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

Background: Meningiomas are slow growing intracranial and intraspinal neoplasms with a tendency to recur locally. WHO grades them as I (benign), II (atypical) and III (anaplastic) in order of their increasing aggressiveness, based on histological parameters and brain parenchymal invasion. Progesterone receptors (PR) are more prevalent amongst the lower grade meningiomas. The objective of this study was to determine the immunohistochemical expression of progesterone receptors in meningiomas of different grades.Material and Methods: A total of 100 cases were selected over a period of 2.5 years. Three to five microns’ thick sections stained with Hematoxylin and Eosin were examined microscopically by a team of two Histopathologists and graded into grades I, II and III, according to 2016 WHO classification criteria. Another section of the original tumor was stained with progesterone receptor antibody using the conventional immunoperoxidase method. Stained slides were than examined by the same team of Histopathologists and declared positive (if nuclear staining was observed in more than 10% of tumor cells) or negative. Statistical analysis was done using SPSS version 21.Results: Out of a total of 100 cases of meningioma, there were 79 cases of benign/typical WHO grade I, 15 cases of atypical/ WHO grade II and 6 cases of anaplastic/ WHO grade III tumor. PR status was positive in 89.8 % (71/79) of grade I meningiomas and 46.6 % (7/15) of grade II/Atypical meningiomas. The 06 cases of Anaplastic/WHO grade III tumors were negative for PR. There was a higher prevalence of Progesterone receptors in female patients (89.8%; 53/59) as compared to male meningioma patients (60.9%; 25/41).Conclusion: We observed a decreased expression of progesterone receptor in higher grades of meningioma in this study. It is an effort to explore conservative treatment options for inoperable lesions, as anti-progesterone therapy may hold a promise as a new treatment option in the near future.

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Major complications from radiotherapy following treatment for atypical meningiomas

Neurosurgical FOCUS ◽

10.3171/2019.3.focus1930 ◽

2019 ◽

Vol 46 (6) ◽

pp. E5 ◽

Cited By ~ 1

Author(s):

Troy Dawley ◽

Zaker Rana ◽

Hussam Abou-Al-Shaar ◽

Anuj Goenka ◽

Michael Schulder

Keyword(s):

Cognitive Decline ◽

Treatment Options ◽

Mass Effect ◽

Postoperative Management ◽

Atypical Meningioma ◽

Normal Brain ◽

Who Grade ◽

Cerebrospinal Fluid Diversion ◽

Major Complications ◽

Grade Ii

OBJECTIVEComplications from radiotherapy (RT), in a primary or adjuvant setting, have overall been described as uncommon, with few detailed descriptions of major complications. The authors present two cases involving significant complications and their management in their review of patients undergoing RT for treatment of atypical meningioma.METHODSThe authors conducted a retrospective review of all patients with pathologically confirmed atypical meningioma (WHO grade II) treated with primary or adjuvant RT from February 2011 through February 2019. They identified two patients with long-term, grade 3 toxicity. The cases of these patients are described in detail.RESULTSTwo patients had major complications associated with postoperative RT. Patients 1 and 2 both were treated with postoperative RT for pathologically confirmed atypical meningioma. Patient 1 experienced worsening behavioral changes, cognitive decline, and hydrocephalus following treatment. This required cerebrospinal fluid diversion. Patient 2 developed radiation necrosis with mass effect and cognitive decline. Neither patient returned to his/her initial post-RT status after steroid therapy, and each remained in need of supportive care. Both patients remained free of tumor progression at 52 and 38 months following treatment.CONCLUSIONSThe postoperative management of patients with atypical meningioma continues to be defined, with questions remaining regarding timing of RT, dose, target delineation, and fractionation. Both of the patients in this study received fractionated RT, which included a greater volume of normal brain than more focal treatment options such as would be required by stereotactic radiosurgery (SRS). Further research is needed to compare SRS and fractionated RT for the management of patients with grade II meningiomas. The more focused nature of SRS may make this a preferred option in certain cases of focal recurrence.

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

European Spine Journal ◽

10.1007/s00586-018-5760-4 ◽

2018 ◽

Vol 28 (2) ◽

pp. 426-433 ◽

Cited By ~ 2

Author(s):

Kazuyoshi Kobayashi ◽

Kei Ando ◽

Fumihiko Kato ◽

Koji Sato ◽

Mitsuhiro Kamiya ◽

...

