Year : 2009 | Volume
: 5 | Issue : 4 | Page : 324--327
An unusual case of clear cell meningioma
Prabal Deb1, Subramanya G.S Datta2,
1 Department of Pathology, Armed Forces Medical College, Pune, India
2 Command Hospital, Udhampur, Jammu & Kashmir, India
Department of Pathology, Armed Forces Medical College, Pune - 411 040
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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Deb P, Datta SG. An unusual case of clear cell meningioma.J Can Res Ther 2009;5:324-327
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Deb P, Datta SG. An unusual case of clear cell meningioma. J Can Res Ther [serial online] 2009 [cited 2021 Jul 24 ];5:324-327
Available from: https://www.cancerjournal.net/text.asp?2009/5/4/324/59902
Clear-cell meningioma (CCM) is an uncommon, aggressive variant of meningiomas usually affecting younger females with a predilection for spinal or cerebellopontine locations. ,,,
We report a rare case of recurrent supratentorial CCM in an adult male, who was operated for an occipital tumor (possibly intra-axial) around 10 years back, followed by extra-axial recurrence. Recurrent tumor had an acute presentation with features of raised intracranial pressure, probably due to intratumoral bleed.
A 58-year-old man presented with sudden severe headache and vomiting, followed by disturbances of speech and vision. On examination, he was disoriented and also had nominal aphasia, recent memory deficits, right homonymous hemianopia, and early papilledema. However, vitals were within normal limits. His Kernopfsky Performance Score (KPS) was 80. There were no other neurological deficits.
Past history revealed occurrence of an intra-axial tumor in the left occipital lobe, around 10 years back, which was managed at a different center. Histopathology revealed an oligodendroglioma, for which he was given radiotherapy. Postoperative follow-up records showed that he was asymptomatic during the following 2 years, and was subsequently lost to follow-up. Old computerized tomography (CT) scan images were lost by the patient and not available.
A contrast-enhanced CT (CECT) scan head showed a left occipital craniotomy defect with a 6.3 × 4.2 cm left parieto-occipital lesion, broad based on bone, at the anterior craniotomy flap edge [Figure 1]A. There was no bony hyperostosis or destruction. The lesion was heterogeneously hyperdense on plain CT without contrast enhancement. There was perilesional edema and mass effect on the ipsilateral lateral ventricle. An area of gliosis was present in the occipital pole at the site of the previous tumor. On MRI, the lesion was heterogeneously hyperintense on T1W [T1 weighted-[Figure 1]B] and T2W [T2 weighted-[Figure 1]C] images, and was not enhancing on contrast. The imaging features were suggestive of an extra-axial lesion, possibly a meningioma, with probable intratumoral bleed.
A wide left parieto-occipital craniotomy was done encompassing the old incision. A free bone flap was raised. On dural opening, it was evident that the tumor was totally extra-axial, well encapsulated, and vascular, with large areas of hemorrhage [Figure 2]. The tumor was excised completely along with dura [Figure 3]A.
Five-micron-thick sections were cut from routine formalin-fixed, paraffin-embedded tissue, and stained by hematoxylin and eosin (H and E) stain, and also PAS stain (Periodic Acid Schiff for glycogen). Immunohistochemistry (IHC) was done using streptavidin-biotin immunoperoxidase technique (LSAB Kit, M/s Dakopatts, Denmark) using monoclonal antibodies to glial fibrillary acidic protein (GFAP), vimentin, S-100 protein, and epithelial membrane antigen (EMA), while the proliferation index was evaluated using an MIB-1 antibody (all antibodies were prediluted and obtained from M/s BioGenex, USA).
Microscopic examination showed a dural-based tumor with sheets of PAS-positive glycogenated polygonal cells with abundant clear cytoplasm and round, uniform, bland nuclei. Focal vague whorl formation with meningothelial cell clusters and psammomatous calcification were diagnostic. Clusters of hemosiderin-laden macrophages were present. There was no mitosis, necrosis, or vascular proliferation [Figure 3]B-H.
A review of the initial biopsy showed an intraparenchymal tumor with similar histomorphology, but lacking any dural attachment [Figure 4]A, B.
Tumor cells from both the biopsies were immunoreactive to EMA, vimentin and S-100 protein. MIB-1 labeling index was Follow-up
Postoperative CECT on the second postoperative day showed a complete excision of tumor [Figure 1]D. He has been on regular follow-up for past 1.5 years. At the last review, he was asymptomatic, ambulant, with normal speech except for some residual right-sided visual loss.
