Intradural extramedullary Ewing's sarcoma: A case report and review of the literature

Paterakis, Konstantinos; Brotis, Alexandros; Τάσιου, Αναστασία; Kotoula, V.; Kapsalaki, Eftychia; Vlychou, Marianna

doi:10.1016/j.pjnns.2016.11.006

Cited by 17 publications

(12 citation statements)

References 22 publications

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“…Of the cases we reviewed, 4 patients presented with or developed diffuse leptomeningeal disease, 10–13 5 patients were found to have multiple lesions on initial presentation, 10 , 14–17 and an additional 4 patients developed new discrete lesions later in the course of the disease. 17–20 …”

Section: Illustrative Casementioning

confidence: 99%

Multifocal primary central nervous system Ewing sarcoma presenting with intracranial hemorrhage and leptomeningeal dissemination: illustrative case

Huguenard¹,

Li²,

Sharifai

et al. 2021

Journal of Neurosurgery: Case Lessons

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BACKGROUND Ewing sarcoma is a neoplasm within the family of small round blue cell tumors and most frequently arises from skeletal bone. Primary involvement of the central nervous system in these lesions is extremely rare, with an incidence of 1%. OBSERVATIONS A case is presented of a 34-year-old man who presented with left facial numbness, multiple intracranial lesions, a lumbar intradural lesion, and diffuse spinal leptomeningeal involvement. A lumbar laminectomy and biopsy were performed, which revealed the diagnosis of extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. The patient had a rapidly progressive clinical decline despite total neuroaxis radiation and multiple lines of chemotherapeutic treatments, eventually dying from his disease and its sequelae 6 months after diagnosis. LESSONS The authors’ review of 40 cases in the literature revealed only 2 patients with isolated intraaxial cranial lesions, 4 patients with cranial and spine involvement, and an additional 34 patients with spine lesions. The unique characteristics of this patient’s case, including his presentation with diffuse disease and pathology that included a rare V600E BRAF mutation, are discussed in the context of the available literature.

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Section: Illustrative Casementioning

confidence: 99%

Multifocal primary central nervous system Ewing sarcoma presenting with intracranial hemorrhage and leptomeningeal dissemination: illustrative case

Huguenard¹,

Li²,

Sharifai

et al. 2021

Journal of Neurosurgery: Case Lessons

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“…On imaging, five cases had a lumbar intradural-extramedullary enhancing lesion and one case had a lumbar enhancing epidural lesion. One case has nerve root evasion [ 11 ], and one case had a mass effect on cauda-equina [ 9 ]. Only one case had CSF analysis which showed predominant red-blood cells, elevated corrected white blood cells, and protein in the setting of choroid plexus papilloma [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…To delineate the clinical presentation from lumbar dural metastasis a systemic review was conducted. The literature review was performed in PubMed including English literature till December 2022 with the search term "Lumbar," "Dural," "metastases" which yielded six case reports [6][7][8][9][10][11]. Cases with cervical and thoracic metastasis were excluded.…”

Section: Discussionmentioning

confidence: 99%

The Unique Case of Lumbar Dural Metastasis From Cervical Carcinoma on Pembrolizumab

Bhagat¹

2022

Cureus

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Lumbar metastasis is a rare manifestation of cervical carcinoma which may present as lower extremities symptoms. Immune-checkpoint inhibitors like pembrolizumab, used for the treatment of various metastatic carcinomas, have been linked to Guillain-Barré Syndrome (GBS), which is characterized by ascending weakness and areflexia. A 52-year-old female with metastatic cervical cancer on pembrolizumab for two months presents with lower back pain, progressive bilateral lower extremities ascending weakness, and areflexia of affected limbs. Cerebrospinal fluid analysis showed albuminocytologic disassociation. Spine magnetic resonance imaging showed L4-L5 fracture associated with metastasis and contrast-enhancing adjacent dura and nerve roots. Despite the clinical presentation being consistent with lumbar metastasis, the possibility of masked pembrolizumab-induced GBS presented a therapeutic challenge. Plasmapheresis conferred no clinical improvement and later nerve conduction study was unrevealing for GBS. Subsequently, laminectomy of lumbar vertebrae improved the symptoms. The unique clinical scenario of lumbar dural metastasis from cervical cancer on pembrolizumab, a condition with a similar clinical presentation, is explored in this case.

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“…As is well documented in the tumor literature, this primary malignancy is radiosensitive and incomplete surgical excision is adequate given the patient's improvement in symptoms and lesion size following a course of RT. The use of chemotherapeutics has also been employed in treatment strategies for intradural metastatic lesions [29]. Again, primary malignancy etiology and possible genetic variations are likely to play a role in whether chemotherapy is effective or not-we advocate early involvement of a multidisciplinary team including oncologic specialists in all cases of IDEM metastatic disease given its late presentation.…”

Section: Adjuvant Therapymentioning

confidence: 99%

Intradural extramedullary metastasis: a review of literature and case report

Land

Bowden

Morpeth

et al. 2019

Spinal Cord Ser Cases

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Introduction Intradural extramedullary (IDEM) metastatic disease is infrequently encountered by spine surgeons and consequently poorly understood. Discovery often corresponds with the onset of neurologic symptoms and no consensus exists regarding the importance of complete resection or anticipated postoperative outcome. We aim to elucidate treatment methodologies that exist in the literature. Case presentation We present a unique case of a 57-year-old male with a known history of esophageal adenocarcinoma, including brain and visceral metastases, who presented with cauda equina syndrome. An IDEM metastatic esophageal adenocarcinoma lesion was identified on advanced imaging and biopsy. This was treated operatively without return of neurologic function. Discussion We reviewed and summarized the existing literature. Trends are highlighted to further guide surgeons treating this unusual metastatic phenomenon. Conclusion Intradural metastasis is a harbinger of advanced disease with a poor prognosis regardless of the etiology of the primary lesion. There are a number of proposed mechanisms for metastatic spread with little available literature for surgeon guidance. Most authors are advocates of a palliative, decompressive approach.

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Intradural extramedullary Ewing's sarcoma: A case report and review of the literature

Cited by 17 publications

References 22 publications

Multifocal primary central nervous system Ewing sarcoma presenting with intracranial hemorrhage and leptomeningeal dissemination: illustrative case

Multifocal primary central nervous system Ewing sarcoma presenting with intracranial hemorrhage and leptomeningeal dissemination: illustrative case

The Unique Case of Lumbar Dural Metastasis From Cervical Carcinoma on Pembrolizumab

Intradural extramedullary metastasis: a review of literature and case report

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