- Email: [email protected]
Meningioma in children: A report of nine cases and review of the literature
ELSEVIER
MENINGIOMA IN CHILDREN: A REPORT OF NINE CASES AND REVIEW OF THE LITERATURE Bassem Y. Sheikh, F.R.C.S., Edir Siqueira, M.D., Ph.D., and Fouad Dayel, M.D. Departments of Neurosciences and Pathology & Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
Sheikh BY, Siqueira E, Dayel F. Meningioma in children: a report of nine cases and review of the literature. Surg Neurol 1996;45:
328-35. BACKGROUND Meningioma is a common tumor of the central nervous system in adults, accounting for lo%-20% of all primary intracranial tumors. It is rare in children, with an incidence of 2.19%(0.85%-2.3%).Although there are numerous casereports in the literature, no firm conclusions can be drawn. For this report we accumulated, reviewed, and analyzed reports in the literature from 1960-93. METHODS
A total of 318 patients with meningiomawere managedin King Faisal Specialist Hospital and ResearchCentre from 1981-93.Nine of these patients (2.8%) were children aged 16 years or less. These cases were analyzed retrospectively with regard to age, sex, clinical presentation, radiologic findings, pathologic findings, management, and outcome. RESULTS
Childhood meningioma represented 2.8% of all meningioma casesand 2.2% of all central nervous system tumors seenin children. There were six malesand three females. The average age at presentation was 10.1 years (range 1-16 years). There were four casesof meningioma in the orbit and one each in the temporal region, foramen magnum, tentorial region, subfrontal base, sellar region, and ethmoidal air sinus. There were two cases of multiple meningioma. Meningothelial meningioma was the type most frequently seen. CONCLUSION Meningioma is rare in children and males are affected more than females. Tumor locations that are rare in adults are more common in children. The meningothelial type is most frequently seen.Prognosisis poor compared with that in adults, as the tumors tend to grow more rapidly and to a larger size, undergo malignant changes, and have a greater rate of recurrence. Whenever feasible, every effort should be made to achieve total resection of the tumor at the first attempt. If radiation therapy is used in children it should be limited to those who have recurAddress reprint requests to: Dr. Bassem Y. Sheikh, Neurosciences, King F&al Specialist Hospital &Research 11211, Saudi Arabia. Received April 28, 1995; accepted August 23, 1995. 0090-3019/96/$15.00 SSDI 00903019(95)00451-3
Department of Centre, Riyadh
rent tumors so as to minimize organic and psychologic complications. KEY
WORDS
Childhood, central nervous system, hormone receptors, meningioma,neoplasia.
M
eningioma is a common tumor of the central
nervous system in adults, accounting for lo%-20% of all primary intracranial tumors. It is rare in children, with an incidence of 2.19% (0.85%2.3%) [1,10,13,15,21,43,50]. Reviewing cases of pediatric meningioma in the available literature from 1960-93, gave a total of 212 cases aged 16 years and under [l-3,5,6,9-11,13-25,27-29,31-38,41,44,46-50, 52,55-59,61,62,64-701. Details were missing from some cases but males were affected more than females (113 and 67, respectively); the average age was 9 years. The meningothelial type was most frequent, followed by the fibroblastic and transitional types. The tumors were frequently in locations that are uncommon in adults. Progesterone receptors were more common than estrogen receptors, as in meningioma in adults. The incidence of meningioma of the spine was even lower than that of intracranial meningioma.
MATERIALSANDMETHODS Nine cases of meningioma in children 5 16 years were managed at King Faisal Specialist Hospital and Research Centre from 1981-93 (Table 1). There were six males and three females; their average age was 10.1 years (range 4-16 years). Patient #4 had features of neurofibromatosis. Patient #5 has been previously reported [62]. The tumors were located in the orbit [4], and one each in the temporal region, tentorial region, foramen magnum, subfrontal 6.55 Avenue
0 1996 by Elsevier Science Inc. of the Americas, New York, NY 10010
Meningioma
in Children
Surg Neurol 1996;45:328-35
329
0
Summary of Cases
PT
AGE/SEX
LOCSTION
PATHOLOGY
1.
*8Y/M
M
Yes
3yLF
2. 3. 4.
1ly/M 6Y/M 9YlI:
M M T
None None None
3yfalive 7YfLF 4y/alive
5. 6. 7. 8. 9.
4y/M 15y/F *8Y/F 14y/M 16YlM
Ethmoidal bone with orbital extension, CPA Orbit Tentorium Orbit, extending to sellar region Foramen ma~um Orbit Orbit, sellar region Subfrontal base Temporal area
Yes None None None None
Gy/alive 1y/alive 1y/alive O.liy/alive O.Sy/alive
M
HORMONE RECEPTORS ESTROGEN/PROGESTERONE
Negative/positive
P T z
Negative/positive Negative/positive
RECURRENCE
OUTCOME
*multiple meningioma. M = meningothe~iomatous. T = transitional. P = psammatous. LF = lost to follow-up.
base, sellar region, and ethmoid air sinus. Two patients presented with multiple meningioma, Presentation included increased intracranial pressure and various focal neurologic deficits, depending on the location (Table 2). Pathologic examination showed meningotheliomatous type in six cases, transitional type in two, and ps~momatous in one case. Hormone receptor studies were done in three males and showed positive progesterone receptors and negative estrogen receptors. In two cases (22.1%), both of which resection had been incomplete, there was recurrence of tumor. The radiologic studies of some patients are demonstrated in Figures l-4.
DISCUSSION As in most brain tumors, the etiology of meningioma is unknown. Factors considered when deter-
q
Clinical Presentation MUMBO
Headache Quadriparesis Paraparesis Proptosis C.N. palsy: II III lv VI VII VIII to XII
mining etiology include association with neurofibromatosis ~15,28,39,48,60,61], radiation [?,8,20,53, 631, congenital [38,66], and familial factors [42,61]. Meningioma is a rare entity in childhood, with an incidence of 2.19% [ 1,10,13,15,21,43,50]. There are several reports in the literature but because of the small number of cases in each report, no firm conclusions could be made. In children, meningioma tends to grow rapidly to a large size, undergoes malignant changes, and is likely to recur [ 10,13,17,20,29]. For these reasons the outcome is poor. In the reports reviewed here, the average age at diagnosis was 10.1 years (range 4 months-16 years). This is higher than the average age in most other primary intracranial tumors in the same age group [21,29,33,34,56]. In contrast to the female predominance in meningioma in adults, the incidence in children is greater in males with a ratio of 1.7:1. The sites of the tumor in children are comparable with those in adults, however, locations considered to be rare in adults are seen more frequently in children, intraventricular, posterior including fossa, orbit, and tumors without dural attachment [12,~3,17,21,24,35,41,43,58,68]. Unfortunately, different classifications of tumor locations have been used in the reports reviewed, which makes it difficult to draw conclusions. In some cases, the large size of the tumor precludes identification of the exact site of origin. The locations are listed in Table 3, which shows that meningioma occurs most frequently in the lateral ventricles, anterior and posterior cranial fossae. When reclassified into supratentorial and infratentorial the incidence was 73.3%
330
She&h et al
Surg ~~urol 1996;45:328-35
B Enhanced CT scan (A), and postgadolinium T, MRI (B), showing the orbital meningioma in an ll-yearold boy.
