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

Abstract BACKGROUND Ependymomas are currently classified into 9 subgroups by DNA methylation profiles. Although spinal cord ependymoma (SP-EPN) is distinct from other tumors, diversity within SP-EPN is still unclear. Here, we used transcriptomic and epigenomic profiles to investigate the diversity among Japanese SP-EPN cases. MATERIALS AND METHODS We analyzed 57 SP-EPN patients (32 males and 25 females, aged from 18 to 78 years, median: 52), including two cases of neurofibromatosis type 2, five cases of grade 3 (WHO grade). We obtained transcriptome (RNA-seq) and DNA methylation (Infinium Methylation EPIC array) data from fresh frozen specimens of SP-EPN resected at the University of Tokyo Hospital and our collaborative groups. RESULTS Three cases had a previous intracranial ependymoma operation. Hierarchical clustering of the DNA methylation data showed that these three cases of intracranial origin as a different cluster from spinal origin. The 45 grade 2 spinal ependymoma showed a relatively homogenous methylation pattern. However, the methylation status of HOX gene cluster regions is compatible with the segment of origin, which reflects the cells of origins are derived after the determination of segment identity. RNA sequencing of 57 cases revealed two subgroups within grade 2. Gene ontology analysis of differentially expressed genes suggested the difference in metabolic state such as rRNA translation and mitochondrial respiration between the two expression subgroups. CONCLUSION Epigenetic analysis indicated the accurate body segment origin of SP-EPN. We observed that metabolic states could divide grade 2 spinal cord ependymoma into 2 subgroups and will present the relationship to clinicopathological information.

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The role of radiosurgery in the management of WHO Grade II and III intracranial meningiomas

Neurosurgical FOCUS ◽

10.3171/2013.9.focus13364 ◽

2013 ◽

Vol 35 (6) ◽

pp. E16 ◽

Cited By ~ 24

Author(s):

Dale Ding ◽

Robert M. Starke ◽

John Hantzmon ◽

Chun-Po Yen ◽

Brian J. Williams ◽

...

Keyword(s):

Radiation Therapy ◽

Stereotactic Radiosurgery ◽

Case Reports ◽

Case Series ◽

External Beam Radiation ◽

Beam Radiation ◽

Who Grade ◽

Intracranial Meningiomas ◽

Grade Ii ◽

Grade Iii

Object WHO Grade II and III intracranial meningiomas are uncommon, but they portend a significantly worse prognosis than their benign Grade I counterparts. The mainstay of current management is resection to obtain cytoreduction and histological tissue diagnosis. The timing and benefit of postoperative fractionated external beam radiation therapy and stereotactic radiosurgery remain controversial. The authors review the stereotactic radiosurgery outcomes for Grade II and III meningiomas. Methods A comprehensive literature search was performed using PubMed to identify all radiosurgery series reporting the treatment outcomes for Grade II and III meningiomas. Case reports and case series involving fewer than 10 patients were excluded. Results From 1998 to 2013, 19 radiosurgery series were published in which 647 Grade II and III meningiomas were treated. Median tumor volumes were 2.2–14.6 cm3. The median margin doses were 14–21 Gy, although generally the margin doses for Grade II meningiomas were 16–20 Gy and the margin doses for Grade III meningiomas were 18–22 Gy. The median 5-year PFS was 59% for Grade II tumors and 13% for Grade III tumors, which may have been affected by patient age, prior radiation therapy, tumor volume, and radiosurgical dose and timing. The median complication rate following radiosurgery was 8%. Conclusions The current data for radiosurgery suggest that it has a role in the management of residual or recurrent Grade II and III meningiomas. However, better studies are needed to fully define this role. Due to the relatively low prevalence of these tumors, it is unlikely that prospective studies will be feasible. As such, well-designed retrospective analyses may improve our understanding of the effect of radiosurgery on tumor recurrence and patient survival and the incidence and impact of treatment-induced complications.

