Intramedullary meningioma: Case report and review of the literature

Intramedullary meningioma: Case report and review of the literature

42 Surg Neurol 1992;37:42-5 Intramedullary Meningioma: Case Report and Review of the Literature Maurizio Salvati, M.D., Marco Artico, M.D., Pierpaol...

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Surg Neurol 1992;37:42-5

Intramedullary Meningioma: Case Report and Review of the Literature Maurizio Salvati, M.D., Marco Artico, M.D., Pierpaolo Lunardi, M.D., and Franco Maria Gagliardi, M.D. Department of Neurological Sciences-Neurosurgery, "La Sapienza" University of Rome, Rome, Italy

Salvati M, Artico M, Lunardi P, Gagliardi FM. Intramedullary meningioma: case report and review of the literature. Surg Neurol 1992;37:42-5. Intramedullary meningioma is a rarely reported clinical entity. As far as we know, only three cases have been reported to date. We describe a further case at the cervical level and review the few published cases.

thesia to temperature and touch and pain up to the C-4 level. Plain x-ray films of the cervical column done elsewhere were unremarkable. Myelography and computed tomography (CT) myelography revealed complete block at C-2. Magnetic resonance imaging (MRI) (Figure 1) showed an intramedullary space-occupying lesion from C-2 to C-4.

KEY WORDS: Intramedullary tumors; Meningiomas; Spinal meningiomas Operation

Meningiomas are among the more common ( 1 0 . 8 % 37.5%) tumors of the spinal canal [2-5,7,8,10]. They occur almost as frequently as neurinomas of this site, except in the Chinese series of Cheng [1] and WenQing et al [11], who found neurinomas to be far more frequent. Spinal meningiomas have a predilection for the thoracic segment of the spine [2,3,5,7,8,10,12], affecting the cervical or lumbar segments more rarely. They are preferentially subdural-extramedullary [2,3,5,7,8,10, 12]. Only three cases of a complete intramedullary meningioma have been reported to date [5,6]; this rare type raises arduous problems of pathogenesis. We report a further case. Case Report

A 67-year-old woman reported a 10-year history of burning pain in the cervicothoracic spine radiating to the upper limbs. For some years, all her limb movements had been awkward and for the preceding 8 months she had experienced sphincter trouble, chiefly retention. Neurological examination showed spastic tetraparesis with increased tendon reflexes and hypoesAddress reprint requests to." Maurizio Salvati, M.D., Via Cardinal Agliardi 15, 00165 Rome, Italy. Received April 8, 1991; acceptedJune 11, 199l.

© 1992 by M. Salvatiet ai.

Laminectomy from C-2 to C-4, followed by opening of the dural sac and cautious exploration of the subarachnoid space, revealed a pale, swollen spinal cord. Posterior myelotomy exposed a pinkish-gray lesion of rubbery consistency that, with the aid of a Cavitron ultrasound surgical aspirator (CUSA), operating microscope (OM), and somatosensory evoked potentials (SEPs), was totally removed (Figure 2). Histological examination (Figure 3) showed the tumor to be a fibroblastic meningioma. After an uneventful postoperative course, the patient was discharged in good general health with slowly improving motor status. She was seen repeatedly and, at 12-month follow-up, the tetraparesis was significantly improved. Discussion Meningioma wholly within the spinal cord has been reported only in three patients [5,6]. T h e r e may be some doubt about case 2 of Pansini and Conti [6], in which the meningioma emerged on the surface and thus might have originated from the pia arachnoid. O f the four patients, including our own, two were men and two were women. Their mean age of 53.5 years corresponds to the preference of spinal meningioma for the fifth, sixth, and seventh decades of life [2,3,5,7,8,10,12]. The cervical level was affected in all four cases [5,6]. This contrasts with the preferred site of spinal meningioma, which is the thoracic segment [2,3,5,7,8,10,12].

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The histotypes of the varieties of meningioma described were: psammomatous (Pansini's case 2), angioblastic (Pansini's case 1), papillary (Pagni's case), and fibroblastic in our case. Case 1 of Pansini and Conti may be doubtful because it is difficult to differentiate between angioblastic meningioma and hemangioblastoma [3,5,7,8,12]. Pagni's case [5] is particularly rare, although the histotype was diagnosed as papillary after exhaustive histological study; there is no doubt, however, about the other two histotypes. The pathogenetic substrate of meningioma at this uncommon site might be explained in the terms suggested by Shuangshoti et al [9], who contend that meningocytes are modified fibroblasts deriving from mesenchymal cells, and that intramedullary meningiomas might originate in the mesenchymal cells lining the perivascular spaces of the neuraxis. The diagnosis of intramedullary meningioma, as for other intramedullary space-ocFigure 2. Intraoperative appearance of the tumor." the enlarged spinal cord

is visible (A); after myelotomy (B) the tumor is clearly shown.