Ia B Sagittal (A), and transaxial (B) ‘I’,-weighted MRI, showing a menin~ioma in the foramen magnum extending into the upper cervical region.
•B
and 16.66%, respectively. Meningioma of the spine is less frequent (8.024%) than intracranial tumor in children [6,16,19,37]. Multiple meningioma is less frequent in children with an incidence of 1%~2.5% compared with 8%-9% in adults [21,39,60,64].
Analysis of the pathologic variants showed that of 131 cases of known pathologic type, 45 were meningotheliomatous (34%), 30 were fibroblastic (23%), 30 were transitional (23%), 14 were sclerosing (ll%), seven were angioblastic (5%), four were psammomatous (3%) and one was chordoid (0.76%) (Table 4). The sclerosing type of menin~oma consists mainly of collagen bundles with a small population of spindle cells, showing perinuclear artifactual halos. This is not biologically different from the
Meningioma
H
in Children
Surg Neural 1996;~5:328-35
Enhanced CT scan of the brain, showing a huge frontal meningioma.
other histofogic types, but it is important in that it may be mistaken for astrocytoma or meningeal sarcoma [ 14,291. Cystic changes occur more frequently in children: 13%-M% compared with 2%-4.6% in adults ~2,12,21,46,~0]. Four types of cysts have been described [45]. The cyst may be intratumoral either in the center (type I) or on the periphery (type II), or peritumoral either within the brain substance (type III), or at the brain-tumor interface (type IV). On computed tomography (CT) scan these cystic men-
H
33 1
a ,
T,-weighted MRf showing a meningioma in a 16-yearold girl involving the orbit and the optic nerve and extending into the optic foramen.
ingiomas can be mistaken for malignant glioma or metastasis [51,71]. Hormone receptor studies in adults are well reported [4,26] but there is little information on the pediatric group 19,271. Our results concur with the few series that have been reported, namely that progesterone receptors are more frequently present than any other receptor. This is so in the pediatric and adult age groups. The CT appearance of meningioma typically shows a hyperdense or isodense mass with variable
Locations Reported in the Literature
Supratentoriai 3rd ventricle Anterior cranial fossa Convexity Diffuse
5 17 12
Frontal Fafx Frontal intraparench~al Lateral ventricle Middle cranial fossa
:5 1 18 13
Parasagittal Parietaf Parietal epidural Parieto-occipitaf P~iet~temporal Sellar region Sphenoid wing Supratentorial Sylvian fissure
Orbital Infratentoriai 4th ventricle Cerebellopont~ne angle Clivus Foramen magnum Spinal
11
Total Temporal
1 2 i
Interpeduncufar cistern Posterior cranial fossa Tentorial Total Total Grand Total
: : 7 1 1 123 (d%) 1 15 27 (16:6%) 13 (8.02%) 163
332
q‘
Surg Nemo1 1996;45:328-35
Sheikh et al
Histologic Variants in Cases Reported in the Literature
Meningothelial Fibroblastic Transitional Sclerosing Angioblastic Psammatous Chordoid
23 15 19 8 4 1 0 70
13 12 11 6 3 2 1 48
calcification, edema, and hyperostosis. l-lomogenous enhancement is usually found following injection of contrast material. There may be an atypical appearance in 6%-7% of cases ~6,31~3~,41,66,67]. Meningioma is usually iso-hypointense on Trweighted MRI and hyperintense on T,-weighted images. Following gadolinium injection it usually shows marked enhancement. Recurrence of tumor depends on the extent of removal at the first craniotomy, the location of the tumor, and histologic evidence of aggressiveness [ 10,15,2~,43,47]. Although the role of radiation therapy in meningioma is not well established, it has been used in cases where only partial resection could be accomplished [ 10,13,47].
SUMMARY Meningioma is rare in children and males are affected more than females. Tumor locations that are rare in adults are more common in children, The meningothelial type is most frequently seen. Prognosis is poor compared with that in adults, as the tumors in children tend to grow more rapidly and to a larger size, to undergo malignant changes, and to have a greater rate of recurrence. Whenever feasible, every effort should be made to achieve total resection of the tumor at the first attempt. If radiation therapy is used in children it should be limited to those who have recurrent tumor so as to minimize organic and psychologic complications. The authors would like to thank Shoaa AI Hejaili for her help in ~ollati~ data, and Yvo~ff~ Lock for editing and typing the report.
REFERENCES 1. Aghadiuno PU, Adeloye A, Uluminde AA, Nottidge VA. Intracranial neoplasms in children in Ibadan, Nigeria. Childs Nerv Syst 1985;1:39-44. 2. Amano K, Miura N, Tajika Y, Matsumori K, Kubo 0,
1 13
45 30 30 14 7 4 1 131
34.4% 22.9% 22.9% 10.7% 5.3% 3.1% 0.8%
Kobayashi N, Kitamura K. Cystic meningioma in a lo-month-old infant: case report. J Neurosurg 1980; 52:829-33. 3. Aydin Y, Yoias C, Kiinte H, Aydin IH. Cystic meningiomas: report of two cases. Neurosurgery 1985;16:8789. 4. BIack PMcL. Meningiom~. Neurosurge~ 199~3~~~ 57. 5. Bueno JC, Esteban HM, Lopez CB, Puentes MLF. Congenital meningioma. Childs Brain 1977;3:304-8. 6. Calogero JA, Moossy J. Extradural spinal meningiomas: report of four cases. J Neurosurg 1972;37:44247. 7. Cantini R, Bur~hianti M, Fabrini MC, Valleriani AM, Simi U. Postirradiation meningioma. Childs Nerv Syst 1987;3:382-84. 8. Cantini R, Giorgetti W, Valleriani AM, Burchianti M, Amodeo C. Radiation-induced cerebral lesions in childhood. Childs Nerv Syst 1989;5:135-39. 9. Carter DA, Holienberg RD. Sex hormone receptors in a congenital meningioma. Pediatr Neurosci 1988;14: 15-I 7. 10. Chan RC, Thompson GB. Intracranial meningiomas in childhood. Surg Neurol 198$;21:319-22. 11. Chen H-J, Lui C-C, Chen L. Meningioma in the anterior III ventricle in a child. Chiids Nerv Syst 19862:X0-61. 12. Ciaveria LE, Sutton D, Tress BM. The radiological diagnosis of meningiomas, the impact of EMR scanning. Brit J Radio1 1977;50:15-22. 13. Crouse SK, Berg BO. Intracranial meningiomas in childhood and adolescence. Neurology 1972;22:13541. 14. Davidson GS, Hope JK. Meningeal tumors of childhood. Cancer 1989~63:1205-10. 15. Deen HG Jr, Scheithauer BW, Ebersold MJ. Clinical and pathological study of meningiomas of the first two decades of life. J Neurosurg 1982;56:317-22. 16. DeSousa AL, Kalsbeck JE, Mealey J Jr, Campbelt RL, Hockey A. Intraspinal tumors in children: a review of 81 cases. J Neurosurg 1979;51:437-45. 17. Diaz P, Maiilo A, Morales F, G~mez-Moreta JA, Hernandez J. Multiple meningiomas of the fourth ventricle in infancy: case report. Neurosurgery 1990; 26: 1057-60. 18. Dinakar I, Rao SB. Cystic meningioma in a child. Indian J Pediatr 1971;38:223-24. 19. Di Rocco C, CaIdarelli M, Puca A, Colosimo C Jr. Multiple spinal meningiom~ in children. Neurochir 1984;27:25-27.