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WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options

Journal of Neuro-Oncology ◽

10.1007/s11060-008-9717-z ◽

2008 ◽

Vol 91 (3) ◽

pp. 323-328 ◽

Cited By ~ 30

Author(s):

Toba N. Niazi ◽

Elizabeth M. Jensen ◽

Randy L. Jensen

Keyword(s):

Treatment Options ◽

Case Series ◽

Who Grade ◽

Grade Ii

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Spinal cord oligodendroglioma with 1p and 19q deletions presenting with cerebral oligodendrogliomatosis

Journal of Neurosurgery Spine ◽

10.3171/2009.2.spine08853 ◽

2009 ◽

Vol 10 (6) ◽

pp. 557-563 ◽

Cited By ~ 16

Author(s):

Kern H. Guppy ◽

Paul T. Akins ◽

Gregory S. Moes ◽

Michael D. Prados

Keyword(s):

Spinal Cord ◽

Tumor Cells ◽

Cervical Spinal Cord ◽

Cervical Cord ◽

Meningeal Enhancement ◽

Who Grade ◽

Term Survival ◽

Radiographic Findings ◽

Grade Ii ◽

Cord Lesion

Oligodendroglioma of the spinal cord is a rare tumor that most often presents with spinal cord symptoms. The authors present a case of spinal cord oligodendroglioma that was associated with cerebral rather than spinal cord symptoms. A 30-year-old woman developed nausea, vomiting, and severe headaches. Magnetic resonance imaging of the brain showed meningeal enhancement. The patient underwent a craniotomy with biopsies of the meninges and brain. The biopsy findings revealed an abnormal arachnoid thickening without tumor cells. The patient later developed hydrocephalus and underwent shunt placement. Cerebrospinal fluid cytological findings were negative for tumor cells or infection. She was found to have a cervical cord lesion at C3–4 that was initially nonenhancing but later enhanced after Gd administration. Biopsy of the cord lesion with partial resection showed a WHO Grade II oligodendroglioma with 1p and 19q deletions determined by fluorescence in situ hybridization. Neurooncological treatment with tumor radiation and temozolomide (Temodor) resulted in improvement in radiographic findings, symptoms, and long-term survival. This paper presents an extensive review of the literature, which revealed only 2 other reported cases of cerebral symptoms in adults that preceded spinal cord symptoms in a patient with oligodendroglioma of the spinal cord. It is also the first reported case of oligodendrogliomatosis due to a cervical spinal cord oligodendroglioma with 1p and 19q deletions.

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

Journal of Neuro-Oncology ◽

10.1007/s11060-016-2135-8 ◽

2016 ◽

Vol 128 (3) ◽

pp. 377-386 ◽

Cited By ~ 27

Author(s):

Emma Celano ◽

Arsalaan Salehani ◽

James G. Malcolm ◽

Erik Reinertsen ◽

Constantinos G. Hadjipanayis

Keyword(s):

Spinal Cord ◽

Case Series ◽

Review Of The Literature ◽

Spinal Cord Ependymoma

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P14.36 Characterising Meningiomas in young patients under 40

Neuro-Oncology ◽

10.1093/neuonc/noab180.156 ◽

2021 ◽

Vol 23 (Supplement_2) ◽

pp. ii45-ii46

Author(s):

M Attia ◽

Y Mirkin ◽

R Hershkovitch ◽

Z R Cohen ◽

Z Zibly ◽

...

Keyword(s):

