Intracranial meningiomas in children: Review of 29 cases

ELSEVIER

Neoplasm

INTRACRANIAL MENINGIOMAS IN CHILDREN: REVIEW OF 29 CASES P. Erdiqler, M.D., G. Lena, M.D.,* A. C. Sanoglu, M.D., C. Kuday, M.D., and M. Choux, M.D.* Department of Neurosurgery, Cerrahpaga Medical School, Istanbul University, Istanbul, Turkey, ‘Department of Pediatric Neurosurgery, Timone Children’s Hospital, Marseille, France

Erdincler P, Lena G, Sanoglu AC, Kuday C, Choux M. Intracranial meningiomas in children: review of 29 cases. Surg Neurol 1998;49:136-41.

KEY WORDS

Meningioma, children, brain neoplasm,neurofibromatosis.

BACKGROUND

Meningiomas are predominantly tumors of the fifth and sixth decades of life. Although rare in infancy and childhood, they represent an important field in pediatric neurosurgery. METHODS

Twenty-nine children under 15years of age with intracranial meningiomaswere treated during the period 19681994in the Department of Pediatric Neurosurgery in Marseille (23 cases) and in the Department of Neurosurgery of CerrahpasaMedical School in Istanbul (6 cases).These tumors represented 2.4% of all central nervous system (CNS) tumors in this age group. RESULTS

There were 18 boys and 11 girls. Eighteen cases (62%) occured between 10 and 15 years of age and two cases were infants. Twelve children (41%) had associated neurofibromatosis. Presenting symptoms were related to the tumor location. Thirty-one intracranial meningiomas were observed in these 29 children. Cerebral convexity was the most common location and 13% of the tumors had no dural attachment. Thirty-one tumors were operated on and total removal was achieved in 25 patients (86.2%). Five patients died, one during surgery. There was no mortality among the 17 children without neurofibromatosis. After a mean 6.5 year follow-up period, 13 patients (45%) are neurologically intact, 8 patients (27.5%) have a moderate disability, without evidence of tumor recurrence. CONCLUSION

Childhood meningiomas occur predominantly in males. Absence of dural attachment is more common in children than in adults. Childhood meningiomashave a low recurrence rate. They are frequently associated with neurofibromatosis; this is the most important factor influencing outcome. 0 1998by Elsevier Science Inc.

Address correspondence and reprint requests to: Pamir ErdinCler, PK 19 34310 K.M. Pwa, Istanbul, Turkey. Received January 17, 1997; accepted April 24, 1997. 0090-3019/98/$19.00 PI1 s0090-3019(97)00343-1

M.D.,

B

ecause of their benign nature in the great majority of cases, meningiomas have always fascinated neurosurgeons. Although they are uncommon in infancy and childhood, some features of childhood intracranial meningiomas, such as male predominance or lower frequency of dural attachment, have been well described in the literature [8,10,23]. Many controversies exist also and a definitive description of childhood meningiomas still requires much broader experience. This article reviews 29 cases of surgically treated intracranial meningiomas in children under 15 years of age. The findings were compared with those of other studies in children and in adults.

MATERIALSANDMETHODS Twenty-nine children under 15 years of age with intracranial meningiomas were surgically treated during the period of 1968-1994 in the Department of Pediatric Neurosurgery in Marseille (23 cases) and in the Department of Neurosurgery of Cerrahpass Medical School in Istanbul (6 cases). The pathological material of all cases were reviewed. Meningeal fibrosarcomas, fibrous histiocytomas, a variety of other sarcomas, meningeal melanomas, and angioblastic meningeal tumors are not included. Only classic meningiomas according to the World Health Organization (WHO) classification were studied [14]. Clinical information was ob tained by review of patient records and operative reports, and follow-up data was obtained via records of outpatient clinic visits or by contact with the patient’s attending physician. 655 Avenue