Meningioma in Children
20. Donovan WD, Govoni AF. An 11 year-old boy with a history of AML and new onset of headaches. Clinical Imaging 1992; l&272-76. 21. Ferrante L, Acqui M, Artico M, Mastronardi L, Rocchi G, Fortuna A. Cerebral meningiomas in children. Childs Nerv Syst 1989;5:83-86. 22. Ferro JM, Martins IP, Tavora L. Neglect in children. Ann Neurol 1984;15:281-84. 23. For-tuna A, Ferrante L, Acqui M, Guglielmi G, Mastronardi L. Cystic meningiomas. Acta Neurochir 1988; 90923-30. 24. Glasier CM, Husain MM, Chadduck W, Boop FA. Meningiomas in children: MR and histopatholo~~ findings. AJNR 1993;14:237-41. 25. Greenberg SB, Schneck MJ, Faerber EN, Kanev PM. Malignant meningioma in a child: CT and MR findings. AJR 1993;160:1111-12. 26. Grunberg SM, Daniels AM, Muensch H, Daniels JR, Bernstein L, Kortes V, Weiss MH. Correlation of meningioma hormone receptor status with hormone sensitivity in a tumor stem-cell assay. J Neurosurg 1987; 66:404-g. 27. Halper J, Colvard DS, Scheithauer BW, Jiang N-S, Press MF, Graham ML, Rlehl E, Laws ER Jr, Spelsberg TC. Estrogen and progesterone receptors in meningiomas: comparison of nuclear binding, dextran-coated charcoal, and immunoperoxidase staining assays. Neurosurgery 1989;25:546-53. 28. Herz DA, Shapiro K, Shulman K. Intracranial meningiomas of infancy, childhood and adolescence. Review of the literature and addition of 9 case reports. Childs Brain 1980;7:4~56. 29. Hope JKA, Armstrong DA, Babyn PS, Humphreys RR, Harwood-Nash DC, Chuang SH, Marks PV. Primary meningeal tumors in children: correlation of clinical and CT findings with histologic type and prognosis. AJNR 1992;13:1353-64. 30. Jakoblec FA, Depot MJ, Kennerdell JS, Shults WT, Anderson RL, Alper ME, Citrin CM, Housepaln EM, Trokel SL. Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma. Ophthalmology 1984;91:137-55. 31. Johnson TE, Weatherhead RG, Nasr AM, Siqueira EB. Ectopic (extradural) meningioma of the orbit: a report of two cases in children. J Pediatr Ophth~mol Strabismus 1993;3~4~47. 32. Katayama Y, Tsubokawa T, Yoshida K. Cystic meningiomas in infancy. Surg Neurol 1986;25:43-48. 33. Klump TE, McDonald JV. Successful removal of a large meningioma in a three year-old boy. J Neurosurg 1971;34:92-94. 34. Koiluri VRS, Reddy DR, Reddy PK, Naidu MRS, Rao SBP, Sumathi C. Meningiomas in childhood. Childs Nerv Syst 1987;3:271-73. 35. Lee Y-Y, Lin S-R, Horner FA. Third ventricle meningioma mimicking a colloid cyst in a child. AJR 1979;132: 669-71. 36. Leibel SA, Wara WM, Sheline GE, Townsend JJ, Boldrey EB. The treatment of meningiomas in childhood. Cancer 1976;37:2709-12. 37. Liu HC, De Armond SJ, Edwards MSB: An unusual spinal meningioma in a child: case report. Neurosurgery 1985;17:313-16. 38. Lopez MJ, Olivares JL, Ramos F, Redondo JA, Ram&
Surg Neurol 1996;45:328-35
39. 40.
41.
42. 43. 44. 45.
46. 47.
48. 49. 50. 51.
52. 53.
54. 55. 56. 57.
58.
333
Y, Cajal S, Banzo J, Legido A. Giant meningioma in a 5-month-old infant. Childs Nerv Syst 1988;4:112-15. Lusins JO, Nakagawa H. Multiple meningiomas evaluated by computed tomography. Neurosurgery 1981; 9137-41. Mani RL, Hedgcock MW, Mass SI, Gilmor RL, Enzmann DR, Eisenberg RL. Radiographic diagnosis of meningioma of the lateral ventricle. J Neurosurg 1978;49: 249-55. Markwalder T-M, Seiler RW, Markwalder RV, Huber P, Markwalder HM. Meningioma of the anterior part of the third ventricle in a child. Surg Neurol 1979;12:2932. Memon MY. Multiple and familial meninglomas without evidence of neurofibromatosis. Neurosurgery 1980;7:262-64. Merten DF, Gooding CA, Newton TH, Malamud N. Meningiomas of childhood and adolescence. J Pediatr 197~5:696-700. Mircevskl M, Mircevsak D, Bojadziev I, Basevska R. Surgical treatment of intraventricular meningiomas in childhood. Acta Neurochir 1985;35:89-91. Nauta HJW, Tucker WS, Horsey WJ, Bilbao JM, Gonsalves C. Xanthochromic cysts associated with meningloma. J Neural Neurosurg Psychiatr 1979;42:52935. Parisi G, Tropea R, Giuffrida S, Lombard0 M, Giuffre F. Cystic meningiomas: report of seven cases. J Neurosurg 1986;64:35-38. Perilongo G, Sutton LN, Goldwein JW, Gusnard D, Schut L, Biegel JA, Rorke LB, Lanage B, D’Angio GJ. Childhood meningiomas: experience in the modern imaging era. Pediatr Neurosurg 1992;18:16-23. Porras CL. Meningioma in the foramen magnum in a boy aged 8 years. J Neurosurg 1962;20:167-68. Rao SB, Dinakar I, Rao KS. Giant intracranial epidural meningioma: case report. J Neurosurg 1971;35:74850. Reddy DR, Kolluri VRS, Rao KS, Reddy PK, Naldu MRC, Rao SBP, Sesikeran B. Cystic meningiomas in children. Childs Nerv Syst 1986;2:317-19. Rengachary S, Batnitzky S, Kepes JJ, Moran& RA, O’Boynick P, Watanabe 1. Cystic lesions associated with intracranial meningiomas. Neurosurgery 1979;4: 107-14. Roberts B Jr, Horner FA, McDon~d JV. Anterior third ventricle meningioma in a child. Childs Brain 1981;8: 145-48. Rubinstein AB, Shalit MN, Cohen ML, Zandbank U, Reichenthal E. Radiation-induced cerebral meningioma: a recognizable entity. J Neurosurg 1984,61:96671. Russell EJ, George AE, Kricheff If, Budzilo~ch G. Atypicaf computed tomographic features of intracranial meningioma. Radiology 1980;135:673-82. Sakaki S, Nakagawa K, Kimura H, Ohue S. Intracranial meningiomas in infancy. Surg Neurol 1987;28:51-57. Sano K, Wakai S, Ochiai C, Takakura K. Characteristics of intracranial meningiomas in childhood. Childs Brain 1981:~98-~06. Satanarayana K, Chandy SM, Rao TS, Chandy MJ, Abraham J. Congenital meningioma with subdural hygroma and porencephaly (case report). Neurology (lndia) 1975;23:57-58. Schiffer J, Mundel G, Lahat E, Schwarzmann H, Shental
334
59.