Keyword(s):

Spinal Cord ◽

Case Series ◽

Who Grade ◽

Grade Ii ◽

Spinal Cord Ependymoma ◽

Mri Changes

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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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Diagnosis and Predictors of Treatment Outcomes in Meningiomas with Atypical or Anaplastic Histology

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v27i2.751 ◽

2018 ◽

Vol 27 (2) ◽

pp. 100-104 ◽

Cited By ~ 1

Author(s):

Gustavo Simiano Jung ◽

Ricardo Ramina ◽

Erasmo Barros Da Silva Jr ◽

Maurício Coelho Neto

Keyword(s):

Treatment Outcomes ◽

Treatment Options ◽

Gross Total Resection ◽

Total Resection ◽

Who Grade ◽

Grade Ii ◽

Anaplastic Histology ◽

Biological Nature

Atypical and anaplastic meningiomas (WHO grade II and III) are uncommon tumors with poorer prognosis than benign meningiomas. They represent a small and heterogeneous subgroup of meningiomas that has more aggressive biological nature and higher frequency of recurrence. Treatment of these tumors remains challenging and recurrence is common even after gross total resection. We report five year experience of an experienced neurosurgical center (INC) reviewing treatment options and predictor of treatment outcomes for malignant meningiomas.

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A basic review on systemic treatment options in WHO grade II-III gliomas

Cancer Treatment Reviews ◽

10.1016/j.ctrv.2020.102124 ◽

2021 ◽

Vol 92 ◽

pp. 102124

Author(s):

Maximilian J. Mair ◽

Marjolein Geurts ◽

Martin J. van den Bent ◽

Anna S. Berghoff

Keyword(s):

Systemic Treatment ◽

Treatment Options ◽

Who Grade ◽

Grade Ii

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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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P04.11 10-Year Institutional Retrospective Case Series of WHO Grade II Gliomas and Correlation of Seizures, Lobar Location, Histopathological Subtype and IDH-1 Mutation (2010–2020)

Neuro-Oncology ◽

10.1093/neuonc/noab180.068 ◽

2021 ◽

Vol 23 (Supplement_2) ◽

pp. ii21-ii21

Author(s):

S Lammy ◽

E Bridgman ◽

H Johnson ◽

A Taylor ◽

A Grivas

Keyword(s):

Case Series ◽

World Health Organisation ◽

World Health ◽

Neurological Deficits ◽

Retrospective Case ◽

Who Grade ◽

Retrospective Case Series ◽

Grade Ii ◽

Who Grade Ii Gliomas ◽

Histopathological Subtype

Abstract BACKGROUND A 10-year retrospective case series was undertaken of all patients who had a tissue diagnosis of a World Health Organisation (WHO) Grade II glioma, i.e. low grade glioma (LGGM), at the Institute of Neurological Sciences (INS) between January 2010 and January 2020 (NB: pre-2016 World Health Organisation classification). The objective was to assess the correlation of World Health Organisation (WHO) Grade II gliomas to seizure symptomology, intracerebral tumour location, histopathological glioma sub-type and molecular markers including isocitrate dehydrogenase-1 (IDH-1) mutation. MATERIAL AND METHODS We extracted data regarding clinical, radiological, histological, molecular discriminators and functional outcomes in patients. The pre-operative symptomology was restricted to seizures, headache and focal neurological deficits. RESULTS 84 patients underwent resection and had a mean age of 42rs (range: 21-77yrs]). Seizures (NB: pre-2017 International League against Epilepsy classification [ILAE]) occurred in 71% and of these 52% were generalised, 37% partial and 11% mixed. 31% had ongoing seizures postoperatively. Headache occurred in 38% (of these 50% had headache and seizures). Focal neurological deficits (FNDs) occurred in 21% (of these 61% had FNDs and seizures). Seizure, as an isolated pre-operative symptom occurred in 48% patients compared to isolated headache in 10% and isolated FNDs in 4%. Anatomically, 58% of lesions were frontal. The seizure spatial frequency was 60% frontal. Histologically, 60% were diffuse astrocytomas and 40% oligodendrogliomas. Furthermore, 71% of oligodendrogliomas and 49% of diffuse astrocytomas had a frontal location. IDH-1 mutation occurred in 80% patients (75% of these had seizures) and of these 66% were frontal. CONCLUSIONS Our analysis confirms a correlation between incidence of seizures, frontal lobar location, histopathological subtype and IDH-1 mutations (p = <0.05

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