Tiirkis/i Neiirosiirgery 6: 79 - 82, 1996 Güner: C/iildliood Mellingioma

Childhood Malignant Meningioma

Çocukluk Çaginda Malign Meninjioma

METIN GÜNER, UNAL KIRISOGLU,ENGIN UÇAR, TANSU MERTOL, KUTSAL YÖRÜKOGLU

Dokuz Eylül University, School of Medicine, Departments of Neurosurgery (MG,ÜK,EU,TM) and Pathology (KY), Izmir, Turkey

Abstract: Childhood meningiomas, besides their rarity,grow fast, recur frequently and have a dismal prognosiswhen compared with adult tumorso A five year old patientpresenting with headache, emesis and left hemiparesia wasadmitted to our hospital in 1990. On computerizedtomography a 85x80x87 mm mass in the rightfrontoparietal region surrounded by significant edema,having a wide base on the falx causing 2 cm shift to leftwas found. The patient was operated on in our clinic witha diagnosis of multilobuled giant intracranial meningioma.The tumor showing frequent recurrence and sarcomatouschanges is presented and discussed. Although the CTfindings in this case suggest malignancy the behavior ofthe meningiomas is usually determined by histologicnature. In cases where frequent recurrence is seen theperiod between recurrences diminishes in time. ThereforeCT control s are important in diagnosing recurrence. Totalexcision in early stage may increase the chance of survival.

Key words: Childhood, malignant, meningioma

INTRODPCTION

Intracranial meningiomas rarely occur inchildhood and constitute about 0.4-3,5 % of all

pediatric brain tumors (3,7,8,12,16,19,23,26). Matsonreported onlyone patient in his series of 313 cases ofintracranial tumors (8), and only three children withmeningioma in his series of 750 cases of intracranialtumors und er 14 years of age. In Cushing andEisenhardt's series there are only six cases among313 patients with intracranial meningiomas (3).Childhood meningioma series in the literature areshown in Table I (3,5,9,10,13,14,15,17,25,31).

We present a multilobulated giant intracranialmeningioma case with frequent recurrence andsarcomatous changes.

Özet: Çocukluk çagi meninjiomalari nadir olmalarininyanisira eriskin tümörleriyle karsilastirildiginda hizlibüyüme, sik nüksetme, ve kötü bir prognoza sahip olmagibi özelliklere sahiptirler. Basagrisi, kusma ve solhemipareziyle basvuran bes yasinda bir hasta 1990 yilindahastanemize yatirildi. Bilgisayarli tomografide sagfrontoparietal bölgede 85x80x87 mm boyutlarindabelirgin ödemi olan, falksa genis bir tabanla yapismis, ortahatta 2 cm kaymaya neden olan bir kitle saptandi. Hasta,dev multilobule kafa içi meninjioma tanisiyla klinigimizdeameliyat edildi. Sik nüks ve sarkomatöz degisikliklergösteren tümör sunuldu ve tartisildi. Bu olgudabilgisayarli tomografi bulgulari malignitedüsündürmesine ragmen meninjiomalarin davranisigenellikle histolojik yapisiyla belirlenir. Sik nüks gösterenolgularda nüksler arasindaki süre giderek azalir.Dolayisiyla aralikli bilgisayarli tomografi kontrollerinüksü tanimada önemlidir. Erken dönemde tümörün tamçikarilmasi yasam sansini arttirabilir.Anahtar sözcükler: Çocukluk, ma lign, meningioma

CASE REPORT

A five year old girl complaining of headache,vomiting ,and left hemiparesis was admitted to ourclinic in 1990. Her neurological examination showedbilateral papilledema, left sided spastic hemiparesis,increased deep tendon reflexes ,and Babinski sign.Preoperative computed tomography (CT) showed amass situated in the left frontoparietal region,spontaneously hyperdense, intensely enhancing af tercontrast material injection, measuring 85x80x87 mm.The mass features fringes and irregular borders witha wide base on the falx (Figure 1,a and b).

The patient underwent a right centralcraniotomy and the mass was removed subtotally.Histological specimens revealed a meningioma with

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TurkisJi Neurosurgery 6: 79 - 82, 1996 Güiier: C/iildJiood Meiiiiigioma

Figure 1, a and b: Preoperative computed tomography showed a multilobu!ated, hyperdense, intense!y contrast enhancing,85x80x87 mm !eft frontoparieta! mass with fringes and irregu!ar borders.

anaplastic features (Figure 2). Postoperativeneurological examination did not show anyadditional neurological deficit. A CT scan performed

Figure 2: First postoperative contrast enhanced CT­showing residua! mass and abscess formation.

