Clear cell meningioma: case report and literature review

Clear cell meningioma: case report and literature review

264 Kuzeyli et al. 1 Department of Neurosurgery, Karadeniz Technical University, School of Medicine, Trabzon 61080, Turkey, 2Department of Pathology,...

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264 Kuzeyli et al. 1

Department of Neurosurgery, Karadeniz Technical University, School of Medicine, Trabzon 61080, Turkey, 2Department of Pathology, Karadeniz Technical University, School of Medicine, Trabzon 61080, Turkey, 3 Department of Neurosurgery, Celal Bayar University, School of Medicine Trabzon 61080, Turkey

Summary The clear cell meningioma is rare and a recently described histologic variant of meningioma. The most interesting aspect of clear cell meningioma is the high recurrence rate and agressiveness. Until now 17 intracranial clear cell meningioma cases had been reported in the English language literature. We present 2 new cases of clear cell meningioma which is discussed with the relevant literature. ª 2003 Elsevier Science Ltd. All rights reserved. Journal of Clinical Neuroscience (2003) 10(2), 264–266 0967-5868/03/$ - see front matter ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(02)00287-4

Fig. 2 Photomicrograph of the ependymoma showing a moderately cellular tumour comprising ependymal cells oriented around lumina forming ependymal rosettes. H&E, original magnification 160.

Due to the widespread extensions, the treatment options in such a case are limited. Histological confirmation of the lesion by biopsy, craniospinal irradiation and chemotherapy appears to be a rational sequence.3;9–11 Introduction of a cerebrospinal fluid ventriculoperitoneal shunt for obstructive hydrocephalus is debatable considering the high possibility of abdominal metastasis.12

Keywords: clear cell, meningioma, magnetic resonance imaging, multiple Received 15 February 2002 Accepted 12 April 2002 € niversitesi, Tıp Correspondence to: Dr Kayhan Kuzeylı_, Karadeniz Teknik U €ltesi, Beyin ve Sinir Cerrahi Anabilim D. 61080 Trabzon, Turkey. Faku Tel.: +90-462-377-54-54; E-mail: [email protected]

INTRODUCTION REFERENCES 1. Bailey P. A study of tumors arising from ependymal cells. Arch Neurol Psychiatry (Chic) 1924; 11: 1–27. 2. Centeno RS, Lee AA, Winter J, Barba D. Supratentorial ependymomas. Neuroimaging and clinicopathological correlation. J Neurosurg 1986; 64: 209–216. 3. Oppenheim JS, Strauss RC, Mormino J, Sachdev VP, Rothman AS. Ependymomas of the third ventricle. Neurosurgery 1994; 34: 350–352. 4. Ernestus R-I. Intracranial ependymomas. In: Palmer JD (ed) Neurosurgery 96 – Manual of Neurosurgery. New York: Churchill Livingstone, 1996, pp. 242–247. 5. Miller C. The ultrastructure of the conus medullaris and filum terminale. J Comp Neurol 1968; 132: 547–566. 6. Fokes EC, Earle KM. Ependymomas: clinical and pathologic aspects. J Neurosurg 1969; 30: 585–594. 7. de Tribolet N, Deruaz JP, Zander E. Cerebrospinal fluid spread of ependymomas. Schweiz Arch Neurol Neurochir Psychiatr 1978; 123: 83–91. 8. Ernestus R-I, Wilcke O. Spinal metastases of intracranial ependymomas. Four case reports and review of literature. Neurosurg Rev 1990; 13: 147–154. 9. Dohrmann GJ, Farwell JR, Flannery JT. Ependymomas and ependymoblastomas in children. J Neurosurg 1976; 45: 273–283. 10. Healy EA, Barnes PD et al. The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 1991; 28: 666–671. 11. Zuccaro G, Sosa F, Cuccia V, Lubieniecky F, Monges J. Lateral ventricle tumors in children: a series of 54 cases. Childs Nerv Syst 1999; 15: 774–785. 12. Oberbauer RW, Tritthart H, Ascher PW, Walter GF, Becker H. Shunt metastases in posterior fossa tumors. Neuropadiatrie 1979; 10: 296–300.

Clear cell meningioma: case report and literature review  rul C Kayhan Kuzeylı_1, Ertug ¸ akir1, Haydar Usul1, 1 € kalp Karaarslan , A. Kadir Reı_s2, Cu € neyt Temı_z3, Go 1 € leyman Baykal Su Journal of Clinical Neuroscience (2003) 10(2)

Clear cell meningioma (CCM) is described in the last World Health Organisation (WHO) classification as a variant of the meningioma.1–3 Although it is a benign tumour, recurrence and metastasis are very common in CCM.1–6 Seventeen cases of CCM have been previously reported.1–3;6–11 Here we present an additional 2 new cases. In one of our cases which has multiple meningiomas, CCM was found with other accompanying types of meningiomas. This is the first reported case of a CCM in a patient with multiple meningiomas.

