- Email: [email protected]
Intraventricular malignant meningioma in a 6-year-old child
41 ELSEVIER
Intraventricular Malignant Meningioma in a 6-Year-Old Child Spiros Sgouros, F.R .C.S ., A. Richard Walsh, F.R .C .S., and Peter Barber, M .R.C.Path . Department of Neurosurgery, Birmingham Children's Hospital ; and Department of Neuropathology, University of Birmingham Medical School, U .K .
Sgouros S, Walsh AR, Barber P . Intraventricular malignant meninginma in a 6-year-old child . Surg Neural 1994:42 :41-5 . The case of a malignant intraventricular meningioma in a 6-year-old child is reported . He presented with symptoms of raised intracranial pressure and computed tomography scan revealed an enhancing tumor at the region of the septum pellucidum . After surgical excision, histology showed appearances of meningioma with atypical features indicating malignant behavior . Childhood ; Intraventricular ; Malignant ; Menitr gioma; Third ventricle KEY WORDS :
Meningiomas are rare in childhood, accounting for 1 %2% of all intracranial tumors . Intraventricular meningiomas comprise 15%-17% of all childhood meningiomas . Several reports of intraventricular meningiomas in young children have been published, most of them the benign histologic type We report a case of intraventricular malignant meningioma in a 6-year-old child . Case Report A 6-year-old boy presented with a 4-week history of intermittent frontal headaches associated with vomiting . The headaches were present on awaking and were followed by vomiting soon after getting out of bed . As a result of the persistent vomiting, he had lost 3 kg of weight in the last month . He did not report any visual complaints . On examination he had marked papilloedema in both eyes with no other abnormal neurologic signs . The visual acuity was normal in both eyes . His symptoms and signs indicated raised intracranial pressure . A computed tomography (CT) scan of his head showed a large, 5 cm in diameter, well-defined mass in the region of the septum pellucidum, obliterating the third ventricle and obstructing the outlet of both lateral ventricles, causing biventricular hydrocephalus (Figure Address reprint requests to : S . Sgouros, F.R .C .S ., Department of Neurosurgery, Birmingham Children's Hospital, I .adywnod Middleway, Birmingham B16 SET, United Kingdom . Received August 2, 1993 ; accepted January 6, 1994 . 0 1994 by Elsevier Science Is, -
I A) . After administration of intravenous contrast, the lesion enhanced uniformly (Figure 1 B .) Through a bicoronal incision a right midline frontal craniotomy was performed, and the rumor was approached transcallosally . It was seen in the region of the septum and the roof of the third ventricle, separate from the corpus callosum . An apparent complete removal was achieved with the aid of the Cavitron Ultrasonic Surgical Aspirator . At the end of the procedure the two lateral ventricles and the third ventricle were in communication freely . An external ventricular drain, which had been introduced in the left lateral ventricle through a separate left frontal burr hole at the beginning of the operation, was left in place . The right lateral ventricle was drained through a separate ventricular drain . He made a good postoperative recovery and eventually did not require any cerebrospinal fluid diversion procedure . A postoperative CT scan confirmed the free communication of the lateral and third ventricles and demonstrated apparent complete surgical removal of the tumor . (Figure 2), although a magnetic resonance imaging (MRI) obtained at 3 and 6 months showed some residual tumor that had not increased in the size (Figure 3) . A subdural collection over the greater convexity of the right cerebral hemisphere was apparent in both examinations . As the patient was asymptomaric, no attempt of treatment was made . Twelve months after operation, the patient remains very well and follows the school activities as per normal . No treatment of the subdural collection is contemplated . Histologic examination showed the characteristic features of a meningioma . In most areas the tumor was composed of elongated, fibroblastic cells with fibrillary cytoplasm and a distinct whorling architectural pattern (Figure 4) . Small islands of tumor cells with a more syncytical or "meningorhelial" appearance were interspersed . The tumor cell nuclei were generally oval, with open chromatin and one to three small nucleoli . There was moderate nuclear pleomorphism . Mitoses were frequent in some areas, and numerous irregular foci of degeneration and necrosis were present (Figure 5) . Immunoperoxidase stains were positive for epithelial membrane antigen (EMA) (Figure 6) and negative for filial fibrillary acidic protein and cytokerarins . Electron miIi 9a ;m9a944 7 uu
Surg Ncurol 1994 ; 4 2 :41-5
Sgouros et nl
Figure 1 . (A) : Unaurtrarted bead CT "art rhau hig a large r/;a e-oaeR/ryi e lesion in the region of the septune pllncidinm, ~hstrnaing the antler of both lateral xemrvieler anti raeaeng liiornntrtaalar hsdrart/rbaku . (B): Contrarkv1 CT seam shonvng increased enha,,,.,nt of the septal truuor.