0 1998 by Elsevier Science Inc. of the Americas, New York, NY 10010

Surg Neurol 1998;49:136-41

Intracranial Childhood Meningiomas

0

Age and Sex Distribution Meningioma

of 29 Cases of Childhood TOTAL

MALE O-12 l-5 years months 6-10 years 11-15 years Total

-4 2

12 18

21 2 6

11

: 4

18 29

RESULTS INCIDENCE, AGE AND SEX DISTRIBUTION Out of 1,200 pediatric brain tumors operated on during the study period in the referred hospitals only 29 (2.4%) were histologically identified as meningiomas. There were 18 boys and 11 girls. Eighteen cases (62%) occured in patients between 10 and 15 years of age and two cases were infants under 1 year of age. The average age was 10 years (Table 1). CLINICAL PRESENTATIONS Presenting symptoms were related to the tumor location. The most common symptoms and signs were increased intracranial pressure observed in 12 patients (41%), motor deficit in 6 patients (21%), and cranial nerve palsy in 4 patients (14%). Five patients had a cranial deformity as a result of parieta1 swelling or parietal softening. Four of them were under 5 years of age. When the diagnostic criteria for central or peripheral neuroiibromatosis (NF) were applied, seven patients had peripheral (NFl) and five patients had central type (NF2) NF. Seven out of twelve patients with associated NF were female. In a 6-year-old girl, 5 years after the diagnosis of a posterior fossa meningioma, cutaneous manifestations of central NF with bilateral acoustic neurinomas were observed. In another child 13 years old with brachiofacial hemiparesis, blurred vision, and hypoacousia 2 years after the initial diagnosis of a small third ventricular tumor on computed tomography (CT) scan, magnetic resonance imaging (MRI) scan showed multiple tumors located on the clivus, the two cerebellopontine angles, and the third ventricle, with several associated intraspinal tumors. The clival and intraventricular tumors were removed and identified as meningiomas. RADIOLOGICAL FINDINGS Plain skull radiographs demonstrated significant abnormalities in more than 70% of patients with meningiomas. Hyperostosis and bone erosion were

q

137

Summarv of Tumor Location in 29 Cases*

LOCALIZATION

No

Supratentorial Convexity Intraventricular Intraosseous Sphenoid wing Parasellar Total Infratentorial CPA Posterior fossa Total *Two children

17 3

1 2 2 25 3 3 6

had two meningiomas

each.

the most common and specific findings on plain skull films. They were present in one third of our cases. Cerebral angiography was helpful in the diagnosis of eight patients before CT scan was available. In 14 of our patients, in whom cerebral angiography was performed, feeding arteries coming from meningeal and/or choroidal arteries were demonstrated in eight. In the other six patients, this study showed only vessel displacements. Preoperative MRI scan was performed in nine children and suggested the diagnosis of meningioma but did not provide pathologic specifity. The border of the tumor could be delineated on MRI, and all meningiomas enhanced markedly after gadolinium administration. Fifty-nine percent of meningiomas were greater than 5 cm. ANATOMICAL DISTRIBUTION Thirty-one intracranial meningiomas were observed in these 29 children. Nineteen percent of the tumors were infratentorial and 13% had no dural attachment (Table 2). Two children with associated NF had multiple meningiomas. PATHOLOGY The histological subtypes of tumors are summarized in Table 3. Fifty-two percent were meningothelial, and only one tumor showed the character-

El Histological Meningothelial Transitional Fibroblastic Psammomatous Anaplastic Myxoid

Subtypes of the 29 Meningiomas NFl

NF2

ONLY TUMOR

4

2 2

9 4

1 1 1

-

1 -

-

1 2 -

1

TOTAL 15 7 3 2

1 1

138

0’

Erdin@er et al

Surg Neurol 1998;49:136-41

DISCUSSION

Results: 29 Patients GOOD MODERATELY LOST TO EVOLUTION DISABLED DIED FOLLOW-UP

NFl NF2 Only tumor Total

1 1 11 13

1

4

3 4 8

1 5

1 2 3

istic patterns of an anaplastic meningioma. No atypical or papillary meningiomas were found. Two meningiomas diagnosed during infancy were psammomatous. Aggressive behavior of a fibroblastic meningioma was noted in a 15year-old girl, in whom a parietal parasagittal tumor metastatized to the cervical lymph nodes 3 years after the surgery. PROGNOSIS AND TREATMENT Thirty-one tumors were operated on and total removal was achieved in 25 patients (86.2%). Preoperative embolization was helpful in two giant meningiomas. Adjuvant chemotherapy and radiotherapy were done in a unique case of anaplastic meningioma that was reoperated. Radiotherapy was used in a 14year-old boy with a meningothelial meningioma in the parietal convexity, which recurred after three consecutive surgeries over a 13year period. Both patients died. Three patients underwent partial excision of their tumors, One of them had a parasellar meningioma and although two consecutive surgeries were performed, one transcranially and the other one transsphenoidally, the tumor could not be totally removed because of its tight adherence to major neural and vascular structures. The other two patients had tumors located in the posterior fossa. All three patients are still alive after a mean 5-year follow-up, without clinical or radiological evidence of recurrence. There was one operative death because of excessive bleeding from the superior sagittal sinus; 4 patients died because of recurrent disease after total excision of their tumor. The remaining 18 patients who underwent total excision showed no recurrence; 3 patients were lost to follow-up. Two patients who underwent surgery in infancy are still alive with 18 and 22 years follow-up respectively. There was no mortality among the 17 children without NF. With a mean follow-up period of 6.5 years, 13 patients (45%) are neurologically intact and 8 (27.5%) have moderate disabilities without evidence of tumor recurrence (Table 4).