A

B

Figure 1. MRI of the cervical spine in (A) lateral and (B) anteroposterior view, showing an intramedullary lesion extending from C-2 to C-4.

The neurological symptoms in three cases, including our own, began many years earlier (30 years in the two cases of Pansini and Conti [6], 10 years in our case), whereas in the case of Pagni [5] onset was more acute (7 months). The first symptom was pain in two cases (ours and Pagni's) and a motor syndrome in the two cases of Pansini and Conti [6].

B

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A

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B

Figure 3. Histological picture of excised tumor showing (A) a fibroblastic meningioma (hemotoxylin and eosin stain; calibration bar, 17 Ixm); at higher magnification (B) some psammomatous bodies are clearly visible (hematoxylin and eosin stain; calibration bar: 15 Ixm). In another area (C), various meningiomatous whorls in a "fibroblastic pattern" are recognizable (hematoxylin and eosin stain × 80).

cupying lesions, rests chiefly on myelography, CT myelography and, most important, magnetic resonance imaging (MRI). This procedure, especially with paramagnetic contrast medium (gadolinium), supplies a more comprehensive overall view of the region and affords a picture of the relationship of the lesion with the surrounding tissues. Myelography was used in all the recorded cases, supplemented in two (Pagni's case and our own) by CT myelography. A CT scan performed in case 2 of Pansini and Conti did not contribute any information. In our case, a spinal MRI, performed to complete the previous

investigations, proved to be illuminating. In Pagni's case [5], the plain x~ray films of the cervicothoracic column imaged a small calcification at the tumor level. Surgical removal was total in all cases. In two cases (case 1 of Pansini and Conti [6] and our own) the neurological pattern improved considerably. In case 2 of Pansini and Conti, the patient regained movements of the upper limbs, but died from respiratory complications 2 months after operation. H o w Pagni's patient ultimately fared is not reported. In conclusion, when confronted by clinical and in~ strumental findings that point to an intramedullary space-occupying lesion in a patient with symptoms of

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long standing, one must include meningioma in the differential diagnosis. Surgery may be curative, making use of all modern methods of surgical technique and monitoring of spinal cord function.

References 1. Cheng MK. Spinal cord tumors in the People's Republic of China: a statistical review. Neurosurgery 1982;10:22-4. 2. Guidetti B, Fortuna A, Moscatelli G, Riccio A. I tumori intramidollari. Lay Neuropsich 1964;35:1-409. 3. Kepes JJ: Meningiomas. Biology, pathology and differential diagnosis. New York: Masson, 1982:1-206. 4. Niebeling HG, Hohrein P. 400 intraspinal space-narrowing processes: a clinical study. Zentrabl Neurochir 1978;39:241-52. 5. Pagni CA. I meningiomi spinali. Roma: CIC Edizioni Internazionali, 1989:1-249. 6. Pansini A, Conti P. On some rare spreading cervical lesions. J Neurosurg Sci 1981;25:255-7.

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7. Russell DS, Rubinstein LJ. Tumours of the meninges and related tissues. In: Russell DS, Rubinstein W, eds. Tumours of the nervous system. 5th ed. London: Edward Arnold, 1989:499-532. 8. Schiffer D, Fabiani A. Meningiomi. In: Schiffer D, Fabiani A, eds. I tumori cerebrali. Biologia e patologia. Istologia, ultrastruttura e diagnostica neuro-oncologica, 2nd ed. Roma: Il Pensiero Scientirico Editore, 1975:320-53. 9. Shuangshoti S, Netsky MG, Jane JA. Neoplasm of mixed mesenchymal and neuroepithelial type, with consideration of the relationship between meningioma and neurinoma. J Neurol Sci 1971;14:277-91. 10. Sloof JL, Kernohan JW, MacCarty CS. Primary intramedullary tumors of the spinal cord and ilium terminale. Philadelphia: WB Saunders, 1964:1-255. 11. Wen-Qing H, Shi-Ju Z, Qing-Sheng T, Yu-Xia L, Qing-Zhong X,

Zi-Jun L, Wen-Cui Z. Statitical analysis of the central nervous system tumors in China. J Neurosurg 1982;56:555-64. 12. Zulch KJ. Tumors of meningeal and related tissues. In: Zulch KJ, ed. Brain tumors. Their biology and pathology, 3rd ed. Berlin: Springer-Verlag. 1986:357-93, 532-3, 540-1.