B
croscopy clearly showed intercellular desmosomal reactions (Figure 7) . The histologic appearance was felt to be that of an atypical or malignant meningioma_ Chromosomal analysis performed on white cells of a peripheral blood sample, using the method of in situ hybridization, failed to demonstrate deletion of the long arm of chromosome 22, thus ruling out the presence of neurofibromatosis . Discussion Intraventricular meningiomas are rare intracranial tumors . Meningiomas in general comprise 1%-2% of
all central nervous system (CNS) tumors in childhood [2,(x8,12-14,16,18] . Intraventricular meningiomas account for 15%-19% of all meningiomas in children [2,8,12-14,16,18] . They are believed to originate from the choroid plexuses, the vela choroidea, or the velum interpositum [2,12,14,15] . The majority are found in the trigone of the left lateral ventricle [2,5,6,1012,14,17,181, bur both the third and the fourth ventricles have been reported as sites of origin [1,4, 7,9,10,121 . The majority of meningiomas of childhood are of benign histology, although sarcomatous change has been reported to vary between 7%-38% [3,6,13,14,16] .
Figure 2 . Postoperative CI scan showing
Figure 3 . AIRI scan obtained at 6 ntoruhr pnstoperatit~ely shotubeg reu theel Cantor at the region of the septim, pellucideem .
Figure 4 . Lot, , power photomicrograph of the intravantricelar tumor. shoteing the u'horling pattern of spindle cell a gth foil of necrosis (arrows) . Hernatoxylin and Loan Sca/c bar 250 µnn .
Sure Neurol 1994 ;42 :4 1 -5
Sgouros or al
Figure 5 . Higher paaeer photomicrograph showing the cellular morphology ge the tumor with fibrillary cytoplast and open, rmnd to oral nuclei shoeing moderate pleomorphism . Mitosis (arrows) are frequent. Hentatoxylin and eosin . Scale bar = 75 ant .
The incidence of neurofibromatosis in children with intracranial meningiomas has been reported to be 19%21% [2,6,10,14] . There is a well-known association of NF-II with multiple CNS tumors that tend to be of malignant biological behavior, but in our patient this possibility has been ruled out with appropriate chromosomal analysis . We report a case of a malignant intraventricular meningioma in a child . Although the bulk of the tumor was in relation to the septum pellucidum, it is preFigure 6 . lnutu noperozhl ce staining of the tumor for- epithelial membrane antigen (EMfd) . cellular morphologZ is not well seen, but the density of sktin indicates that almart all the tumor celh bn this field .,to ;,; pestle sly . Scale bar = 15 0 pas.
sumed to have originated from the stroma of the choroid plexus of the third ventricle, as is commonly the case [1,4,9,12] . Malignant meningiomas in that situation are distinctly uncommon . Malignant meningiomas of childhood have a poor prognosis with a 5-year survival of 40% [6], and radiotherapy does not offer any significant improvement . It is of interest that despite its malignant histologic profile, the meningioma of our patient has so far behaved in a benign fashion, although the limited available follow-up calls for reserved predictions .
Figure 7 . Electron micrograph showing two intercellular junctions botweeo tumor cell.. Scale bar = 0 . 5 am .
Intraventricular Malignant Meningioma in Child
Surg Neurol
45
1994 ;4 2 :41-5
References 1 . Avman N, Dincer C : Meningiomas of the third ventricle . Acta Neurochirurgica 1978 ;42 :217-24 . 2 . Byard RW, Bourne AJ, Clark B, Hanieh A : Clinicopathological and radiological features of two cases of intraventricular meningioma in childhood . Pediatr Neurosci 1989 ;15 :260-4.
10 . Hen DA, Shapiro K : Intracranial meningiomas of infancy, childhood and adolescence . Child's Brain 1980 ;7 :43-56 . 11 . Jelinek J, Smirniotopoulos JG, Parisi JE, Kanzer M : Lateral ventricular neoplasms of the brain : differential diagnosis based on clinical, CT, and MR findings . Am J Roentgenol 1990 ; 155 :365-72 .