CCM is a rare entity, potentially aggressive in that it may recur, spread locally, and even metastasize despite its bland histological appearance.  Zorludemir et al.  first described this neoplasm in 1995 and since then, less than 50 cases have been reported. It was subsequently listed as a distinct entity in the current WHO classification of brain tumors in 2000.  Most cases were single case reports, with more than 50% being reported by Zorludemir et al.,  Pimentel et al.,  and Jain et al.  We hereby report an unusual case of supratentorial CCM in a middle-aged male with multiple features uncharacteristic of most CCMs reported in the literature.
Most patients of CCM are young, usually in the first three decades of life, ,,, although occasional cases in older patients, like the present case, have also been reported. ,, A slight female predominance has been noted.
CCM have most commonly been observed in the spinal intradural (lumbar and thoracic) and posterior fossa (cerebellopontine angle) locations, with few in the fourth ventricle, tentorium-clinoid, base of the skull, and foramen magnum. ,,, The supratentorial region getting affected ,,,,, is uncommon, while intraparenchymal location is rare.
Intraparenchymal meningioma without any dural attachment generally arises from arachnoid cap cells in the Virchow-Robin spaces along the cerebral vasculature or from the pia mater within the brain sulcus. , Most intraparenchymal meningiomas tend to be supratentorial in location with predilection for the frontotemporal , and brainstem regions. Uncommon sites include the deep sylvian, subcortical, pineal, and suprasellar areas.  Except for intraventricular ones, most intraparenchymal meningiomas are fibroblastic type,  and affect male patients, with an average age 20 years less than usual meningiomas.
Prior to the acceptance of CCM as a distinct variant of meningioma, intraparenchymal tumors with clear cell morphology were often reported as metastatic renal cell carcinoma, oligodendroglioma, hemangioblastoma, and clear cell ependymoma ,, especially so when diagnoses were based solely on histomorphology. The absence of IHC facility during the initial biopsy (in 1997), compounded with lack of sufficient data pertaining to CCM possibly attributed to misinterpretation of initial tumor as an oligodendroglioma. This is not unlike that observed by other centers, and it further reiterates the importance of IHC in the diagnostic workup of neurosurgical specimen.
The biological behavior of CCM may be inordinately aggressive, despite its benign histological appearance,  and may display inconsistent correlation with MIB-1 proliferation. Zorludemir  noted high MIB-1 LI (range 3.3-25.7%; mean 13.3%) with 61% recurrence, while, Jain series  noted 22% recurrence despite low MIB-1 LI, which was similar to the present case. In menigioma, factors with prognostic significance generally include histological subtype, tumor grade, MIB-1 proliferation, mitotic index, brain invasion, and extent of surgical resection.  Zorludemir et al. in their series found a recurrence rate of 61%, but failed to note any definite correlation between tumor recurrence and factors as mitotic activity, PCNA proliferation indices, percent S-phase determination, or DNA ploidy status. However, Pimentel et al.  noted that CCMs which recurred were in the intracranial location, and ones with subtotal resection, while Lee et al.  documented a case of CCM, in which initial MR imaging showed leptomeningeal enhancement that had progressed into entire subarachnoid space after surgical resection of the primary tumor.
Currently, molecular genetics and karyotypic studies have also found consistent correlation between meningioma recurrence and loss of heterozygosity (LOH) 22q, 1p, and 14q.  In order to gain insight into the pathophysiology behind the aggressive behavior of CCM, it is imperative to have a detailed cytogenetic evaluation, especially in a large series, prior to attributing aggressiveness in CCM to any definite factor.
Treatment of choice in CCM is surgical resection, with radiosurgery and radiotherapy being reserved for recurrent cases. To the best of our knowledge, data pertaining to recurrence of intraparenchymal CCM following radiotherapy of initial tumor, as seen in the present case, are not available. However, Lee et al. document a case who had diffuse leptomeningeal seeding after initial surgery. He was managed with radiotherapy, with perceptible improvement in the clinicoradiological profile. 
The association of meningiomas with intratumoral, peritumoral, or subdural hemorrhage is a rare phenomenon. A review by Bosnjak et al.  found association of fibrous meningiomas in cases 70 years of age to have intratumoral bleed. Interestingly, none of the CCMs reported till date has described this feature.
To conclude, this report documents a rare variant of meningioma with multiple unusual characteristics, namely elderly age; supratentorial location; intra-axial tumor with extra-axial recurrence; intratumoral bleed; and recurrence despite low MIB-1LI. To the best of our knowledge, this is the first case of CCM associated with multiple unusual features being reported in the literature. It also highlights the need for incorporating immunohistochemistry to supplement conventional histomorphology in the routine diagnostic workup of intraparenchymal clear-cell tumors, which includes a wide spectrum of conditions ranging from oligodendroglioma, hemangioblastoma, clear-cell ependymoma, extraventricular neurocytoma, clear-cell meningiomas, and metastatic tumors, each having a distinctive biological behavior, therapeutic protocol, and prognostic outcome.
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