Clear cell ependymoma with trisomy 19 developing bone metastases

Payet, M; Conter, Cécile Faure; Labrousse, François; Paula, André Maues De; Marabelle, Aurélien; Branger, Dominique Figarella; Bouvier, R; Frappaz, Didier

doi:10.1007/s00381-011-1656-x

Cited by 6 publications

(8 citation statements)

References 13 publications

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“…[ 30 ] the two biggest cohorts on the subject who have reported this disease in pediatric age group along with others who have also reported this disease in the pediatric age group. [ 11 27 28 ] Similar to our age group, we found comparable cohorts of Kawano et al ., Min and Scheithauer[ 16 23 ] along with few case reports from others. [ 3 5 14 31 32 33 ]…”

Section: Discussionsupporting

confidence: 89%

“…[ 22 31 ] However, Kawano's second series showed all 6 cases in cerebellum, whereas his first series showed 2 of 4 cases in cerebellum. Furthermore, occipital CCE has also been reported in the literature[ 16 17 27 28 ] and to add on Amatya et al . [ 3 ] reported CCE in medulla oblongata, Katoh et al .…”

Section: Discussionmentioning

confidence: 74%

“…The literature has also described more cases of ODG like clear cells present in anaplastic ependymoma Grade III rather than ependymoma Grade II. [ 6 8 13 22 28 30 ]…”

Section: Discussionmentioning

confidence: 99%

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Ependymal tumors with oligodendroglioma like clear cells: Experience from a tertiary care hospital in Pakistan

Hashmi¹,

Khan²,

Waqas³

et al. 2015

Surg Neurol Int

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Background:Ependymal tumors with oligodendroglioma like clear cells have never been reported from Pakistan. We aimed to see the features and outcomes of this rare entity.Methods:It was retrospective cohort conducted at the Department of Neurosurgery, Aga Khan University from 2003 to 2013. The medical records and radiology of patients with proven histopathology were reviewed. Analysis was done on SPSS 20.Results:Eleven cases of ependymal tumors with clear cells were found, which equated to 1.5% of the total tumor burden in 11 years. The median age was 49 years. Most common presenting symptom was headache 54.5%. Out of 11 patients, 9 patients had a supratentorial tumor. Magnetic resonance imaging showed hypointense signals on T1 and hyperintense signals on T2-weighted images in all cases. Contrast enhancement was found in 9 patients (77.8%), necrosis and hemorrhage was found in 4 (36%) and 3 (27%) patients, respectively. Immunohistochemistry showed glial fibrillary acidic protein and epithelial membrane antigen positivity in all cases. Ki-67 showed high proliferative index in 6 patients. According to the World Health Organization grading of ependymal tumors, 2 patients had Grade II tumors, and 9 patients had Grade III tumors with clear cells. Gross total resection was achieved in 6 (54.5%) and subtotal resection in 5 patients (45.4%). Recurrence was observed in 9 patients. Six patients died of the disease. Median progression-free survival and overall survival was 8 months and 10 months, respectively.Conclusion:Ependymal tumors with clear cells presented more commonly in Grade III lesions and were more aggressive in behavior with poorer outcome compared to similar studies.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 74%

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Ependymal tumors with oligodendroglioma like clear cells: Experience from a tertiary care hospital in Pakistan

Hashmi¹,

Khan²,

Waqas³

et al. 2015

Surg Neurol Int

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“…One case out of 11 was previously reported. 9 Patients included retrospectively in this study have provided their written consent for clinical data collection and genetic analysis according to national policies and all studies have been reviewed by the appropriate ethics committee. In all cases, the following pathological features were assessed as present or absent: clear cell cytology, branching vessels, calcifications, perivascular pseudorosettes, ependymal rosettes, microvascular proliferation, necrosis, and mitotic count (cutoff ¼ 5/10 high-power fields).…”

Section: Patient's Samplesmentioning

confidence: 99%

Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling

et al. 2016

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“…[ 18 ] In contrast to the WHO classification designating CCE as Grade 2, we have 9 out of 32 cases demonstrating the ependymal clear cells in AE Grade 3 instead of ependymoma Grade 2 – also, previously highlighted in the literature. [ 10 19 20 21 22 23 ]…”

Section: Discussionmentioning

confidence: 99%

Prognostic implications of histological clear cells in high-grade intracranial ependymal tumors: A retrospective analysis from a tertiary care hospital in Pakistan

et al. 2018

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Background:Clear cell variant in ependymal tumors is rare. We aimed to compare the features and outcome of the World Health Organization (WHO) Grade 3 ependymal tumors with clear cells to the WHO Grade 3 classic anaplastic ependymoma (AE).Materials and Methods:A retrospective cohort study conducted at the Department of Neurosurgery, Aga Khan University, Pakistan, from 2003 to 2013. The medical records and radiology of patients with proven histopathology were reviewed. The analysis was done on SPSS 20.Results:Nine cases of clear cell variant and 23 cases of classic AE were found, both of which combined equated to 4% of the total tumor burden in 11 years. The median age of clear cell ependymoma (CCE) and AE were 49 and 37 years, respectively. Presenting symptoms included headache in 66% of CCE and 63% in AE, raised intracranial pressure accounting for 33% of CCE and 54% of AE, dizziness in 22% CCE and 39% AE, while seizures presented equally in both. Supratentorial location was observed in 77% CCE and 48% AE. Both showed hypointense signals on T1-weighted images of magnetic resonance imaging (MRI) while T2-weighted images showed hyperintensity in all cases of CCE but only 80% of AE. MRI characteristics such as the presence of cystic component were found in 89% of CCE and 68% of AE, necrosis in 33% CCE, and 22% AE, hemorrhagic in 22% CCE and 9% AE, and equivalent contrast enhancement. Gross total resection was achieved in 5 (55%) patients of CCE, compared to 6 (26%) patients in AE. Subtotal resection was done in 4 (44%) patients of CCE and 15 (65%) patients of AE. Radiotherapy was given to 5 (55%) patients of CCE and 12 (52%) patients of AE. Recurrence was observed in 77% cases of CCE and 70% of AE, with metastasis in 29% of AE and in only one patient of CCE. Repeat surgery was done in 3 (33%) cases of CCE and 8 (35%) cases of AE. Median progression-free survival and overall survival were 9 and 13 months, respectively, in CCE while 14 and 18 months, respectively, for AE.Conclusion:Clear cells in the WHO Grade 3 ependymal tumors are found mainly in a comparatively older adult population with a predilection for supratentorial location and are more aggressive in behavior with poorer outcome than AE.

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Clear cell ependymoma with trisomy 19 developing bone metastases

Cited by 6 publications

References 13 publications

Ependymal tumors with oligodendroglioma like clear cells: Experience from a tertiary care hospital in Pakistan

Ependymal tumors with oligodendroglioma like clear cells: Experience from a tertiary care hospital in Pakistan

Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling

Prognostic implications of histological clear cells in high-grade intracranial ependymal tumors: A retrospective analysis from a tertiary care hospital in Pakistan

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