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Intracranial clear cell meningioma: a clinicopathologic study of 15 cases

  • Clinical Article
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Abstract

Object

Clear cell meningioma (CCM) is a rare histological variant of meningioma. CCM has a high recurrence rate and aggressiveness. In this study, we reviewed our experience in the treatment of the lesion.

Methods

Here we present a series of 15 patients with intracranial CCM. The clinical data were retrieved from the records of our Neurosurgery Department and the patients’ prognoses were attained by clinic service and telephone. Immunohistochemistry for epithelial membrane antigen (EMA), vimentin, glial fibrillary acidic protein (GFAP), CD10, and S-100 was done, and the MIB-1 labeling index was calculated in all cases.

Results

The 15 patients included eight males and seven females; the mean age was 34.8 years. The most frequent initial symptoms were headache and hearing loss. The most common location was the cerebellopontine angle (CPA) zone. Eleven patients had total removal and four patients underwent subtotal removal. Histological features of atypia were present in different proportions, from 6.7% to 100%, and six cases accorded with atypia. Three tumors showed brain invasion. EMA and vimentin were 100% positive, and CD10 was 100% negative. GFAP was 87% negative and S-100 was 93% negative. The mean follow-up period was 36.7 months. Three patients with brain invasion all recurred and five cases with atypia recurred. In 11 patients with total removal, six patients recurred. In four patients with subtotal removal, three patients recurred. Kaplan-Maier analysis showed that incomplete surgical resection was significantly associated with recurrence (p = 0.001). The MIB-1 labeling index for recurrence was 5.7 ± 2.7% versus 2.8 ± 1.5% for no recurrence (p = 0.036).

Conclusions

CCM is a rare subtype of meningioma, with a tendency to present in younger patients and a propensity to recur. Immunohistochemistry plays a vital role in differentiating CCM from other tumors. Brain invasion, atypia and MIB-1 labeling index are likely to predict the recurrence. The extent of resection might be connected with the prognosis.

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Acknowledgements

This work was supported by grants from the Project of Fudan University Science Foundation (no. BH009132).

Conflicts of interest

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Authors and Affiliations

  1. Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai, 200040, China

    Hong Chen & Yin Wang

  2. Department of Neurosurgery, Putuo District Center Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, 200062, China

    Xue-Min Li

  3. Institute of Antibiotics, Huashan Hospital, Fudan University, Shanghai, 200040, China

    Yuan-Cheng Chen

  4. Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, 200040, China

    Jin-Song Wu, Jian Xu, Ping Zhong, Cheng-Chuan Jiang & Xiao-Qiang Wang

  5. Department of Radiology, Huashan Hospital, Fudan University, Shanghai, 200040, China

    Ya-Fang Dou

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  1. Hong Chen
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Correspondence to Cheng-Chuan Jiang or Xiao-Qiang Wang.

Additional information

Comment

An important part of neuro-oncology group practice is the recognition of unusual to very rare CNS tumors and the tailoring of optimal therapy and follow-up for their carriers—based on more or less scarce published data. The trouble of identifying and analyzing series of rare CNS tumours from the last 30 years, i.e., the CT period, should be supported in the era of genomics. Never trust the previous histologic diagnosis but have it re-evaluated by your neuropathologist—more than one if in doubt.

In the authors’ hospital in Shanghai, a staggering number of 5,423 intracranial meningiomas were diagnosed in a 10-year period, which allowed the compilation of the largest series of intracranial clear cell meningioma (CCM), a rare (0.28%) WHO grade II subtype of meningiomas. The authors, importantly, solidify the clinical spectrum of intracranial CCMs:

1. Younger average age at diagnosis, some 30 years.

2. Totally different intracranial distribution with the petroclival and CPA regions overrepresented.

3. MRI and CT not suggestive of subtype diagnosis.

4. Immunostaining helpful in the differentiation from other primary and metastatic clear cell tumors, such as microcystic meningioma, central neurocytoma, clear cell ependymoma, oligodendroglioma or renal clear cell carcinoma.

5. Recurrence rate of some 60% after seemingly complete removal.

6. Time to recurrence some 4 years.

So, regular MRI controls are mandatory, in our practice at 12-month intervals, as for other grade II meningiomas. But no one has produced convincing data on the impact of local or stereotactic radiotherapy on the recurrence/regrowth rate of CCMs.

Juha E Jääskeläinen

Kuopio, Finland

This is an interesting paper and it seems to be the largest series of clear cell meningiomas reported in the literature. The authors present 15 cases of this rare meningioma variant, which is associated with a more aggressive behaviour, including frequent recurrence and occasional cerebrospinal fluid seeding despite a bland cytology in most cases. They are different from other subtypes of meningiomas on clinical and histopathological manifestations, i.e they are composed of sheets of polygonal cells with clear cytoplasm and perivascular hyalinization without whorls formation and psammoma bodies. As stressed in this paper, it could be hard to diagnose them based only on hematoxylin and eosin examinations, and a definite diagnosis needs other specific immunohistochemical stains to permit their distinction from other subtypes of meningiomas with less aggressive behaviour.

Domenico d’Avella

Marina Paola Gardiman

Padova, Italy

H. Chen and X.-M. Li contributed equally to this study

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Chen, H., Li, XM., Chen, YC. et al. Intracranial clear cell meningioma: a clinicopathologic study of 15 cases. Acta Neurochir 153, 1769–1780 (2011). https://doi.org/10.1007/s00701-011-1052-z

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  • DOI: https://doi.org/10.1007/s00701-011-1052-z

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