Intradural Extramedullary Nonconus Nonfilum Spinal Ependymomas: Report of a Rare Variant and Newer Insights into Their Histogenesis with Proposal of a Classification Scheme and a Management Algorithm Based on a Review of Literature

Das, Kuntal Kanti; Attri, Gagandeep; Singh, Shanker K.; Bhaisora, Kamlesh Singh; Verma, Pawan Kumar; Jaiswal, Sushila; Jaiswal, Awadeshkumar; Behari, Sanjay

doi:10.1016/j.wneu.2019.10.152

Cited by 5 publications

(4 citation statements)

References 54 publications

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“…In the review of IDEM ependymomas described by Das et al, they classified all 54 reported cases into four types, and showed “pure IDEM ependymoma” was the most common type (32 cases). 41) Like their report, our literature review revealed that almost all IDEM ependymoma had no connection with spinal cord, nerve root, or dura mater, and the present case was also thought to be “pure IDEM ependymoma.”…”

Section: Discussionsupporting

confidence: 61%

“…The case reports on IDEM ependymoma except for the lesion in the filum terminale and the conus medullaris were searched, and 44 cases in 41 articles were found finally. 1 – 41) Characteristics of all 45 cases (including the present case) are summarized in Table 1 . There were more women than men (19 men and 26 women), and tumor location was mainly in thoracic level; two tumors were in cranio-cervical junction, six in cervical, 23 in thoracic, four tumors lay from cervical to thoracic level, and 10 cases showed multiple lesions.…”

Section: Case Presentationmentioning

confidence: 99%

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A Case of Spinal Ependymoma Developed in the Extramedullary Location: A Case Report and Literature Review

Makino

Kawanabe

Fujimoto

et al. 2021

NMC Case Report Journal

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Intradural extramedullary (IDEM) ependymoma except for tumors originated from the filum terminale or conus medullaris is rare. The present study showed a case of IDEM ependymoma. A 16-year-old boy was referred to our hospital with a complaint of right hypochondriac pain and motor weakness in his right leg. MRI revealed a solitary intradural tumor at Th5-8 level with syringomyelia at Th2-4 level. Microscopic total tumor resection was performed with right hemi-laminectomy of Th4-9. Histological diagnosis was ependymoma (WHO grade 2). Although his leg weakness was worsened transiently, he showed improvement in leg weakness being able to go up and down the stairs 1 month after the surgery. There was no tumor recurrence until now, 7 years after the surgery, without any adjunctive therapies. A total of 44 cases of IDEM ependymoma had been reported in the past literatures. They are thought to arise from ependymal cells which remained during the process of neural tube closure. Like intramedullary ependymomas, most of the IDEM ependymomas have clear border to surrounding tissue and often removed completely. However, a small number of recurrences and malignant transformations had been reported after complete resections despite benign histological features tumors. In the case of totally resected low grade IDEM ependymoma, it is thought to be reasonable to perform long-term periodical radiographic follow-up without postoperative adjunctive therapy.

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Section: Discussionsupporting

confidence: 61%

Section: Case Presentationmentioning

confidence: 99%

A Case of Spinal Ependymoma Developed in the Extramedullary Location: A Case Report and Literature Review

Makino

Kawanabe

Fujimoto

et al. 2021

NMC Case Report Journal

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“…Intramedullary tumors can present with nonspecific symptoms, posing challenges in accurate diagnosis [ 8 ]. The growth of the neoplasm leads to a gradual deformation of the spinal cord, which in turn can cause a range of symptoms, including pain, numbness, sensory deficits, motor weakness, and disturbances in bowel and bladder function [ 3 , 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…The growth of the neoplasm leads to a gradual deformation of the spinal cord, which in turn can cause a range of symptoms, including pain, numbness, sensory deficits, motor weakness, and disturbances in bowel and bladder function [ 3 , 9 , 10 ]. Similarly, tumors with extramedullary components, depending on their location, may produce comparable symptoms [ 8 , 11 ]. In our study, the most frequently observed clinical symptoms were pain and weakness.…”

Section: Discussionmentioning

confidence: 99%

A comprehensive evaluation of imaging features in pediatric spinal gliomas and their value in predicting tumor grade and histology

Cerron-Vela,

Gonçalves,

Tierradentro-García

et al. 2024

Neuroradiology

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Purpose Pediatric spinal cord gliomas (PSGs) are rare in children and few reports detail their imaging features. We tested the association of tumoral grade with imaging features and proposed a novel approach to categorize post-contrast enhancement patterns in PSGs. Methods This single-center, retrospective study included patients <21 years of age with preoperative spinal MRI and confirmed pathological diagnosis of PSG from 2000-2022. Tumors were classified using the 5th edition of the WHO CNS Tumors Classification. Two radiologists reviewed multiple imaging features, and classified enhancement patterns using a novel approach. Fisher's exact test determined associations between imaging and histological features. Results Forty-one PSGs were reviewed. Thirty-four were intramedullary, and seven were extramedullary. Pilocytic astrocytoma was the most common tumor (39.02%). Pain and weakness were the most prevalent symptoms. Seven patients (17.07%) died. Cyst, syringomyelia, and leptomeningeal enhancement were associated with tumor grade. Widening of the spinal canal was observed only in low-grade astrocytomas. There was a significant association between tumor grade and contrast enhancement pattern. Specifically, low-grade PSGs were more likely to exhibit type 1A enhancement (mass-like, with well-defined enhancing margins) and less likely to exhibit type 1B enhancement (mass-like, with ill-defined enhancing margins). Conclusion PSGs display overlapping imaging features, making grade differentiation challenging based solely on imaging. The correlation between tumor grade and contrast enhancement patterns suggests a potential diagnostic avenue, requiring further validation with larger, multicenter studies. Furthermore, Low-grade PSGs display cysts and syringomyelia more frequently, and leptomeningeal enhancement is less common.

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Ependymoma of the spinal cord with multiple intradural extramedullary ependymomas: The first case report and literature review

Wang¹,

Luo²,

Tan³

et al. 2022

Clinical Imaging

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Intradural Extramedullary Nonconus Nonfilum Spinal Ependymomas: Report of a Rare Variant and Newer Insights into Their Histogenesis with Proposal of a Classification Scheme and a Management Algorithm Based on a Review of Literature

Cited by 5 publications

References 54 publications

A Case of Spinal Ependymoma Developed in the Extramedullary Location: A Case Report and Literature Review

A Case of Spinal Ependymoma Developed in the Extramedullary Location: A Case Report and Literature Review

A comprehensive evaluation of imaging features in pediatric spinal gliomas and their value in predicting tumor grade and histology

Ependymoma of the spinal cord with multiple intradural extramedullary ependymomas: The first case report and literature review

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