60. 61, 62. 63. 64. 65. 66. 67. 68. 69. 70. 71.
Surn Neural 199&45:328-35 i. Multiple meningiom~ in separate neuroaxial compartments in a child. Childs Brain 1980;6:281-88. Schroeder BA, Samaraweera RN, Starshak RJ, Oechler HW. Intraparenchymal meningioma in a child: CT and MRI findings. J Comput Assist Tomogr 1987;11:192200. Sheehy JP, Crockard HA. Multiple meningiomas a long-term view. J Neurosurg 1983;59:1-5. Sieb JP, P&t S-M, Buch A. Familial CNS tumors. J Neurol 1992;23~34~44. Siqueira EB, Kanaan I, Ah MA. Large meningioma of the foramen magnum in a 4-year-old child. Surg Neurol 1989;31:409-11. Soffer D, Pittaluga S, Feiner M, Beller AJ. Intracrani~ meningiomas following low-dose irradiation to the head. J Neurosurg 1983;59:1048-53. Spittaler PJ, Johnston IH. Multiple extracranial meningiomas in a child. Childs Nerv Syst 1992;8:144-46. Tamaki N, Kuwamura K, Kudo H, lshida K, Matsumoto S, Okada S. Meningioma in the interpeduncular cistern in a child. Chiids Nerv Syst 1985;1:123-25. Taptas JN. Intracranial meningioma in a four-monthold infant simulating subdural hematoma. J Neurosurg 1961;1~120-21. Teng P, Papatheodorou C. Suprachiasmal and intraventricular meningioma in a four-year-old child. Neurol Neurosurg Psychiatr 1961;24:379-80. Vassilouthis J, Ambrose JAE. Intraventricular meningioma in a child. Surg Neural 1978;10:105-7. Waga S, Matsuda M, Wanda H, Matsushima M, Ando K. Multiple meninglomas: report of four cases. J Neurosurg 1972;37:348-51, Wentworth AF, Clare FB, Bell HS. Angioblastic meningioma in a child. J Neurosurg 1961;18:25.2-54. Wo~hin~on C, Caron J-L, Meianson D, Leblanc R. Meningioma cysts. Neurology 1985;35:1720-24.
COMMENTARY
The authors describe a personal series of nine children with intracranial meningioma and provide a useful review of the literature concerning such tumors. From their analysis, meningiomas account for 2.2% of all central nervous system tumors occurring in the pediatric age group; they predominate in males, with a ratio of 1.7:1, tend to grow rapidly to large size, and to undergo malignant changes. The average age at diagnosis is 10 years, higher than that observed in most other primary intracrania1 tumors in the same age group. Further characteristics of meningiomas in children are the higher frequency of intraventri~ular, posterior fossa, and orbital tumors as compared to adults, as well as the absence of dural attachment in a large number of cases. Although the analysis is accurate and illustrates the general aspects of this type of tumor, more detailed characteristics could have been delineated if the data from the literature were subdivided according to the different pediatric age groups. In a retrospective cooperative study on 764 intracranial
Sheikh et a1
tumors in the first year of life that was carried out by the International Society for Pediatric Neurosurgery USPN)PI9 meningiomas accounted for 2%similar to the percentage reported by the present authors for the whole pediatric population. In most of these cases, however, the diagnosis had been obtained after investigations performed because of symptoms and signs of increased intracranial pressure, mainly related to the large size of the tumor. Nevertheless, in most recent reports that reflect the impact of the wide diffusion of modern diagnostic tools for neuroimaging, the incidence of meningiomas in infants is two or three times higher than that observed in the retrospective ISPN study. Seizure disorder, sometimes with EEG tracing mimicking those of hypsarrhythmia, have become a relatively frequent presenting clinical manifestation prior to the occurrence of signs and symptoms of intracranial hypertension. As a consequence, the interval between the occurrence of the symptomatolo~ and the diagnosis has become considerably shorter. In spite of the exceedingly large dimensions that meningiomas can reach in this age group, early surgical treatment is currently satisfactory in a large majority of cases 121. Owing to the relatively low number of cases reported in infants, it is difficult to establish whether sarcomatous degeneration is actually as frequent in infants as in children. fn children from 2-12 years of age, the sarcomatous degeneration of meningiomas has been reported in 14% of the cases. However, it has been pointed out that sarcomatous degeneration could not have the ominous significance that the term would imply, as long survivals have been reported in patients undergoing such an event; similarly, it has been stressed that it is necessary to distinguish the phenomenon from true meningeal sarcomas, which obviously carry a considerably worse prognosis [3]. Nearly one-half of the children with meningiomas have seizures, signs of intracranial hypertension, and paresis. The occurrence of an associated hemianopsia would distin~ish the patients harbouring lateral ventricle tumors from those with parasagittal or convexity meningiomas. Indeed, in this age group the intraventri~ul~ location is relatively more frequent (7%-lo%, in contrast to 0.2%-4% observed in adults). Males outnumber females in both infants and children. In older children and adolescents, however, females are more frequently affected than males, similar to what occurs in adults. In the same way, the time interval between the onset of symp toms and the diagnosis is usually signi~cantly longer than in infants and children, almost reaching
Meningioma
in Children
that recorded in adults patients. In this specific age
subgroup, the percentage of subjects with von Recklinghausen’s disease-about one-fifth of the cases-appears particularly significant. As for other types of tumors of the central nervous system, early diagnosis and treatment are associated with higher rates of success; furthermore, better intraoperative and postoperative care currently available in the treatment of infants and children have resulted in considerably more rewarding outcomes than those recorded in the past. Consequently, the statement that meningiomas in children often carry a poor prognosis should be reconsidered in the light of the experience gained in recent years. This is also apparently demonstrated by the present report, which describes
Surg Neurol 1996;45:328-35
335
satisfactory results in eight out of the nine patients considered. Concezio
Di Rocco, M.D. Rome, ~~~iy
REFERENCES 1. Di Rocco C, Iannelli A, Ceddia A. Intracranial tumors of the first year of life. A cooperative study of the 19861987 Education Committee of the ISPN. Child’s Nerv syst 1991;7:150-53. 2. Di Rocco C, Ceddia A. Iannelli A. Intracranial tumours in the first year of life. A report on 51 cases. Acta Neurochir (Wien) 1993;123:14-24. 3. Shapiro K, Shulman K. Meningiomas. In: Pediatric Neurosurgery. New York Grune & Stratton, 1982:445-g.