Figure 3: Histopatho!ogica! specimens revea! an anap!asticmeningioma. (H&E,X200)

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17 days after the operation revealed residual massand abscess formation (Figure 3). After antibiotictherapy for one month the abscess was drained andthe culture revealed Staphylococcus epidermidis andaureus. Appropriate antibiotic therapy was givenbut a control CT two months later showed the mass

and the persisting abscess. The patient wasreoperated and the abscess was totally removed withresidual mass. Histopatological examinationrevealed an anaplastic meningioma and secondaryinfection (Figure 4). Earlyand Iate control CT didnot show any mass. Although radiotherapy wasplanned, the family refused further adjuvant therapy.Three months later the patient was readmitted to ourclinic with severe headache. The CT showed a

multilobulated heterogenous mass which wasremoved totally with repeat surgery. Postoperativecourse was uneventful and histopatological

Figure 4: Histopatho!ogica! examination reveals ananap!astic meningioma and secondaryinfedion. (H&E,X200)

Turkish Neurosiirgery 6: 79 - 82, 1996

Table i. Childhood meningioma series.Author AgeNumber of BenignAtypical Malignanl

casesSosnik&Wrzesczynski (31) At birth

11

Fessard (14)

Atbirth11

Aorin&Reid (15)

Atbirth11

Cuneo&Rand (9)

Atbirth11

Endo&Aihara (13)

3 days11

Benli at aL. (5)

5 days11

Mendiratta at al. (25)

7 days11

Alp (3)

14 months1 1

Huang(17)

6 years1 1

Davidson (10)

4 months-16 years 27234(2 sarcomatous)

examination showed meningioma with sarcomatouscomponent. The control CT did not show anyintracranial mass. Any further diagnostic study ortherapy was refused by the family.

it was later learned that the patient hadsuccumbed to the disease two years after the initialsigns and symptoms.

DISCUSSION

Meningiomas are rarely seen in children(3,7,8,12,16,19,23,26) and differ from adult tumors byatendeney toward malignancy, increase in mass, anda worse prognosis (8,16,18). The incidence ofintraventricular meningioma in children is reportedto be higher than adults (17). Although themalignancy of meningioma is determined byhistological examination CT mayaIso hint atmalignant behavior.

Computed tomography is an excellent methodin the diagnosis of meningioma (4,16,27). Gd-DTPAenhanced magnetic resonance (MR) seans areconsidered to have a slightly higher diagnostic valuethan contrast enhanced CT 01,16,28,29). The typicalCT findings are hyperdense or isodense mass withdifferent rates of calcification, hyperostosis ,andedema.showing contrast enhancement after contrastinjection (4,11,16,19,27,28,29). The definite findingsof malignancy and atypia are heterogeneousenhancement, hemorrhage, cyst formation, poorlydefined or fringed margins, marked edema andosteolysis (4,11,16,19,29).

Computed tomography findings of this case areintratumoral hypodense areas, uncertain boundaries,fringes and extensive heterogeneous enhancementwhich seem to be the findings of malignancy.

Güiier: Childhood Meiiiiigioma

Histopathological findings conclusivelydetermine the malignant behavior of meningiomas(4,10). The extent of the surgical procedure (accordingto Simpson O» and the degree of anaplasia (presenceof increased cellularity, loss of architecture, nuclearpleomorphism, mitotic figures, focal necrosis andbrain infiltration) are the factors that effect tumorrecurrence 0,4,19). The variants of meningioma inthe new World Health Organization (WHO)c1assification (21) are meningothelial, fibrous,transitional, psammomatous and metaplastic(secretory, microcystic, clear cell, lymphoplasmacyte­rich) subgroups. The new c1assification inc1udesatypical meningioma in the intermediate biologicbehaviour group and in the malignant group,malignant and papillary types (21).

Malignant meningiomas retain enoughhistologic features to be recognized as meningiomas,but in addition have conspicuous mitoses, tumornecrosis, and invasion (21).

There are different grading schemes modifiyingWHO criteria. In general grade I meningiomas areaccepted as benign, grade II,III, and IV as atypical,anaplastic, and sarcomatous, respectively (6,22). Thefirst and second biopsies of our case were evaluatedas grade III. The last biopsy was evaluated as gradeIV.

Anaplastic (malignant) meningioma can berecognized easily but mayaIso be confused withanaplastic glioma, fibrosarcoma ,and schwannoma.Meningiomas are epithehal membrane antigen (EMA)and vimentin positive immunohistochemically andnegatiye for glial fibrillary acidic protein (GFAP).Gliomas are positive for GFAP and fibrosarcomasare positive for vimentin but negative for EMA.Differention from schwannoma necessitate electron

microscopy as well as positivity for 5-100 protein(24). The tumor in our case was positive focally forEMA, and vimentin, but negative for GFAP and 5­100 protein. Also meningothelial whorls, reticulinand collagen content were prominent focally in allof the biopsy materials.

After complete removal the 5 year recurrencerate is 78 % for anaplastic tumors (9) and againsurvival decreases in tumors with sarcomatouschanges 0,4,19). Retrospective analysis show ed thatradiotherapy decreases recurrence rates andi orprevents recurrence 0,4,18). Metastatic meningiomais rare, constituting about 0.1 % of all meningiomas(2).

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Tiirkish Neiirosiirgery 6: 79 - 82, 1996

Radiation therapy was advocated inhistologically malignant meningiomas (4,19). Againsame retrospedive studies showed a decrease in therecurrence rates of subtotally excised malignantmeningiomas af ter radiotherapy (30).

Contrary to this idea, some authors believe thatradiotherapy has some benefits, but it may have littlevalue in the management of recurrent meningiomas(20).

Recurrence intervals get shorter every time,therefore it is important to follow the patient withperiodic neuroimaging studies and to remove thetumor completely in order to prolong survIval.

Correspondence: Metin Güner, MDDokuz Eylül UniversitySchool of Medicine

Department of Neurosurgery,35340 Inciralti, Izmir, TurkeyTelephone: (232) 259 59 59 / 3300

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