3 . Chan RC, Thomson GB : Intracranial meningiomas in childhood . Surg Neurol 1984 ;21 :319-22 .
12 . Markwalder TM . Seiler RW, Markwalder RV, Huber P, Markwalder HM : Meningioma of the anterior part of the third ventricle in a child . Surg Neurol 1979 ;12 :29-32 .
4 . Chen HJ, Lui CC, Chen L : Meningioma in the anterior III ventricle in a child . Child's Nerv Syst 1986 ;2 :160-1 .
13 . Merten DF, Gooding CA, Newton TH, Malamud N : Meningiomas of childhood and adolescence . J Pediatr 1974 ;84 :696-700 .
5 . Davidson GS, Hope JK : Meningial tumors of childhood . Cancer 1989 ;63 :1205-10 .
14 . Parker DR, Eschelman DJ, Gibbens DT, Rabinov JD, O'Connor JF : Neuroradiology case of the day . Intraventricular meningioma . Am J Roentgenol 1991 ;156 :1307-8 .
6 . Deen HG, Scheithauer BW, Ebersold MJ : Clinical and pathological study of meningiomas of the first two decades of life . J Neurosurg 1982 ;56 :317-22 .
15 . Russell DS, Rubinstein LJ : Pathology of tumors of the nervous system . 5th ed . London: Edward Arnold, 1989:457 .
7 . Diaz P, Maillo A, Morales F, Gomez-Moreta JA, Hernadez J : Multiple meningiomas of the fourth ventricle in infancy : case report . Neurosurgery 1990 ;26 :1057-60 .
16 . Sano K, Wakai S, Ochiai C, Takamuro K : Characteristics of intracranial meningiomas in childhood . Child's Brain 1981 ; 8 :98-106.
S . Ferrante L, Acqui M, Artico M, Mastronardi L, Rocchi G, Fortuna A : Cerebral meningiomas in children . Child's Nerv Syst 1989 ;5 :83-6 .
17 . Teng P, Papatheodorou C : Suprachiasmal and intraventricular meningioma in a four-year-old child . J Neurosurg 1963 ; 20 :174-6.
9 . Hanieh A : Meningioma of the third ventricle Child's Nerv Syst 1989;5 :41-2 .
1S. Vassilouthis J, Ambrose JAE : Intraventricular meningioma in a child . Surg Neurol 1978 ;10 :105-7 .
Intraventricular Malignant Meningioma in a 6-Year-Old Child Spiros Sgouros, F.R .C.S ., A. Richard Walsh, F.R .C .S., and Peter Barber, M .R.C.Path . Department of Neurosurgery, Birmingham Children's Hospital ; and Department of Neuropathology, University of Birmingham Medical School, U .K .
Sgouros S, Walsh AR, Barber P . Intraventricular malignant meninginma in a 6-year-old child . Surg Neural 1994:42 :41-5 . The case of a malignant intraventricular meningioma in a 6-year-old child is reported . He presented with symptoms of raised intracranial pressure and computed tomography scan revealed an enhancing tumor at the region of the septum pellucidum . After surgical excision, histology showed appearances of meningioma with atypical features indicating malignant behavior . Childhood ; Intraventricular ; Malignant ; Menitr gioma; Third ventricle KEY WORDS :
Meningiomas are rare in childhood, accounting for 1 %2% of all intracranial tumors . Intraventricular meningiomas comprise 15%-17% of all childhood meningiomas . Several reports of intraventricular meningiomas in young children have been published, most of them the benign histologic type We report a case of intraventricular malignant meningioma in a 6-year-old child . Case Report A 6-year-old boy presented with a 4-week history of intermittent frontal headaches associated with vomiting . The headaches were present on awaking and were followed by vomiting soon after getting out of bed . As a result of the persistent vomiting, he had lost 3 kg of weight in the last month . He did not report any visual complaints . On examination he had marked papilloedema in both eyes with no other abnormal neurologic signs . The visual acuity was normal in both eyes . His symptoms and signs indicated raised intracranial pressure . A computed tomography (CT) scan of his head showed a large, 5 cm in diameter, well-defined mass in the region of the septum pellucidum, obliterating the third ventricle and obstructing the outlet of both lateral ventricles, causing biventricular hydrocephalus (Figure Address reprint requests to : S . Sgouros, F.R .C .S ., Department of Neurosurgery, Birmingham Children's Hospital, I .adywnod Middleway, Birmingham B16 SET, United Kingdom . Received August 2, 1993 ; accepted January 6, 1994 . 0 1994 by Elsevier Science Is, -