MENINGIOMA IN CHILDREN: A REPORT OF NINE CASES AND REVIEW OF THE LITERATURE Bassem Y. Sheikh, F.R.C.S., Edir Siqueira, M.D., Ph.D., and Fouad Dayel, M.D. Departments of Neurosciences and Pathology & Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
Sheikh BY, Siqueira E, Dayel F. Meningioma in children: a report of nine cases and review of the literature. Surg Neurol 1996;45:
328-35. BACKGROUND Meningioma is a common tumor of the central nervous system in adults, accounting for lo%-20% of all primary intracranial tumors. It is rare in children, with an incidence of 2.19%(0.85%-2.3%).Although there are numerous casereports in the literature, no firm conclusions can be drawn. For this report we accumulated, reviewed, and analyzed reports in the literature from 1960-93. METHODS
A total of 318 patients with meningiomawere managedin King Faisal Specialist Hospital and ResearchCentre from 1981-93.Nine of these patients (2.8%) were children aged 16 years or less. These cases were analyzed retrospectively with regard to age, sex, clinical presentation, radiologic findings, pathologic findings, management, and outcome. RESULTS
Childhood meningioma represented 2.8% of all meningioma casesand 2.2% of all central nervous system tumors seenin children. There were six malesand three females. The average age at presentation was 10.1 years (range 1-16 years). There were four casesof meningioma in the orbit and one each in the temporal region, foramen magnum, tentorial region, subfrontal base, sellar region, and ethmoidal air sinus. There were two cases of multiple meningioma. Meningothelial meningioma was the type most frequently seen. CONCLUSION Meningioma is rare in children and males are affected more than females. Tumor locations that are rare in adults are more common in children. The meningothelial type is most frequently seen.Prognosisis poor compared with that in adults, as the tumors tend to grow more rapidly and to a larger size, undergo malignant changes, and have a greater rate of recurrence. Whenever feasible, every effort should be made to achieve total resection of the tumor at the first attempt. If radiation therapy is used in children it should be limited to those who have recurAddress reprint requests to: Dr. Bassem Y. Sheikh, Neurosciences, King F&al Specialist Hospital &Research 11211, Saudi Arabia. Received April 28, 1995; accepted August 23, 1995. 0090-3019/96/$15.00 SSDI 00903019(95)00451-3
Department of Centre, Riyadh
rent tumors so as to minimize organic and psychologic complications. KEY
WORDS
Childhood, central nervous system, hormone receptors, meningioma,neoplasia.
M
eningioma is a common tumor of the central
nervous system in adults, accounting for lo%-20% of all primary intracranial tumors. It is rare in children, with an incidence of 2.19% (0.85%2.3%) [1,10,13,15,21,43,50]. Reviewing cases of pediatric meningioma in the available literature from 1960-93, gave a total of 212 cases aged 16 years and under [l-3,5,6,9-11,13-25,27-29,31-38,41,44,46-50, 52,55-59,61,62,64-701. Details were missing from some cases but males were affected more than females (113 and 67, respectively); the average age was 9 years. The meningothelial type was most frequent, followed by the fibroblastic and transitional types. The tumors were frequently in locations that are uncommon in adults. Progesterone receptors were more common than estrogen receptors, as in meningioma in adults. The incidence of meningioma of the spine was even lower than that of intracranial meningioma.
MATERIALSANDMETHODS Nine cases of meningioma in children 5 16 years were managed at King Faisal Specialist Hospital and Research Centre from 1981-93 (Table 1). There were six males and three females; their average age was 10.1 years (range 4-16 years). Patient #4 had features of neurofibromatosis. Patient #5 has been previously reported [62]. The tumors were located in the orbit [4], and one each in the temporal region, tentorial region, foramen magnum, subfrontal 6.55 Avenue
0 1996 by Elsevier Science Inc. of the Americas, New York, NY 10010
Meningioma
in Children
Surg Neurol 1996;45:328-35
329
0
Summary of Cases
PT
AGE/SEX
LOCSTION
PATHOLOGY
1.
*8Y/M
M
Yes
3yLF
2. 3. 4.
1ly/M 6Y/M 9YlI:
M M T
None None None
3yfalive 7YfLF 4y/alive
5. 6. 7. 8. 9.
4y/M 15y/F *8Y/F 14y/M 16YlM
Ethmoidal bone with orbital extension, CPA Orbit Tentorium Orbit, extending to sellar region Foramen ma~um Orbit Orbit, sellar region Subfrontal base Temporal area
Yes None None None None
Gy/alive 1y/alive 1y/alive O.liy/alive O.Sy/alive
M
HORMONE RECEPTORS ESTROGEN/PROGESTERONE
Negative/positive
P T z
Negative/positive Negative/positive
RECURRENCE
OUTCOME
*multiple meningioma. M = meningothe~iomatous. T = transitional. P = psammatous. LF = lost to follow-up.
base, sellar region, and ethmoid air sinus. Two patients presented with multiple meningioma, Presentation included increased intracranial pressure and various focal neurologic deficits, depending on the location (Table 2). Pathologic examination showed meningotheliomatous type in six cases, transitional type in two, and ps~momatous in one case. Hormone receptor studies were done in three males and showed positive progesterone receptors and negative estrogen receptors. In two cases (22.1%), both of which resection had been incomplete, there was recurrence of tumor. The radiologic studies of some patients are demonstrated in Figures l-4.
DISCUSSION As in most brain tumors, the etiology of meningioma is unknown. Factors considered when deter-
q
Clinical Presentation MUMBO
Headache Quadriparesis Paraparesis Proptosis C.N. palsy: II III lv VI VII VIII to XII
mining etiology include association with neurofibromatosis ~15,28,39,48,60,61], radiation [?,8,20,53, 631, congenital [38,66], and familial factors [42,61]. Meningioma is a rare entity in childhood, with an incidence of 2.19% [ 1,10,13,15,21,43,50]. There are several reports in the literature but because of the small number of cases in each report, no firm conclusions could be made. In children, meningioma tends to grow rapidly to a large size, undergoes malignant changes, and is likely to recur [ 10,13,17,20,29]. For these reasons the outcome is poor. In the reports reviewed here, the average age at diagnosis was 10.1 years (range 4 months-16 years). This is higher than the average age in most other primary intracranial tumors in the same age group [21,29,33,34,56]. In contrast to the female predominance in meningioma in adults, the incidence in children is greater in males with a ratio of 1.7:1. The sites of the tumor in children are comparable with those in adults, however, locations considered to be rare in adults are seen more frequently in children, intraventricular, posterior including fossa, orbit, and tumors without dural attachment [12,~3,17,21,24,35,41,43,58,68]. Unfortunately, different classifications of tumor locations have been used in the reports reviewed, which makes it difficult to draw conclusions. In some cases, the large size of the tumor precludes identification of the exact site of origin. The locations are listed in Table 3, which shows that meningioma occurs most frequently in the lateral ventricles, anterior and posterior cranial fossae. When reclassified into supratentorial and infratentorial the incidence was 73.3%
330
She&h et al
Surg ~~urol 1996;45:328-35
B Enhanced CT scan (A), and postgadolinium T, MRI (B), showing the orbital meningioma in an ll-yearold boy.
