or “small round blue cell tumor,” is an extremely rare entity with limited representation in the literature beyond the pediatric population. OBSERVATIONS A 67-year-old male suffering occipital headache, nausea, and gait disturbance was found to have a large, avidly contrast-enhancing cerebellopontine angle mass extending into the cervical spinal canal with associated mass effect on medulla, cerebellum, fourth ventricle, and cervical spinal cord. This mass was not present on the imaging from 8 years prior. He underwent surgical debulking and pathology results demonstrated a malignant small round cell tumor showing diffuse immunopositivity for cytokeratins, CD99 and NKX2.2 with EWRS1-FLI1 rearrangement in 84% of the nuclei confirmatory of Ewing’s sarcoma. After 14 cycles of chemotherapy and 6 weeks of radiotherapy, 22 months after discovery, the patient remains in clinical and radiographic remission with complete return to his baseline functioning. LESSONS Primary skull base extraosseous Ewing’s sarcoma should be considered in the differential diagnosis even in the elderly population when imaging studies demonstrate aggressive tumor growth patterns. Tumor debulking to establish a diagnosis followed by adjuvant chemoradiation therapy can result in clinical improvement with remission.

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