Multiple intradural-extramedullary spinal ependymomas including tumors with different histological features

Honda, Akira; Iizuka, Yoichi; Hirato, Junko; Kiyohara, Hiroki; Iizuka, Haku

doi:10.1007/s00586-017-5055-1

Cited by 10 publications

(7 citation statements)

References 11 publications

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“…Although IDEM ependymomas are typically benign, they have been reported to recur, undergo anaplastic transformation, and seed in the CSF. 3 , 4 , 6 , 9 , 12 , 21 Because IDEM ependymomas are usually solitary and isolated, complete resection is frequently performed and offers the best prognosis for a full recovery without recurrence. Adjuvant radiotherapy and chemotherapy remain controversial.…”

Section: Discussionmentioning

confidence: 99%

“…However, the former has been shown to significantly improve overall survival, and the latter is usually reserved for salvage therapy if both surgery and radiotherapy fail. 4 , 6 , 14 , 21 …”

Section: Discussionmentioning

confidence: 99%

“…Only nine cases of multifocal IDEM ependymomas have been reported in the literature ( Table 1 ). 1–4 , 6 , 7 , 13 , 14 The mean age was 38 years (range, 26 to 53 years), and five (56%) patients were female. All patients had either neck and/or low back pain, paresthesia of the lower extremities, and/or gait abnormalities.…”

Section: Discussionmentioning

confidence: 99%

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Multifocal intradural extramedullary ependymoma, MYCN amplified: illustrative case

Shields

Sun

Highfield³

et al. 2022

Journal of Neurosurgery: Case Lessons

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BACKGROUND Ependymomas are the most frequent tumors of the adult spinal cord, representing 1.9% of all central nervous system tumors and 60% of spinal cord tumors. Spinal ependymomas are usually solitary, intramedullary lesions. While intradural extramedullary (IDEM) ependymomas are infrequent, multifocal IDEM ependymomas are exceptionally rare. OBSERVATIONS The authors reported the first case in the literature of a patient diagnosed with multifocal IDEM ependymomas who was treated with tumor resection and brain and spinal radiotherapy. The patient presented with a 10-day history of bilateral leg numbness extending to the umbilicus and gait instability. Magnetic resonance imaging (MRI) studies revealed multiple enhancing nodular nodules throughout the entire spinal canal. Brain MRI revealed no abnormal lesions. A World Health Organization grade II ependymoma was confirmed histologically. At 31 months postoperatively, the patient remained clinically asymptomatic. Although cervical and thoracic MRI revealed stable intradural nodules and several areas of leptomeningeal enhancement, no malignant cells were seen in the cerebrospinal fluid (CSF). He underwent genetic testing to determine the appropriate chemotherapeutic agent if activation of the tumor should arise. LESSONS Because complete resection of multifocal IDEM ependymomas is not feasible, continued monitoring with brain and spine MRI is warranted to detect potential tumor dissemination in the CSF.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Multifocal intradural extramedullary ependymoma, MYCN amplified: illustrative case

Shields

Sun

Highfield³

et al. 2022

Journal of Neurosurgery: Case Lessons

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“…Our cohort of patients had features similar to the published literature in as far as age and gender occurrence of IDEM tumours are concerned, as well as main clinical features. [2][3][4] The frequency of IDEM tumours as a subset of all spinal tumours varies widely in the literature, as does the relative prevalence of the different types of tumour within this group. 2,[4][5][6] Our patients do, on average, present late.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4] The frequency of IDEM tumours as a subset of all spinal tumours varies widely in the literature, as does the relative prevalence of the different types of tumour within this group. 2,[4][5][6] Our patients do, on average, present late. This could be due to the socio-economic challenges prevalent in the demographic of patients that access public hospitals.…”

Section: Discussionmentioning

confidence: 99%

Intradural extramedullary spinal masses treated at the Wits teaching hospitals between 2014 - 2017

Ouma¹

2019

S. Afr. j. surg.

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Background: Intradural extramedullary (IDEM) spinal masses are common lesions with varying histological diagnoses often associated with significant neurological deficits. This study aimed to describe the epidemiology, management and perioperative outcome of IDEM tumours seen at the teaching hospitals of the University of the Witwatersrand, Johannesburg, between 2014 and2017. Results: 92 patients were included in the study. The ages ranged from 21 to 87 years, sex ratio was M:F 1:1.4, and duration of symptoms prior to diagnosis ranged between 3 days to 18 months. Local and radicular type pain as well as motor weakness were the commonest symptoms. 67% had severe neurological deficit McCormick Grade III and IV. Schwannoma (26) Neurofibromas (21) and Meningiomas (16) and were the most frequent tumour types. Meningiomas predominantly occurred at the cranio-cervical and thoracic levels. Nerve sheath tumours were mostly found at the cervical and lumbar levels while filum terminale ependymomas occurred at the thoracolumbar area. Laminectomy was the commonest surgical approach employed, and the extent of resection varied, with total excision in half the cases. Neurological function was regained in 3 patients, deteriorated in two and was unchanged in the remainder. Conclusions: IDEM tumours are an important subset of spinal cord compressive lesions Presentation with severe neurological deficit is common and though resection is feasible neurological deficit remains in the vast majority. Earlier detection should improve the results of surgery.

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Variety of preoperative MRI changes in spinal cord ependymoma of WHO grade II: a case series

et al. 2018

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MRI in cases of spinal ependymomas of WHO grade II showed characteristics such as hemorrhage and cyst formation that varied over time. In particular, cases with cyst and hemosiderin showed tumor enlargement, including enlargement of lesions on the caudal and rostral sides and enlargement of Gd-enhanced lesions. These characteristics might influence gait ability during preoperative period. We emphasize that early surgery is still the standard of care for cervical intramedullary ependymoma, and our findings in this study should not be interpreted to indicate that such early surgery is not necessary in symptomatic cases. These slides can be retrieved under electronic supplementary material.

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Multiple intradural-extramedullary spinal ependymomas including tumors with different histological features

Abstract: Surgical resections and subsequent radiotherapy and chemotherapy for cases with multiple intradural-extramedullary ependymomas can result in a good postoperative course.

Cited by 10 publications

References 11 publications

Multifocal intradural extramedullary ependymoma, MYCN amplified: illustrative case

Multifocal intradural extramedullary ependymoma, MYCN amplified: illustrative case

Intradural extramedullary spinal masses treated at the Wits teaching hospitals between 2014 - 2017

Variety of preoperative MRI changes in spinal cord ependymoma of WHO grade II: a case series

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