Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature

Scantland, Joshua; Gondim, Mercia; Koivuniemi, Andrew S.; Fulkerson, Daniel H.; Shih, Chie‐Schin

doi:10.1159/000488767

Cited by 12 publications

(12 citation statements)

References 22 publications

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“…4 Thus, primary spinal Intradural Extramedullary Extraosseous ES (IEEES) is extremely rare. 4–12 We, here, report an unusual, yet the interesting presentation of Primary Intradural Extramedullary ES/pPNET with extradural extension in the lumbar spine.…”

Section: Introductionmentioning

confidence: 81%

Primary Spinal Intradural Extramedullary Ewing’s Sarcoma/Peripheral Neuroectodermal Tumour Masquerading Clinically as a Neurogenic Tumour: A Case Report and Review of Literature

Praveen,

Das,

Gupta

et al. 2023

Annals of Neurosciences

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Ewing’s Sarcomas (ES)/Peripheral neuroectodermal tumour (pPNET) are heterogenous group of rare, highly malignant, undifferentiated primitive round-cell neoplasms of neuroectodermal origin. pPNETs are seldom observed to involve the spine of which Spinal Intradural Extramedullary Extraosseous Primary ES/pPNET are extremely rare. We report a case of a 23-year-old male with complaints of low backache and hip pain radiating to the left inguinal region for four months. Radiology findings were suggestive of a neurogenic tumour. Cytomorphology, histomorphology and immunohistochemistry evaluation were done. Diagnosis was consistent with ES/pPNET. Careful correlation between clinical history, cytomorphology, histopathology, immunohistochemical and molecular analysis can help to distinguish primary spinal ES/PNET from other primary spinal tumours and will help clinicians to start treatment at the earliest.

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Section: Introductionmentioning

confidence: 81%

Primary Spinal Intradural Extramedullary Ewing’s Sarcoma/Peripheral Neuroectodermal Tumour Masquerading Clinically as a Neurogenic Tumour: A Case Report and Review of Literature

Praveen,

Das,

Gupta

et al. 2023

Annals of Neurosciences

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“…6 This is only the 6th documented case of adolescent intradural extramedullary Ewing sarcoma in the published literature. 8 As such, not only was it rare for the patient to have an underlying sinister pathology, but the pathology itself was in addition extremely rare. As the patient did not respond to conservative treatment or analgesics, the patient was referred to secondary care diagnostic imaging under the assumption that the imaging findings would change the management of her condition, which reflects current guideline recommendations.…”

Section: Discussionmentioning

confidence: 99%

Intradural Extramedullary Ewing Sarcoma in an Adolescent Female

Kunwald¹,

Straszek²,

Bjarkam³

et al. 2022

AHMT

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A 15-year-old female experiencing lumbar pain without prior trauma. Despite being prescribed strong analgesics by her family physician, the symptoms intensified, and she was referred to diagnostic imaging. Magnetic resonance imaging revealed an intradural extramedullary tumor in the spinal canal, located behind the L3 corpora. The patient underwent neurosurgery with a complete resection of the tumor. The histopathologic examination revealed Ewing sarcoma by identifying EWSR1 gene in the extracted tissue. The patient was subsequently referred for extensive specialized oncological treatment including photon irradiation therapy and chemotherapy. Thirty-six months on from the initial diagnosis, the patient is doing well and suffers no sequelae apart from hypogonadism.

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“…7,8 Other neoplasms, such as melanotic schwannomas and extraosseous Ewing sarcoma, cannot be excluded because they can seem hyperintense on T1-weighted images and hypointense on T2-weighted images. 7,9 Confirmatory diagnosis requires a biopsy.…”

Section: Sectionmentioning

confidence: 99%

Clinical Reasoning: Longitudinally Extensive Spinal Cord Lesions in a Middle-aged Man

Xie

Zhou

2022

Neurology

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An immunocompetent 47-year-old man presented with a five-month history of progressive lower limb weakness, back pain, sphincter dysfunction, and intermittent fever, suggesting myelopathy in a chronic deteriorating course. A comprehensive analysis comprising of blood tests, neuroimaging, CSF profiling, molecular analysis, and histopathology was performed. Notably, enhanced spinal cord MRI revealed longitudinally extensive intradural-extramedullary lesions involving the cervical, thoracic, and lumbosacral spinal cord, with homogeneous enhancement and spinal cord compression. Serum TPHA and RPR tests were positive. CSF profiling showed pleocytosis, significant protein elevations, hypoglycorrhachia, and positive TPHA test. 18F-FDG-PET/CT indicated slightly increased intraspinal FDG uptake. Spinal cord biopsy further showed small round blue cells in poorly differentiated tissues. Immunostaining was positive for NKX2.2, CD56, CD99, Synaptophysin, and Ki67 (50%). Molecular analysis detected a novel MALAT-CYSLTR1 fusion protein and variants in oncogenic genes including PTCH1, TERT, CREBBP, SPEN, and STK11. The diagnosis of intraspinal extraosseous Ewing’s sarcoma (ES) was confirmed. Briefly, our case details the diagnosis of a patient with intradural-extramedullary ES and highlights the value of spinal cord biopsy in progressive myelopathy of unknown causes.

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Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature

Cited by 12 publications

References 22 publications

Primary Spinal Intradural Extramedullary Ewing’s Sarcoma/Peripheral Neuroectodermal Tumour Masquerading Clinically as a Neurogenic Tumour: A Case Report and Review of Literature

Primary Spinal Intradural Extramedullary Ewing’s Sarcoma/Peripheral Neuroectodermal Tumour Masquerading Clinically as a Neurogenic Tumour: A Case Report and Review of Literature

Intradural Extramedullary Ewing Sarcoma in an Adolescent Female

Clinical Reasoning: Longitudinally Extensive Spinal Cord Lesions in a Middle-aged Man

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