Spinal cord ependymoma: a review of the literature and case series of ten patients

Celano, Emma C.; Salehani, Arsalaan; Malcolm, James G.; Reinertsen, Erik; Hadjipanayis, Constantinos

doi:10.1007/s11060-016-2135-8

Cited by 84 publications

(112 citation statements)

References 48 publications

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“…[15][16][17] GTR is facilitated in characteristically well-defined and encapsulated World Health Organization (WHO) Grade I tumors; however, this is less likely in infiltrative grade II and the more aggressive grade III ependymomas. 18 Whenever possible, intramedullary ependymomas should be removed en bloc, as piecemeal removal may facilitate CSF dissemination. 19 Although surgical excision of spinal ependymomas is the most important factor determining PFS and OS, it is not without risk.…”

Section: Intramedullary Spinal Cord Ependymomamentioning

confidence: 99%

Case-based review: ependymomas in adults

Cachia

Johnson

Kaufmann

et al. 2018

Neuro-Oncology Practice

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Ependymomas are rare primary central nervous system (CNS) tumors in adults. They occur most commonly in the spinal cord, and have classically been graded histologically into World Health Organization (WHO) grades I, II, or III based on the level of anaplasia. Recent data are showing that genetic heterogeneity occurs within the same histological subgroup and that ependymomas arising from different CNS locations have different molecular signatures. This has renewed interest in developing targeting therapies based on molecular profiles especially given the variable outcomes with radiation and the poor results with cytotoxic agents. In this paper, we present the case of a 46-year-old woman with a classic presentation of spinal cord ependymoma and discuss the current histopathological and molecular classification for ependymomas as well as current guidelines for patient management.

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Section: Intramedullary Spinal Cord Ependymomamentioning

confidence: 99%

Case-based review: ependymomas in adults

Cachia

Johnson

Kaufmann

et al. 2018

Neuro-Oncology Practice

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“…Ependymoma is a rare primary tumor of the central nervous system and can present anywhere along the ventricular system and spinal cord. Surgical intervention is usually necessary to ensure favorable neurological outcome [ 1 , 2 ]. It is known that specific types of central nervous system tumors (for instance meningiomas) can accelerate in growth during pregnancy [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Severe Nightly Thoracic Pain Presenting during Pregnancy: A Case Report

2018

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We present the case of a woman who developed severe nightly thoracic pain during pregnancy without neurological deficits upon examination. Spontaneously after childbirth, the pain was markedly reduced. Further investigation showed that her pain was caused by an ependymoma in the cervicothoracic spinal cord. Gross total resection was accomplished, and the patient has been free of pain ever since. With this case, we want to draw attention to a rare, but possibly very disabling, cause of increasing nightly thoracic pain during pregnancy. Spontaneous improvement after childbirth could erroneously cause postponement of further investigation.

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“…In adults, ependymomas are the most common ISCT, comprising approximately 50%-60% of ISCTs. 2,3 Most ependymomas are slow growing and well demarcated from the spinal cord parenchyma. 7 Accordingly, gross-total resection (GTR) is possible in most cases of ependymoma.…”

mentioning

confidence: 99%

“…Previous studies have reported that the extent of resection is a strong predictor of progression-free survival (PFS). 2,8,9 Intramedullary astrocytomas, the second most common ISCT, possess more infiltrative features than ependymomas. 7,14 Because of this infiltrative characteristic, the relationship between GTR and PFS in astrocytoma is less clear, 12 and adjuvant therapy such as radiotherapy and chemotherapy is often combined with resection.…”

mentioning

confidence: 99%

Intramedullary spinal cord ependymoma and astrocytoma: intraoperative frozen-section diagnosis, extent of resection, and outcomes

Hongo¹,

Takai²,

Komori

et al. 2019

Journal of Neurosurgery: Spine

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OBJECTIVEThe intraoperative differentiation of ependymomas from astrocytomas is important because neurosurgical strategies differ between these two tumor groups. Previous studies have reported that the diagnostic accuracy of intraoperative frozen sections of intracranial central nervous system (CNS) tumors is higher than 83%–97%, whereas that for spinal intramedullary tumors remains unknown. Herein, authors tested the hypothesis that intraoperative frozen-section diagnosis is the gold standard for a differential diagnosis of intramedullary spinal cord tumors.METHODSThe clinical characteristics, intraoperative histological diagnosis from frozen sections, extent of tumor resection, progression-free survival (PFS), and overall survival (OS) of 49 cases of intramedullary spinal cord ependymomas (n = 32) and astrocytomas (n = 17) were retrospectively evaluated.RESULTSThe frozen-section diagnosis and final diagnosis with permanent sections agreed in 23 (72%) of 32 cases of ependymoma. Of the 9 cases of ependymoma in which the frozen-section diagnosis disagreed with the final diagnosis, 4 were incorrectly diagnosed as astrocytoma and the other 5 cases had a nonspecific diagnosis, such as glioma. Nonetheless, gross-total resection was achieved in 6 of these 9 cases given the presence of a dissection plane. The frozen-section diagnosis and final diagnosis agreed in 12 (71%) of 17 cases of astrocytoma. Of the 5 cases of astrocytoma in which the frozen-section diagnosis disagreed with the final diagnosis, 1 was incorrectly diagnosed as ependymoma and the other 4 had a nonspecific diagnosis. Gross-total resection was achieved in only 1 of these 5 cases.A relationship between the size of tumor specimens and the diagnostic accuracy of frozen sections was not observed. Ependymal rosettes and perivascular pseudorosettes were observed in 30% and 57% of ependymomas, respectively, but were absent in astrocytomas.Progression-free survival and OS were both significantly longer in cases of ependymoma than in cases of astrocytoma (p < 0.001). Gross-total resection was achieved in 69% of ependymomas and was associated with longer PFS (p = 0.041). In the astrocytoma group, gross-total resection was achieved in only 12% and there was no relationship between extent of resection and OS. Tumor grades tended to correlate with OS in astrocytomas (p = 0.079).CONCLUSIONSThe diagnostic accuracy of intraoperative frozen sections was lower for intramedullary spinal cord ependymomas and astrocytomas in the present study than that for intracranial CNS tumors reported on in the literature. Surgical strategies need to be selected based on multiple factors, such as clinical characteristics, preoperative imaging, frozen-section diagnosis, and intraoperative findings of the tumor plane.

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Spinal cord ependymoma: a review of the literature and case series of ten patients

Cited by 84 publications

References 48 publications

Case-based review: ependymomas in adults

Case-based review: ependymomas in adults

Severe Nightly Thoracic Pain Presenting during Pregnancy: A Case Report

Intramedullary spinal cord ependymoma and astrocytoma: intraoperative frozen-section diagnosis, extent of resection, and outcomes

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