CASE REPORT Case #1 A 52 year old female was admitted with complaints of severe headache, nausea, vomiting and visual disturbance. Neurologic examination revealed slight impairment of cognitive functions, a right papilloedema and slight left hemiparesis. Laboratory examinations were within normal limits. Magnetic resonance imaging (MRI) showed multiple intracranial masses of 5– 50 mm in size. The masses homogeneously enhanced after intravenous contrast injection (Fig. 1A). The mass at the right frontal region caused peritumoural oedema and midline shift with dilatation of the left lateral ventricle (Fig. 1B). Antioedema medication was started and an emergency right frontoparietal craniotomy was performed. When the dura was opened it was seen that the tumours were invading the adjacent dura and convexity. Five small tumours of 5–15 mm in size were excised, along with 1 large frontoparietal tumour of histopathological diagnosis of meningoendothelial meningioma. Fifteen days later a second operation was performed. At the second operation a left frontal v-p shunt was inserted and a total excision of the large mass at the right occipital region was achieved. The histopathological diagnosis of the second mass ª 2003 Elsevier Science Ltd. All rights reserved.

Clear cell meningioma

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Fig. 1 (A) Contrast enhanced sagittal MR image showing the homogeneously contrast enhancing multiple masses. Note the frontal peritumoral edema and mass effect. (B) Contrast enhanced axial MRI showing the same lesions causing midline shift and dilatation of the left lateral ventricle. (C) Photomicrograph showing whirl formation of the meningeal cells with clear cytoplasms. Note the centric or epicentric positions of the nuclei within the clear cytoplasms (H & E  100). (D) The tumour cells exhibit Epithelial Membrane Antigen (EMA) immunreactivity. (Immunoperoxidase EMA). Table 1

Summary of all patients with intracranial clear cell meningiomas reported in the English literature

Authors and year

No. of cases

Shiraishi, 1991 Kakita et al., 1995 Zorludemir et al., 1995 Shih et al., 1996 Imlay et al., 1998 Primentel et al., 1998

1 1 7 1 1 3

Teo et al., 1998 Lee et al., 2000 Heth et al., 2000 Kuzeylı_ et al., 2001

1 1 1 2

Sex, age (yrs) F, 49 F, 64 Age range 11–82 M, 12 F, 21 F, 24 M, 61 M, 19 F, 2 M, 17 F, 31 F, 52 M, 50

Location

Surgery

Frontal lobe Frontal lobe 2 Supra tentorial, 5 Posterior fossa CPA Posterior fossa Tentorium-clinoid Convexity Basal skull Intraparenchymal brainstem Parietal lobe Foramen magnum Occipital-lobe Temporal-lobe

GTR GTR 5 GTR; 2 STR GTR STR STR GTR STR STR GTR STR TR STR

CPA, cerebellopontine angle; GTR, gross-total resection; TR, total resection; STR, subtotal resection.

was CCM (Fig. 1C,D). Twenty days later a third operation was performed due to an increase in her left hemiparesis. Multiple masses in the right parietal parasaggital region were excised. The histopathological diagnosis was psammomatous meningioma. After 6 months of follow-up she is in good condition and neurological examination is within normal limits except for a slight left hemiparesis.

pilloedema, right hemiparesis of 2/5 strength and slight motor aphasia. MRI showed a large homogeneously contrast enhancing mass which filled the left temporal fossa (Fig. 2A). A preoperative diagnosis of a meningioma was made. A right frontotemporal craniotomy was performed. A subtotal excison of the mass which was encapsulating the internal carotid artery, middle cerebral artery, and its branches was achieved. The histopathological diagnosis was CCM (Fig. 2B). The patient died 45 days later due to congestive heart failure.

Case #2 A 50 year old male was admitted with severe headache, nausea and vomiting, right hemiparesis and speech disturbances. Physical examination revealed hypertension (200/110 mmHg) and congestive heart failure. Neurological examination revealed left paª 2003 Elsevier Science Ltd. All rights reserved.