The incidence of childhood meningiomas ranges from 1% to 7.7% of all CNS tumors [8,15]. In our experience they constitute 2.4% of all pediatric CNS tumors. AGE AND SEX As in our series, the male predominance of childhood meningiomas has been confirmed in many literature series [9,10,15,23]. This is in strong contrast with adult series of meningiomas [21,22]. Perhaps, with age, females are more vulnerable to the factors predisposing to meningioma formation such as trauma, sex hormones, radiation therapy, and others [l]. Females also seem more affected by genetic factors in our series, because 7 of 12 patients with associated NF were female. In a literature review of childhood intracranial meningiomas after high-dose irradiation, Ghim et al [ 111 found nine girls versus six boys. Congenital meningiomas diagnosed in infancy are more rare. A recent literature review described only 27 meningiomas in patients between the ages of 1 week and 12 months

POl* CLINICAL PRESENTATION Nonspecific symptoms and signs were found in most children with meningiomas; their onset was usually insidious. Bulging of the parietal region was noticed at birth in two patients in our series. Local swelling or tumor formation on the cranial vault may be the initial and only sign of meningiomas in childhood, particularly in younger children. In our series, this symptom was observed in 5 of 29 patients, including a case of intraosseous meningioma. In 41% of our cases an association with neurofibromatosis was observed; this association has been reported in only 23% of patients by Deen et al [ 71 and Merten et al [ 181 and in 13% of patients by German0 et al [lo]. RADIOLOGICAL FINDINGS Recent reports of MlU findings in childhood meningiomas show a high frequency of huge and cystic tumors [5,12]. We have no cystic tumors in our series, but in 59% of cases, the tumor was larger than 5 cm. Of the conventional methods, plain skull radiographs, even if they are not specific, are still valuable. Tumor blush, observed in 60% of our cases, is the same that was observed in the literature [9,18]. ANATOMICAL DISTRIBUTION The convexity is the most frequent location of these tumors in adults [ 211. Although a convexity location

Intracranial Childhood Meningiomas

was found in 55% of our cases, our series differs from those of adults because of the higher frequency of infratentorial localization, noted in 19% of the cases. The incidence of intraventricular tumors in this age group is also particularly high. A literature review of 278 meningiomas in the first two decades of life gave an incidence of 9.4% for intraventricular location [lo] versus 5% for adult series [ 2 11. Although intraventricular meningiomas are relatively frequent in pediatric patients, they are extremely rare in the third ventricle [ 171. Meningiomas in this location may arise either from the velum interpositum, the choroid plexus, or from the floor of the third ventricle [ 171. Two giant meningiomas located in the third ventricle were an uncommon feature of our series. Meningiomas with lack of dural attachment, other than the intraventricular ones, are more frequent in children than in adults [8]. They can occur in the deep sylvian fissure [8], in the suprasellar region [26], and from intraosseous locations within the cranial vault [3]. PATHOLOGY Childhood meningiomas are known to have a higher incidence of malignant changes than meningiomas in adults. This aggressive behavior was seen in two cases; the first one was a fibroblastic meningioma of the parietal convexity that metastatized to the cervical lymph nodes 3 years after the craniotomy. The second was an anaplastic meningioma in an 18 month-old boy that recurred 6 months after the surgery. Although meningothelial meningiomas constituted the major subtype in our series, in our two infants we didn’t find this subtype of tumor. Also, in a recent review of the literature by Mollestone [20], the majority of infantile meningiomas were fibroblastic and no meningothelial subtype was found. Papillary meningioma, which has been reported as a malignant variant of meningioma [ 161, was not seen in our series. However, this variant was found in 9.8% by Deen et al and had a 2-year mean recurrence time compared with 9.4 years for all other histological types [7]. PROGNOSIS AND TREATMENT Meningiomas are generally thought to be slowgrowing and benign tumors and every effort is made to achieve total removal. Some tumors, however, represent a formidable technical challenge because they adhere to vital neural and vascular structures. Although its role in diagnosing meningiomas has changed, angiography still remains an important pretreatment evaluation technique; it also demonstrates the tumor’s vascularity and its feeders in