I A) . After administration of intravenous contrast, the lesion enhanced uniformly (Figure 1 B .) Through a bicoronal incision a right midline frontal craniotomy was performed, and the rumor was approached transcallosally . It was seen in the region of the septum and the roof of the third ventricle, separate from the corpus callosum . An apparent complete removal was achieved with the aid of the Cavitron Ultrasonic Surgical Aspirator . At the end of the procedure the two lateral ventricles and the third ventricle were in communication freely . An external ventricular drain, which had been introduced in the left lateral ventricle through a separate left frontal burr hole at the beginning of the operation, was left in place . The right lateral ventricle was drained through a separate ventricular drain . He made a good postoperative recovery and eventually did not require any cerebrospinal fluid diversion procedure . A postoperative CT scan confirmed the free communication of the lateral and third ventricles and demonstrated apparent complete surgical removal of the tumor . (Figure 2), although a magnetic resonance imaging (MRI) obtained at 3 and 6 months showed some residual tumor that had not increased in the size (Figure 3) . A subdural collection over the greater convexity of the right cerebral hemisphere was apparent in both examinations . As the patient was asymptomaric, no attempt of treatment was made . Twelve months after operation, the patient remains very well and follows the school activities as per normal . No treatment of the subdural collection is contemplated . Histologic examination showed the characteristic features of a meningioma . In most areas the tumor was composed of elongated, fibroblastic cells with fibrillary cytoplasm and a distinct whorling architectural pattern (Figure 4) . Small islands of tumor cells with a more syncytical or "meningorhelial" appearance were interspersed . The tumor cell nuclei were generally oval, with open chromatin and one to three small nucleoli . There was moderate nuclear pleomorphism . Mitoses were frequent in some areas, and numerous irregular foci of degeneration and necrosis were present (Figure 5) . Immunoperoxidase stains were positive for epithelial membrane antigen (EMA) (Figure 6) and negative for filial fibrillary acidic protein and cytokerarins . Electron miIi 9a ;m9a944 7 uu
Surg Ncurol 1994 ; 4 2 :41-5
Sgouros et nl
Figure 1 . (A) : Unaurtrarted bead CT "art rhau hig a large r/;a e-oaeR/ryi e lesion in the region of the septune pllncidinm, ~hstrnaing the antler of both lateral xemrvieler anti raeaeng liiornntrtaalar hsdrart/rbaku . (B): Contrarkv1 CT seam shonvng increased enha,,,.,nt of the septal truuor.
B
croscopy clearly showed intercellular desmosomal reactions (Figure 7) . The histologic appearance was felt to be that of an atypical or malignant meningioma_ Chromosomal analysis performed on white cells of a peripheral blood sample, using the method of in situ hybridization, failed to demonstrate deletion of the long arm of chromosome 22, thus ruling out the presence of neurofibromatosis . Discussion Intraventricular meningiomas are rare intracranial tumors . Meningiomas in general comprise 1%-2% of
all central nervous system (CNS) tumors in childhood [2,(x8,12-14,16,18] . Intraventricular meningiomas account for 15%-19% of all meningiomas in children [2,8,12-14,16,18] . They are believed to originate from the choroid plexuses, the vela choroidea, or the velum interpositum [2,12,14,15] . The majority are found in the trigone of the left lateral ventricle [2,5,6,1012,14,17,181, bur both the third and the fourth ventricles have been reported as sites of origin [1,4, 7,9,10,121 . The majority of meningiomas of childhood are of benign histology, although sarcomatous change has been reported to vary between 7%-38% [3,6,13,14,16] .
Figure 2 . Postoperative CI scan showing
Figure 3 . AIRI scan obtained at 6 ntoruhr pnstoperatit~ely shotubeg reu theel Cantor at the region of the septim, pellucideem .
Figure 4 . Lot, , power photomicrograph of the intravantricelar tumor. shoteing the u'horling pattern of spindle cell a gth foil of necrosis (arrows) . Hernatoxylin and Loan Sca/c bar 250 µnn .
Sure Neurol 1994 ;42 :4 1 -5
Sgouros or al
Figure 5 . Higher paaeer photomicrograph showing the cellular morphology ge the tumor with fibrillary cytoplast and open, rmnd to oral nuclei shoeing moderate pleomorphism . Mitosis (arrows) are frequent. Hentatoxylin and eosin . Scale bar = 75 ant .