Skull Base ◽

Medical Center ◽

Young Patients ◽

Case Series ◽

Population Level ◽

Who Grade ◽

Younger Patients ◽

Grade Ii ◽

Grade Iii

Abstract BACKGROUND Meningiomas are the most common primary CNS tumor in adults, with a median age at diagnosis of 65 years and a female to male ratio of 2–3:1 (except in grade II, III meningiomas). Younger patients usually have a genetic background or history of prior radiation exposure. Based on population-level studies, approximately 80–85% of meningiomas are WHO grade I (benign), 15–18% grade II (atypical), and 1–3% grade III (anaplastic). This case series describes a single center experience of the presentation and pathology of meningiomas in patients under the age of 40. MATERIALS AND METHODS We reviewed the Sheba Medical Center Neurosurgery and radiotherapy databases containing patients who were operated on for meningioma between the years 2011–2020. Patients under 40 were selected and information from digital medical records was gathered on these patients, including pathology reports, surgical reports, clinical and radiological data, and the use of radiotherapy or medical treatments. RESULTS Of 600 patients in the database, 49 patients under 40 (8.3%) were identified and 54 tumors were reviewed (five patients were operated on for multiple meningiomas). 31 patients were female (63%); 2 were under 20 years old, 15 were age 20–29 and 32 were age 30–39 at first presentation. Seven patients had received prior radiation therapy for a childhood malignancy. 28 meningiomas were histology confirmed grade I (52%), 22 were grade II (41%), 2 were grade III (4%), and 2 (4%) were grade undetermined. 22 tumors were located in the skull base (41%) and 32 (59%) in the brain convexity. Of the skull base tumors, 16 were grade I, 5 were grade II, and one was grade III. Of the convexity tumors, 12 were grade I, 16 were grade II, and 2 were grade III. Median follow-up was 35 months, and longest follow-up time was 98 months. Six patients were treated with additional radiotherapy after surgery. CONCLUSION Our experience with meningioma patients under 40 revealed a different grade segregation than the older population, with younger patients showing a higher incidence of grade II tumors. These tumors are generally more aggressive, and require careful resection and consideration for post-surgical radiotherapy. Further validation with population based databases is required.

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CBIO-18. TP53-MUTANT OLIGODENDROGLIOMA: A SINGLE INSTITUTION CASE SERIES

Neuro-Oncology ◽

10.1093/neuonc/noab196.118 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi30-vi31

Author(s):

Mason Webb ◽

Sani Kizilbash ◽

Thomas Kollmeyer ◽

Robert Jenkins ◽

Sarah Sung ◽

...

Keyword(s):

Gene Mutation ◽

Tp53 Mutation ◽

Case Series ◽

Molecular Testing ◽

Diffuse Glioma ◽

Who Grade ◽

Tp53 Mutations ◽

Grade Ii ◽

Who Grade Iii ◽

Grade Iii

Abstract TP53 mutations are frequent in IDH-mutant astrocytomas but unusual in oligodendroglioma and the clinical significance of TP53 mutations in oligodendroglioma are not well characterized. We reviewed genetically defined oligodendroglioma (i.e., IDH-mutant, whole-arm 1p/19q-codeleted diffuse glioma) cases that were molecularly profiled (2017-2020) at our institution and identified 7 cases with TP53 mutation (9%; n=76). Molecular testing was performed using targeted neuro-oncology NGS panel (50-gene mutation and/or 187-gene mutation/rearrangement) and OncoScan™ microarray. Four (of 7) patients were female. Median age at diagnosis was 43 years (range, 23-63). Most common presenting symptom was seizures (3 of 7). All tumors were supratentorial. Histologically, 3 tumors were WHO grade II and 4 were WHO grade III. Two (of 3) patients with a WHO grade II tumor underwent biopsy and radiotherapy at diagnosis followed by temozolomide at recurrence (progression at 67 and 157 months after diagnosis; overall survival of 124 and 201 months). Three (of 4) patients with a WHO grade III tumor were diagnosed within the last two years and are currently progression-free after standard therapy. Molecularly, in addition to TP53 mutation(s), all cases had an IDH1 and TERT promoter mutation as well as other gene mutation(s) including FUBP1 (n=5), SETD2 (n=4), PIK3R1 (n=4), PIK3CA (n=3), NF1 (n=3) and CIC (n=3). In 3 (of 7) cases, the mutational profile with high mutation count enriched for C >T/G >A transitions was highly suggestive of a hypermutation phenotype (2 cases were recurrent tumors treated with temozolomide; a recurrent and a treatment-naïve tumor had mismatch repair gene mutation). Five (of 7) cases, including the 3 hypermutant cases, lacked functional TP53 (1 case with 2 mutations, 2 cases with 1 mutation plus loss of other copy, 2 cases with 1 mutation plus copy neutral loss-of-heterozygosity). TP53 mutations are uncommon in oligodendroglioma and appear enriched in hypermutant tumors.