Surg Neurol 1998;49:136-41

139

preparation for preoperative embolization in cases of giant tumors. The prognosis of childhood meningiomas is still controversial. Some reports give a poor prognosis for childhood meningiomas [2,4]; others are more encouraging [8,10]. It is clear that with modern medical support, there is a tendency toward better results. In a recent review of the literature on infantile meningiomas by Molleston et al [20], 10 of 11 patients diagnosed since 1980 were alive, versus 4 of 8 who survived between 1970 and 1980. Total removal of the tumor is an important factor influencing the outcome [9,24]. Total removal of the tumor was achieved in 86.2% of our patients; it has been reported in 54% to 79% in the literature [g-10, 181. But all of our four patients who died of recurrent disease had total tumor removal and only one of them had an anaplastic meningioma. The histological subtype of the tumor may also influence the outcome. Meningeal sarcomas and other malignant tumors of meningeal origin such as hemangiopericytomas and angioblastic and melanocytic tumors are more aggressive than classic meningiomas and have a poor prognosis [6,8, lo]. Only classic meningiomas were studied in this series and aggressive behavior, in term of distant metastasis, was seen only in fibroblastic meningioma. Recurrence is related to tumor location [7], histological type, and extent of removal [7,9]. According to Merten et al [ 181, 5 of 20 patients with partial resection or biopsy of the tumor are alive and all have recurrent tumor. Regrowth after subtotal surgical resection in adults was found in 91% at 15 years by Mirimanoff et al [ 191. Ten years after surgery, Taylor et al [25] reported tumor progression rates of 82% after subtotal resection and 33% after total resection of meningiomas. An 80% recurrence rate was found in papillary meningiomas and 35% in those with more benign histological sub types, by Deen et al [7]. When only classic meningiomas were included, no recurrence was found by German0 et al [lo]. In our three cases with partial removal, we have not observed regrowth. Adjuvant chemotherapy or radiotherapy were not beneficial in two cases in our series. In a recent report on adult meningiomas, radiotherapy was found to increase survival in subtotally resected tumors [13], but the risks of radiation therapy in young children are such that its use for a supposedly benign tumor is unjustified, especially in children under 3 years of age. Reoperation, even if only partial, is preferable to irradiation of the tumor for increasing the time and the quality of survival. The prognosis of childhood meningiomas is difficult to predict and seems influenced in our series only by

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the association with NF. Children with NF are often confronted with complications because of the complex natural history of their disease. Because cutaneous lesions may be absent, patients in the pediatric age group with meningiomas should be carefully followed for a long time after treatment, not only for their postoperative care, but also because of possible occurrence of a second tumor. REFERENCES Black PM. Meningiomas.

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COMMENTARY

This article by Erdincler et al is a retrospective review from two centers, one in Marseille and the other in Istanbul; it adds 29 cases to the world literature on this type of tumor in children. The authors’ findings regarding sex ratio, incidence, clinical presentation, imaging data, location, surgical treatment, and outcome do not differ very much from the other series reviewed in the article. When looking at the literature, there is always a great deal of difference in the reported incidence rates; for example, during the pre-CT era, Matson reported only three cases of meningioma out of 750 intracranial tumors (0.4%) [5]; Koos and Miller reported 20 cases in 700 lesions (2.8%) [4]; although in an even more ancient report, Bailey, Buchanan, and Bucy [ 11 found 8 in only 100 cases (8%)! On the other hand, after the introduction of CT and MFU, the incidence of pediatric meningioma has varied from 1 to 4.2%. The reason for that is not only the availability of CT or MRI, but also better surgical techniques, histopathological diagnosis, intensive care units, and other medical support-all of these influence today’s perioperative mortality rate of almost zero. Another important fact to keep in mind is that meningiomas are difficult to diagnose and classify because their histological appearance is highly variable and sometimes they can mimic glial tumors; in these situations the use of immunohistochemical stains using vimentin and GFAP can help establish