Frontotemporal clear cell meningioma. Report of 3 cases

Clear-cell meningioma (CCM) is an uncommon, aggressive variant of meningioma, usually affecting younger females and having predilection for infratentorial locations. We present a rare case of recurrent supratentorial CCM in a 58-year-old male. Ten years back, he had an intra-axial tumor in the left occipital lobe, which was managed by surgical excision and radiotherapy. Currently, the patient presented with sudden severe headache along with speech and vision disturbances. Neuroimaging revealed an extra-axial parietooccipital tumor, with intratumoural bleed. Histopathology of both tumors showed features of CCM, immunopositive for epithelial membrane antigen (EMA) and vimentin. This case illustrates multiple unusual features of a rare variant of meningioma in the form of affection of an adult age group, supratentorial location, recurrence, and intratumoral bleed. It also highlights the importance of incorporating immunohistochemistry in the diagnostic workup, to exclude CCM mimics, each having distinctive biological behavior, and prognostic outcome, and warranting different therapeutic protocols.

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“…[1][2][3][4] The supratentorial region getting affected [1][2][3][4][5][6] is uncommon, while intraparenchymal location is rare.…”

Section: Discussionmentioning

confidence: 99%

An unusual case of clear cell meningioma

Deb

Datta

2009

J Can Res Ther

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“…In contrast to ordinary meningiomas, the recurrence rate in CCM is 46–80% 2,3 . Intraspinal CCM also has an aggressive clinical course.…”

Section: Discussionmentioning

confidence: 99%

“…CCM are classified as grade II in the current World Health Organization (WHO) tumor grading system 1 . They represent only 0.2% of all meningiomas and they usually occur in young patients 2,3 . Their more frequent localization is at the cerebellopontaine region and at the spinal cord 2–4 .…”

Section: Introductionmentioning

confidence: 99%

“…They represent only 0.2% of all meningiomas and they usually occur in young patients 2,3 . Their more frequent localization is at the cerebellopontaine region and at the spinal cord 2–4 . CCM may behave aggressively with local recurrence and cerebrospinal fluid metastasis, and often require multiple reoperations or radiation therapy 3,4 .…”

Section: Introductionmentioning

confidence: 99%

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Clear cell meningioma with histologically aggressive appearance and clinically aggressive behavior: A case report

et al. 2011

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Clear cell meningioma (CCM) is an uncommon variant of meningioma, corresponding to WHO grade II. We present a case of CCM with histologically aggressive appearance and clinically aggressive behavior. The tumor demonstrated rapid regrowth and brain metastasis. The histological progression from the ordinal CCM to the atypical area and higher MIB-1 index was observed. We assume that the short time of recurrence and metastasis may result from atypical histological features in our case. If the CCM has a histologically aggressive appearance as in our case, we suggest that postoperative adjuvant radiotherapy should be performed despite total resection of the tumor.

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“…Clear cell meningioma (ccm) classified as grade 2 is a rare, potentially aggressive entity that represents about 0.2% of all meningiomas, with most cases being described in young women [1][2][3][4][5] . Surgical resection and postoperative radiotherapy are the standard treatment for primary disease and local recurrences.…”

Section: Introductionmentioning

confidence: 99%

Molecular Progression in Unusual Recurrent Non-Pediatric Intracranial Clear Cell Meningioma

et al. 2017

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We report a case of a recurrent clear cell meningioma (ccm) in the frontal lobe of the brain of a 67-year-old man. The patient developed three recurrences: at 3, 10, and 12 years after his initial surgery. Histopathology observations revealed a grade 2 ccm with positivity for vimentin and epithelial membrane antigen. Expression of E-cadherin was positive only in the primary tumour and in the first available recurrence. Fluorescence in situ hybridization analyses demonstrated 1p and 14q deletions within the last recurrence. Multiplex ligation-dependent probe amplification studies revealed a heterozygous partial NF2 gene deletion, which progressed to total loss in the last recurrence. The last recurrence showed homozygous deletions in CDKN2A and CDKN2B. The RASSF1 gene was hypermethylated during tumour evolution.In this report, we show the genetic alterations of a primary ccm and its recurrences to elucidate their relationships with the changes involved in the progression of this rare neoplasm.

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Frontotemporal clear cell meningioma. Report of 3 cases

Cited by 19 publications

References 24 publications

An unusual case of clear cell meningioma

An unusual case of clear cell meningioma

Clear cell meningioma with histologically aggressive appearance and clinically aggressive behavior: A case report

Molecular Progression in Unusual Recurrent Non-Pediatric Intracranial Clear Cell Meningioma

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