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A comprehensive model including preoperative peripheral blood inflammatory markers for prediction of the prognosis of diffuse spinal cord astrocytoma following surgery

European Spine Journal ◽

10.1007/s00586-021-06724-4 ◽

2021 ◽

Author(s):

Bo Pang ◽

Rui-Chao Chai ◽

Yao-Wu Zhang ◽

Yu-Zhou Chang ◽

Wei-Hao Liu ◽

...

Keyword(s):

Spinal Cord ◽

Peripheral Blood ◽

Inflammatory Markers ◽

Prognostic Model ◽

Cox Regression ◽

Histological Grade ◽

Ki 67 ◽

Who Grade ◽

Grade Ii ◽

Spinal Cord Astrocytoma

Abstract Purpose Due to the rarity of diffuse spinal cord astrocytoma, an effective model is still lacking to stratify their prognosis. Here, we aimed to establish a prognostic model through comprehensively evaluating clinicopathological features and preoperative peripheral blood inflammatory markers in 89 cases. Methods We performed univariate and multivariate Cox regression to identify prognosis factors. The Kaplan–Meier curves and ROC curves were employed to compare the prognostic value of selected factors. Results In addition to clinicopathological factors, we revealed the preoperative peripheral blood leukocyte count, neutrophils-to-lymphocytes ratio (NLR), and platelet-to-lymphocyte ratio (PLR) were also significantly correlated with overall survival of spinal cord astrocytoma in univariate Cox regression, and NLR was still significant in multivariate Cox analysis. Further, we demonstrated that NLR ≤ 3.65 and preoperative McCormick score (MMS) ≤ 3 were independently correlated with better survival of WHO grade IV tumors. Meanwhile, Ki-67 < 10% and resection extent ≥ 90% were independent prognostic factors in WHO grade II/III tumors. Finally, we developed a prognostic model that had better predictive efficiencies than WHO grade and histological grade for 1-year (AUC = 76.6), 2- year (AUC = 80.9), and 3-year (AUC = 80.3) survival. This model could classify tumors into 4 classifications with increasingly poor prognosis: 1, WHO grade II/III, with Ki-67 < 10% and resection extent ≥ 90%; 2, WHO grade II/III, Ki-67 ≥ 10% or resection < 90%; 3, WHO grade IV, NLR ≤ 3.65 and MMS ≤ 3; 4, WHO grade IV, with NRL > 3.65 or MMS = 4. Conclusion We successfully constructed a comprehensive prognostic model including preoperative peripheral blood inflammatory markers, which can stratify diffuse spinal cord astrocytoma into 4 subgroups.

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Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.5.peds09553 ◽

2010 ◽

Vol 6 (2) ◽

pp. 137-144 ◽

Cited By ~ 48

Author(s):

Martin Benesch ◽

Daniela Weber-Mzell ◽

Nicolas U. Gerber ◽

Katja von Hoff ◽

Frank Deinlein ◽

...

Keyword(s):

