Abstract
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.
Highlights
Clear cell meningioma 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]
Pleomorphism, nucleolar prominence, and focal micro-necrosis were observed in the recurrences [Figure 1(B,C)]
Expression of the glial fibrillary acidic protein was negative in both the original tumour and the two recurrences
Summary
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]. The genesis of meningioma is associated with loss of genetic material on chromosome 22. Monosomy of that chromosome is the most common genetic alteration in meningioma and is linked to mutations of the NF2 gene, located in 22q12.2, which codes for the tumour suppressor protein merlin[6]. Losses of 1p and alterations in chromosome 14 are present in many atypical meningiomas and have been associated with tumour progression[7,8]. Histologic and genetic studies of the primary tumour and its recurrences showed changes in NF2 and other tumour suppressor genes. We analyzed the evolution and implications of the various genetic aberrations present in this neoplasm with the aim of elucidating the genetic changes involved in the progression of ccm
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