Ia B Sagittal (A), and transaxial (B) ‘I’,-weighted MRI, showing a menin~ioma in the foramen magnum extending into the upper cervical region.
•B
and 16.66%, respectively. Meningioma of the spine is less frequent (8.024%) than intracranial tumor in children [6,16,19,37]. Multiple meningioma is less frequent in children with an incidence of 1%~2.5% compared with 8%-9% in adults [21,39,60,64].
Analysis of the pathologic variants showed that of 131 cases of known pathologic type, 45 were meningotheliomatous (34%), 30 were fibroblastic (23%), 30 were transitional (23%), 14 were sclerosing (ll%), seven were angioblastic (5%), four were psammomatous (3%) and one was chordoid (0.76%) (Table 4). The sclerosing type of menin~oma consists mainly of collagen bundles with a small population of spindle cells, showing perinuclear artifactual halos. This is not biologically different from the
Meningioma
H
in Children
Surg Neural 1996;~5:328-35
Enhanced CT scan of the brain, showing a huge frontal meningioma.
other histofogic types, but it is important in that it may be mistaken for astrocytoma or meningeal sarcoma [ 14,291. Cystic changes occur more frequently in children: 13%-M% compared with 2%-4.6% in adults ~2,12,21,46,~0]. Four types of cysts have been described [45]. The cyst may be intratumoral either in the center (type I) or on the periphery (type II), or peritumoral either within the brain substance (type III), or at the brain-tumor interface (type IV). On computed tomography (CT) scan these cystic men-
H
33 1
a ,
T,-weighted MRf showing a meningioma in a 16-yearold girl involving the orbit and the optic nerve and extending into the optic foramen.
ingiomas can be mistaken for malignant glioma or metastasis [51,71]. Hormone receptor studies in adults are well reported [4,26] but there is little information on the pediatric group 19,271. Our results concur with the few series that have been reported, namely that progesterone receptors are more frequently present than any other receptor. This is so in the pediatric and adult age groups. The CT appearance of meningioma typically shows a hyperdense or isodense mass with variable
Locations Reported in the Literature
Supratentoriai 3rd ventricle Anterior cranial fossa Convexity Diffuse
5 17 12
Frontal Fafx Frontal intraparench~al Lateral ventricle Middle cranial fossa
:5 1 18 13
Parasagittal Parietaf Parietal epidural Parieto-occipitaf P~iet~temporal Sellar region Sphenoid wing Supratentorial Sylvian fissure
Orbital Infratentoriai 4th ventricle Cerebellopont~ne angle Clivus Foramen magnum Spinal
11
Total Temporal
1 2 i
Interpeduncufar cistern Posterior cranial fossa Tentorial Total Total Grand Total
: : 7 1 1 123 (d%) 1 15 27 (16:6%) 13 (8.02%) 163
332
q‘
Surg Nemo1 1996;45:328-35
Sheikh et al
Histologic Variants in Cases Reported in the Literature
Meningothelial Fibroblastic Transitional Sclerosing Angioblastic Psammatous Chordoid
23 15 19 8 4 1 0 70
13 12 11 6 3 2 1 48
calcification, edema, and hyperostosis. l-lomogenous enhancement is usually found following injection of contrast material. There may be an atypical appearance in 6%-7% of cases ~6,31~3~,41,66,67]. Meningioma is usually iso-hypointense on Trweighted MRI and hyperintense on T,-weighted images. Following gadolinium injection it usually shows marked enhancement. Recurrence of tumor depends on the extent of removal at the first craniotomy, the location of the tumor, and histologic evidence of aggressiveness [ 10,15,2~,43,47]. Although the role of radiation therapy in meningioma is not well established, it has been used in cases where only partial resection could be accomplished [ 10,13,47].
SUMMARY Meningioma is rare in children and males are affected more than females. Tumor locations that are rare in adults are more common in children, The meningothelial type is most frequently seen. Prognosis is poor compared with that in adults, as the tumors in children tend to grow more rapidly and to a larger size, to undergo malignant changes, and to have a greater rate of recurrence. Whenever feasible, every effort should be made to achieve total resection of the tumor at the first attempt. If radiation therapy is used in children it should be limited to those who have recurrent tumor so as to minimize organic and psychologic complications. The authors would like to thank Shoaa AI Hejaili for her help in ~ollati~ data, and Yvo~ff~ Lock for editing and typing the report.
REFERENCES 1. Aghadiuno PU, Adeloye A, Uluminde AA, Nottidge VA. Intracranial neoplasms in children in Ibadan, Nigeria. Childs Nerv Syst 1985;1:39-44. 2. Amano K, Miura N, Tajika Y, Matsumori K, Kubo 0,
1 13
45 30 30 14 7 4 1 131
34.4% 22.9% 22.9% 10.7% 5.3% 3.1% 0.8%
Kobayashi N, Kitamura K. Cystic meningioma in a lo-month-old infant: case report. J Neurosurg 1980; 52:829-33. 3. Aydin Y, Yoias C, Kiinte H, Aydin IH. Cystic meningiomas: report of two cases. Neurosurgery 1985;16:8789. 4. BIack PMcL. Meningiom~. Neurosurge~ 199~3~~~ 57. 5. Bueno JC, Esteban HM, Lopez CB, Puentes MLF. Congenital meningioma. Childs Brain 1977;3:304-8. 6. Calogero JA, Moossy J. Extradural spinal meningiomas: report of four cases. J Neurosurg 1972;37:44247. 7. Cantini R, Bur~hianti M, Fabrini MC, Valleriani AM, Simi U. Postirradiation meningioma. Childs Nerv Syst 1987;3:382-84. 8. Cantini R, Giorgetti W, Valleriani AM, Burchianti M, Amodeo C. Radiation-induced cerebral lesions in childhood. Childs Nerv Syst 1989;5:135-39. 9. Carter DA, Holienberg RD. Sex hormone receptors in a congenital meningioma. Pediatr Neurosci 1988;14: 15-I 7. 10. Chan RC, Thompson GB. Intracranial meningiomas in childhood. Surg Neurol 198$;21:319-22. 11. Chen H-J, Lui C-C, Chen L. Meningioma in the anterior III ventricle in a child. Chiids Nerv Syst 19862:X0-61. 12. Ciaveria LE, Sutton D, Tress BM. The radiological diagnosis of meningiomas, the impact of EMR scanning. Brit J Radio1 1977;50:15-22. 13. Crouse SK, Berg BO. Intracranial meningiomas in childhood and adolescence. Neurology 1972;22:13541. 14. Davidson GS, Hope JK. Meningeal tumors of childhood. Cancer 1989~63:1205-10. 15. Deen HG Jr, Scheithauer BW, Ebersold MJ. Clinical and pathological study of meningiomas of the first two decades of life. J Neurosurg 1982;56:317-22. 16. DeSousa AL, Kalsbeck JE, Mealey J Jr, Campbelt RL, Hockey A. Intraspinal tumors in children: a review of 81 cases. J Neurosurg 1979;51:437-45. 17. Diaz P, Maiilo A, Morales F, G~mez-Moreta JA, Hernandez J. Multiple meningiomas of the fourth ventricle in infancy: case report. Neurosurgery 1990; 26: 1057-60. 18. Dinakar I, Rao SB. Cystic meningioma in a child. Indian J Pediatr 1971;38:223-24. 19. Di Rocco C, CaIdarelli M, Puca A, Colosimo C Jr. Multiple spinal meningiom~ in children. Neurochir 1984;27:25-27.