DISCUSSION Meningiomas are relatively common intracranial tumours and are usually benign. Clear cell meningioma is a recently described histologic variant of meningioma.1–3;5;8 Journal of Clinical Neuroscience (2003) 10(2)

266 C ¸ akir et al.

Seventeen cases have been reported in the English language literature, 7 of the cases have been subtotally and the remaining 10 cases have been totally resected1–3;6–11 (Table 1). Our patient #1 showed multiple meningiomas with different histopathological types and one of these meningiomas was a CCM. A total resection of the tumour was achieved. No CCM was reported in the literature in a patient with multiple meningiomas. This makes our case more interesting. The patient showed no recurrence until that time (7 months). It is obvious that a longer follow up is necessary for this case. Because CCMs have a recurrence time of 4–23 months. The tumour in case #2 was resected subtotally and the patient died 45 days later. When compared with other meningioma operations, neither macroscopic nor surgical differences could be detected in both of the operations. The goal of the treatment is to achieve radical surgical removal as much as possible. Radiotherapy and radio-surgery might be important for patients who are not good candidates for reoperation. CONCLUSION Although the differential diagnosis of CCM with the other clear cell tumours of the CNS is possible, the diagnosis of CCM might be kept in mind in meningioma cases and close follow-up is required due to the high risk of recurrence. REFERENCES

Fig. 2 (A) Contrast enhanced axial MRI showing a homogeneously contrast enhanced left temporal mass. (B) Photomicrograph showing meningeal cells with a clear cytoplasm (H & E 200).

CCMs are usually seen in younger patients and have a predilection for canalis spinalis.2;3;6 CCM are potentially aggressive and may recur, spread locally and even metastasis.2;4;6;12 All of these features make them clinically aggressive.13 Cytopathologically CCM have a whorled syncytial architecture and spindled to polygonal, bland- appearing nuclei.3;7;13;14 The immunohistochemical features included epithelial membrane antigen and vimentin positive cytoplasm.2;11;15 Ultrastructurally, tumour cells showed conspicuous inter digitations of their plasma membranes with frequent junctional complexes and contained numerous glygocen granules in the cytoplasm and its process.4;7;8;13–15 The differential diagnosis of CCMs include intracranial renal cell carcinoma metastases, hemangioblastoma, pleomorphic xanthoastrocytoma, lipid-rich glioblastoma, and clear cell ependymoma.2;6;14 The tumour is isointense with the parenchyma on T1 and T2 weighted MRI. The MRI features of CCM are not much different from the other meningiomas. On T1 and T2 weighted MRI the tumour is isointense with the parenchyma and homogeneously enhances after gadolinium injection.13 Journal of Clinical Neuroscience (2003) 10(2)

1. Heth JA. Intraspinal familial clear cell meningioma in a mother and child. Case report. J Neurosurg Spine 2000; 93: 317–321. 2. Lee W. MR imaging features of clear cell meningioma with diffuse leptomeningeal seeding. AJNR Am J Neuroradiol 2000; 21: 130–132. 3. Pimentel J. Clear cell meningioma variant and clinical aggressiveness. Clin Neuropathol 1998; 17: 141–146. 4. Dubais A. Clear cell carcinoma of the caudoequina. Neuroradiology 1998; 40(11): 743–747. 5. Matsui H. Multifocal clear cell meningioma in the spine: a case report. Neurosurg Rev 1998; 21: 171–173. 6. Shil DF. Clear cell meningioma: a case report. Chung Hua I Hsueh Tsa Chih 1996; 57: 452–456. 7. Imlay SP. Clear Cell meningioma: diagnosis by fine-needle aspiration biopsy. Diagn Cytopathol 1998; 18: 131–136. 8. Kakita A. Clear cell variants of intracranial tumors: meningioma and epandymoma. Brain Tumor Pathol 1995; 12: 111–116. 9. Shiraishi K. Glycogen-rich meningioma. Case report and short review. Neurosurg Rev 1991; 14: 61–64. 10. Teo JG. Intraparenchymal clear cell meningioma of the brain stem in a 2-year-old child. Case report and literature review. Pediatr Neurosurg 1998; 28: 27–30, abst. 11. Zorludemir S. Clear cell meningioma. A clinicopathologic study of potentially agressive variant of meningioma. Am J Surg Pathol 1995; 19: 493–505. 12. Prinz M. Clear cell meningioma:report of a spinal case. Gen diagn Pathol 1996; 141: 261–267. 13. Ho T. A case of clear cell meningioma orginating from the cerebellar tentorium. No shinkei Geka 1998; 26: 265–270, abs. 14. G€okden M. Clear cell neoplasms and pseudoneoplastic lesions of the central nervous system. Semin Diagn Pathol 1997; 14: 253–269. 15. Kubota T. Clear cell (glycogen-rich) meningioma with special reference to spherical collagen deposits. Noshuyo Byori 1995; 12(1): 53–60.

Solitary plasmacytoma with intracranial intraorbital and, paranasal sinus extension € kalp Karaarslan, Haydar Usul, Ertugrul C ¸ akir, Go € Suleyman Baykal, Erhan Arslan ª 2003 Elsevier Science Ltd. All rights reserved.