Spinal Cord ◽

Overall Survival ◽

Adjuvant Treatment ◽

Progression Free Survival ◽

Gross Total Resection ◽

Myxopapillary Ependymoma ◽

Total Resection ◽

Who Grade ◽

Free Survival ◽

Spinal Cord Ependymoma

Object Reports on spinal cord ependymoma in children are rare. The aim of this study was to evaluate the clinical spectrum, treatment, and outcome of children with primary ependymoma of the spinal cord who were registered in the database of the pediatric German brain tumor studies Hirntumor (HIT) '91 and HIT 2000. Methods Between 1991 and 2007, 29 patients (12 male and 17 female, median age at diagnosis 13.6 years) with primary spinal cord ependymoma (myxopapillary ependymoma WHO Grade I, II, and III tumors in 6, 17, and 6 patients, respectively) were identified. Four patients had neurofibromatosis Type 2. Results With a median follow-up of 4.2 years (range 0.48–15 years), 28 patients (96.6%) were alive. Seven patients (24.1%) developed progressive disease or relapse, 2 after gross-total resection (GTR) and 5 after incomplete resection or biopsy. One patient with anaplastic ependymoma (WHO Grade III) died 65 months after diagnosis of disease progression. Primary adjuvant treatment (radiotherapy, chemotherapy, or both) was used in 8 (50%) of 16 patients following GTR and in 9 (82%) of 11 patients who underwent less than a GTR. Three additional patients were treated adjuvantly following progression. Estimated progression-free survival and overall survival rates at 5 years were 72.3% (95% CI 50%–86%) and 100%, respectively. Progression-free survival at 5 years is 84.4% (95% CI 50%–96%) for patients following GTR compared with 57.1% (95% CI 25%–69%) for patients who achieved a less than GTR (p = 0.088, log-rank test). A high relapse incidence (4 of 6) was observed among patients with myxopapillary ependymoma. Conclusions Gross-total resection is the mainstay of treatment for patients with primary spinal cord ependymoma and may be achieved in about 50% of the patients using modern surgical techniques. Primary adjuvant treatment was commonly used in children with spinal cord ependymoma irrespective of the extent of resection or tumor grade. The impact of adjuvant treatment on progression-free and overall survival has to be investigated in a prospective trial.

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P09.01 case series: meningioma in young adult male

Neuro-Oncology ◽

10.1093/neuonc/noz126.133 ◽

2019 ◽

Vol 21 (Supplement_3) ◽

pp. iii38-iii39

Author(s):

Y Diansari ◽

N Karindang ◽

D Anggraeni

Keyword(s):

Young Adult ◽

Adult Male ◽

Case Series ◽

Intracranial Tumors ◽

Tumor Removal ◽

Who Grade ◽

Young Adult Male ◽

First Case ◽

History Of ◽

Grade Ii

Abstract BACKGROUND Intracranial meningiomas are most common among patients in their fifth to seventh decade of life, typically found in older women. Meningiomas are rare in children and young adults; they represent 1–3% of all intracranial tumors in individuals up to age 20 years and 13.5% of intracranial tumors in the 20–34 age group. Meningioma in young adult male is a rare finding. Management of meningioma is similar between young or older patient. The prognosis is depent on the histology finding and type of resection. This case series was made to report an uncommon case including clinical characteristic and management of meningioma in young adult male. MATERIAL AND METHODS We reported 2 cases of meningioma in young adult male. The first patient was hospitalized on 2016 and diagnosed with atypical meningioma (WHO grade II). The second patient was hospitalized on 2018 and diagnosed with trantitional meningioma (WHO grade I). RESULTS First case: 23 y.o male was hospitalized because of severe headache. His physical exam showed weakness of his left side of the body. There was diplopia that significant for bilateral six nerves palsy. Funduscopy examination revealed papil edema in both eyes correlated with his complained of blurry visions. There was no cranial irradiation from his past medical history. Second case, a male 22 y.o was because of decreased of visual acquity progressively since two month before. There was history of throbbing headache in his left head with mild to moderate intensity. On his neurological examination, there was mild weakness in his right body side. Visual acquity on his right and left eyes were 1/300 and 1/60. Bilateral papil edema was found in funduscopy examination. In his past medical history, there was no history of cranial iradiation. Both cases showed similar initial symptoms consist of headache and others neurological deficit. Radiological examination was performed with result leading to meningioma in convexity area in both cases. Craniotomy tumor removal with gross total resection as simpson grade 2 was achieved in both patients. In the second case pre-operative embolization was performed in order to achieve optimal resection. Histopathology examination confirmed as atypical (WHO grade II) in the first case and trantitional Meningioma (WHO grade I) in second case. Follow up imaging post operation showed no residual mass lesion in both patients. There were no further management in these patients. Both of patients showed improvement in their neurologic function and able to activity without restriction in daily living. Serial imaging examination is planned to observe reccurence of the tumor. CONCLUSION Clinical presentation and management of meningioma in these patients are similar as meningioma commonly. Both of patients showed clinical improvement after craniotomy tumor removal succesfully.

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