Meningioma in Children
20. Donovan WD, Govoni AF. An 11 year-old boy with a history of AML and new onset of headaches. Clinical Imaging 1992; l&272-76. 21. Ferrante L, Acqui M, Artico M, Mastronardi L, Rocchi G, Fortuna A. Cerebral meningiomas in children. Childs Nerv Syst 1989;5:83-86. 22. Ferro JM, Martins IP, Tavora L. Neglect in children. Ann Neurol 1984;15:281-84. 23. For-tuna A, Ferrante L, Acqui M, Guglielmi G, Mastronardi L. Cystic meningiomas. Acta Neurochir 1988; 90923-30. 24. Glasier CM, Husain MM, Chadduck W, Boop FA. Meningiomas in children: MR and histopatholo~~ findings. AJNR 1993;14:237-41. 25. Greenberg SB, Schneck MJ, Faerber EN, Kanev PM. Malignant meningioma in a child: CT and MR findings. AJR 1993;160:1111-12. 26. Grunberg SM, Daniels AM, Muensch H, Daniels JR, Bernstein L, Kortes V, Weiss MH. Correlation of meningioma hormone receptor status with hormone sensitivity in a tumor stem-cell assay. J Neurosurg 1987; 66:404-g. 27. Halper J, Colvard DS, Scheithauer BW, Jiang N-S, Press MF, Graham ML, Rlehl E, Laws ER Jr, Spelsberg TC. Estrogen and progesterone receptors in meningiomas: comparison of nuclear binding, dextran-coated charcoal, and immunoperoxidase staining assays. Neurosurgery 1989;25:546-53. 28. Herz DA, Shapiro K, Shulman K. Intracranial meningiomas of infancy, childhood and adolescence. Review of the literature and addition of 9 case reports. Childs Brain 1980;7:4~56. 29. Hope JKA, Armstrong DA, Babyn PS, Humphreys RR, Harwood-Nash DC, Chuang SH, Marks PV. Primary meningeal tumors in children: correlation of clinical and CT findings with histologic type and prognosis. AJNR 1992;13:1353-64. 30. Jakoblec FA, Depot MJ, Kennerdell JS, Shults WT, Anderson RL, Alper ME, Citrin CM, Housepaln EM, Trokel SL. Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma. Ophthalmology 1984;91:137-55. 31. Johnson TE, Weatherhead RG, Nasr AM, Siqueira EB. Ectopic (extradural) meningioma of the orbit: a report of two cases in children. J Pediatr Ophth~mol Strabismus 1993;3~4~47. 32. Katayama Y, Tsubokawa T, Yoshida K. Cystic meningiomas in infancy. Surg Neurol 1986;25:43-48. 33. Klump TE, McDonald JV. Successful removal of a large meningioma in a three year-old boy. J Neurosurg 1971;34:92-94. 34. Koiluri VRS, Reddy DR, Reddy PK, Naidu MRS, Rao SBP, Sumathi C. Meningiomas in childhood. Childs Nerv Syst 1987;3:271-73. 35. Lee Y-Y, Lin S-R, Horner FA. Third ventricle meningioma mimicking a colloid cyst in a child. AJR 1979;132: 669-71. 36. Leibel SA, Wara WM, Sheline GE, Townsend JJ, Boldrey EB. The treatment of meningiomas in childhood. Cancer 1976;37:2709-12. 37. Liu HC, De Armond SJ, Edwards MSB: An unusual spinal meningioma in a child: case report. Neurosurgery 1985;17:313-16. 38. Lopez MJ, Olivares JL, Ramos F, Redondo JA, Ram&
Surg Neurol 1996;45:328-35
39. 40.
41.
42. 43. 44. 45.
46. 47.
48. 49. 50. 51.
52. 53.
54. 55. 56. 57.
58.
333
Y, Cajal S, Banzo J, Legido A. Giant meningioma in a 5-month-old infant. Childs Nerv Syst 1988;4:112-15. Lusins JO, Nakagawa H. Multiple meningiomas evaluated by computed tomography. Neurosurgery 1981; 9137-41. Mani RL, Hedgcock MW, Mass SI, Gilmor RL, Enzmann DR, Eisenberg RL. Radiographic diagnosis of meningioma of the lateral ventricle. J Neurosurg 1978;49: 249-55. Markwalder T-M, Seiler RW, Markwalder RV, Huber P, Markwalder HM. Meningioma of the anterior part of the third ventricle in a child. Surg Neurol 1979;12:2932. Memon MY. Multiple and familial meninglomas without evidence of neurofibromatosis. Neurosurgery 1980;7:262-64. Merten DF, Gooding CA, Newton TH, Malamud N. Meningiomas of childhood and adolescence. J Pediatr 197~5:696-700. Mircevskl M, Mircevsak D, Bojadziev I, Basevska R. Surgical treatment of intraventricular meningiomas in childhood. Acta Neurochir 1985;35:89-91. Nauta HJW, Tucker WS, Horsey WJ, Bilbao JM, Gonsalves C. Xanthochromic cysts associated with meningloma. J Neural Neurosurg Psychiatr 1979;42:52935. Parisi G, Tropea R, Giuffrida S, Lombard0 M, Giuffre F. Cystic meningiomas: report of seven cases. J Neurosurg 1986;64:35-38. Perilongo G, Sutton LN, Goldwein JW, Gusnard D, Schut L, Biegel JA, Rorke LB, Lanage B, D’Angio GJ. Childhood meningiomas: experience in the modern imaging era. Pediatr Neurosurg 1992;18:16-23. Porras CL. Meningioma in the foramen magnum in a boy aged 8 years. J Neurosurg 1962;20:167-68. Rao SB, Dinakar I, Rao KS. Giant intracranial epidural meningioma: case report. J Neurosurg 1971;35:74850. Reddy DR, Kolluri VRS, Rao KS, Reddy PK, Naldu MRC, Rao SBP, Sesikeran B. Cystic meningiomas in children. Childs Nerv Syst 1986;2:317-19. Rengachary S, Batnitzky S, Kepes JJ, Moran& RA, O’Boynick P, Watanabe 1. Cystic lesions associated with intracranial meningiomas. Neurosurgery 1979;4: 107-14. Roberts B Jr, Horner FA, McDon~d JV. Anterior third ventricle meningioma in a child. Childs Brain 1981;8: 145-48. Rubinstein AB, Shalit MN, Cohen ML, Zandbank U, Reichenthal E. Radiation-induced cerebral meningioma: a recognizable entity. J Neurosurg 1984,61:96671. Russell EJ, George AE, Kricheff If, Budzilo~ch G. Atypicaf computed tomographic features of intracranial meningioma. Radiology 1980;135:673-82. Sakaki S, Nakagawa K, Kimura H, Ohue S. Intracranial meningiomas in infancy. Surg Neurol 1987;28:51-57. Sano K, Wakai S, Ochiai C, Takakura K. Characteristics of intracranial meningiomas in childhood. Childs Brain 1981:~98-~06. Satanarayana K, Chandy SM, Rao TS, Chandy MJ, Abraham J. Congenital meningioma with subdural hygroma and porencephaly (case report). Neurology (lndia) 1975;23:57-58. Schiffer J, Mundel G, Lahat E, Schwarzmann H, Shental
334
59.
60. 61, 62. 63. 64. 65. 66. 67. 68. 69. 70. 71.
Surn Neural 199&45:328-35 i. Multiple meningiom~ in separate neuroaxial compartments in a child. Childs Brain 1980;6:281-88. Schroeder BA, Samaraweera RN, Starshak RJ, Oechler HW. Intraparenchymal meningioma in a child: CT and MRI findings. J Comput Assist Tomogr 1987;11:192200. Sheehy JP, Crockard HA. Multiple meningiomas a long-term view. J Neurosurg 1983;59:1-5. Sieb JP, P&t S-M, Buch A. Familial CNS tumors. J Neurol 1992;23~34~44. Siqueira EB, Kanaan I, Ah MA. Large meningioma of the foramen magnum in a 4-year-old child. Surg Neurol 1989;31:409-11. Soffer D, Pittaluga S, Feiner M, Beller AJ. Intracrani~ meningiomas following low-dose irradiation to the head. J Neurosurg 1983;59:1048-53. Spittaler PJ, Johnston IH. Multiple extracranial meningiomas in a child. Childs Nerv Syst 1992;8:144-46. Tamaki N, Kuwamura K, Kudo H, lshida K, Matsumoto S, Okada S. Meningioma in the interpeduncular cistern in a child. Chiids Nerv Syst 1985;1:123-25. Taptas JN. Intracranial meningioma in a four-monthold infant simulating subdural hematoma. J Neurosurg 1961;1~120-21. Teng P, Papatheodorou C. Suprachiasmal and intraventricular meningioma in a four-year-old child. Neurol Neurosurg Psychiatr 1961;24:379-80. Vassilouthis J, Ambrose JAE. Intraventricular meningioma in a child. Surg Neural 1978;10:105-7. Waga S, Matsuda M, Wanda H, Matsushima M, Ando K. Multiple meninglomas: report of four cases. J Neurosurg 1972;37:348-51, Wentworth AF, Clare FB, Bell HS. Angioblastic meningioma in a child. J Neurosurg 1961;18:25.2-54. Wo~hin~on C, Caron J-L, Meianson D, Leblanc R. Meningioma cysts. Neurology 1985;35:1720-24.
COMMENTARY
The authors describe a personal series of nine children with intracranial meningioma and provide a useful review of the literature concerning such tumors. From their analysis, meningiomas account for 2.2% of all central nervous system tumors occurring in the pediatric age group; they predominate in males, with a ratio of 1.7:1, tend to grow rapidly to large size, and to undergo malignant changes. The average age at diagnosis is 10 years, higher than that observed in most other primary intracrania1 tumors in the same age group. Further characteristics of meningiomas in children are the higher frequency of intraventri~ular, posterior fossa, and orbital tumors as compared to adults, as well as the absence of dural attachment in a large number of cases. Although the analysis is accurate and illustrates the general aspects of this type of tumor, more detailed characteristics could have been delineated if the data from the literature were subdivided according to the different pediatric age groups. In a retrospective cooperative study on 764 intracranial
Sheikh et a1
tumors in the first year of life that was carried out by the International Society for Pediatric Neurosurgery USPN)PI9 meningiomas accounted for 2%similar to the percentage reported by the present authors for the whole pediatric population. In most of these cases, however, the diagnosis had been obtained after investigations performed because of symptoms and signs of increased intracranial pressure, mainly related to the large size of the tumor. Nevertheless, in most recent reports that reflect the impact of the wide diffusion of modern diagnostic tools for neuroimaging, the incidence of meningiomas in infants is two or three times higher than that observed in the retrospective ISPN study. Seizure disorder, sometimes with EEG tracing mimicking those of hypsarrhythmia, have become a relatively frequent presenting clinical manifestation prior to the occurrence of signs and symptoms of intracranial hypertension. As a consequence, the interval between the occurrence of the symptomatolo~ and the diagnosis has become considerably shorter. In spite of the exceedingly large dimensions that meningiomas can reach in this age group, early surgical treatment is currently satisfactory in a large majority of cases 121. Owing to the relatively low number of cases reported in infants, it is difficult to establish whether sarcomatous degeneration is actually as frequent in infants as in children. fn children from 2-12 years of age, the sarcomatous degeneration of meningiomas has been reported in 14% of the cases. However, it has been pointed out that sarcomatous degeneration could not have the ominous significance that the term would imply, as long survivals have been reported in patients undergoing such an event; similarly, it has been stressed that it is necessary to distinguish the phenomenon from true meningeal sarcomas, which obviously carry a considerably worse prognosis [3]. Nearly one-half of the children with meningiomas have seizures, signs of intracranial hypertension, and paresis. The occurrence of an associated hemianopsia would distin~ish the patients harbouring lateral ventricle tumors from those with parasagittal or convexity meningiomas. Indeed, in this age group the intraventri~ul~ location is relatively more frequent (7%-lo%, in contrast to 0.2%-4% observed in adults). Males outnumber females in both infants and children. In older children and adolescents, however, females are more frequently affected than males, similar to what occurs in adults. In the same way, the time interval between the onset of symp toms and the diagnosis is usually signi~cantly longer than in infants and children, almost reaching
Meningioma
in Children
that recorded in adults patients. In this specific age
subgroup, the percentage of subjects with von Recklinghausen’s disease-about one-fifth of the cases-appears particularly significant. As for other types of tumors of the central nervous system, early diagnosis and treatment are associated with higher rates of success; furthermore, better intraoperative and postoperative care currently available in the treatment of infants and children have resulted in considerably more rewarding outcomes than those recorded in the past. Consequently, the statement that meningiomas in children often carry a poor prognosis should be reconsidered in the light of the experience gained in recent years. This is also apparently demonstrated by the present report, which describes
Surg Neurol 1996;45:328-35
335
satisfactory results in eight out of the nine patients considered. Concezio
Di Rocco, M.D. Rome, ~~~iy
REFERENCES 1. Di Rocco C, Iannelli A, Ceddia A. Intracranial tumors of the first year of life. A cooperative study of the 19861987 Education Committee of the ISPN. Child’s Nerv syst 1991;7:150-53. 2. Di Rocco C, Ceddia A. Iannelli A. Intracranial tumours in the first year of life. A report on 51 cases. Acta Neurochir (Wien) 1993;123:14-24. 3. Shapiro K, Shulman K. Meningiomas. In: Pediatric Neurosurgery. New York Grune & Stratton, 1982:445-g.