The incidence of neurofibromatosis in children with intracranial meningiomas has been reported to be 19%21% [2,6,10,14] . There is a well-known association of NF-II with multiple CNS tumors that tend to be of malignant biological behavior, but in our patient this possibility has been ruled out with appropriate chromosomal analysis . We report a case of a malignant intraventricular meningioma in a child . Although the bulk of the tumor was in relation to the septum pellucidum, it is preFigure 6 . lnutu noperozhl ce staining of the tumor for- epithelial membrane antigen (EMfd) . cellular morphologZ is not well seen, but the density of sktin indicates that almart all the tumor celh bn this field .,to ;,; pestle sly . Scale bar = 15 0 pas.
sumed to have originated from the stroma of the choroid plexus of the third ventricle, as is commonly the case [1,4,9,12] . Malignant meningiomas in that situation are distinctly uncommon . Malignant meningiomas of childhood have a poor prognosis with a 5-year survival of 40% [6], and radiotherapy does not offer any significant improvement . It is of interest that despite its malignant histologic profile, the meningioma of our patient has so far behaved in a benign fashion, although the limited available follow-up calls for reserved predictions .
Figure 7 . Electron micrograph showing two intercellular junctions botweeo tumor cell.. Scale bar = 0 . 5 am .
Intraventricular Malignant Meningioma in Child
Surg Neurol
45
1994 ;4 2 :41-5
References 1 . Avman N, Dincer C : Meningiomas of the third ventricle . Acta Neurochirurgica 1978 ;42 :217-24 . 2 . Byard RW, Bourne AJ, Clark B, Hanieh A : Clinicopathological and radiological features of two cases of intraventricular meningioma in childhood . Pediatr Neurosci 1989 ;15 :260-4.
10 . Hen DA, Shapiro K : Intracranial meningiomas of infancy, childhood and adolescence . Child's Brain 1980 ;7 :43-56 . 11 . Jelinek J, Smirniotopoulos JG, Parisi JE, Kanzer M : Lateral ventricular neoplasms of the brain : differential diagnosis based on clinical, CT, and MR findings . Am J Roentgenol 1990 ; 155 :365-72 .
3 . Chan RC, Thomson GB : Intracranial meningiomas in childhood . Surg Neurol 1984 ;21 :319-22 .
12 . Markwalder TM . Seiler RW, Markwalder RV, Huber P, Markwalder HM : Meningioma of the anterior part of the third ventricle in a child . Surg Neurol 1979 ;12 :29-32 .
4 . Chen HJ, Lui CC, Chen L : Meningioma in the anterior III ventricle in a child . Child's Nerv Syst 1986 ;2 :160-1 .
13 . Merten DF, Gooding CA, Newton TH, Malamud N : Meningiomas of childhood and adolescence . J Pediatr 1974 ;84 :696-700 .
5 . Davidson GS, Hope JK : Meningial tumors of childhood . Cancer 1989 ;63 :1205-10 .
14 . Parker DR, Eschelman DJ, Gibbens DT, Rabinov JD, O'Connor JF : Neuroradiology case of the day . Intraventricular meningioma . Am J Roentgenol 1991 ;156 :1307-8 .
6 . Deen HG, Scheithauer BW, Ebersold MJ : Clinical and pathological study of meningiomas of the first two decades of life . J Neurosurg 1982 ;56 :317-22 .
15 . Russell DS, Rubinstein LJ : Pathology of tumors of the nervous system . 5th ed . London: Edward Arnold, 1989:457 .
7 . Diaz P, Maillo A, Morales F, Gomez-Moreta JA, Hernadez J : Multiple meningiomas of the fourth ventricle in infancy : case report . Neurosurgery 1990 ;26 :1057-60 .
16 . Sano K, Wakai S, Ochiai C, Takamuro K : Characteristics of intracranial meningiomas in childhood . Child's Brain 1981 ; 8 :98-106.
S . Ferrante L, Acqui M, Artico M, Mastronardi L, Rocchi G, Fortuna A : Cerebral meningiomas in children . Child's Nerv Syst 1989 ;5 :83-6 .
17 . Teng P, Papatheodorou C : Suprachiasmal and intraventricular meningioma in a four-year-old child . J Neurosurg 1963 ; 20 :174-6.
9 . Hanieh A : Meningioma of the third ventricle Child's Nerv Syst 1989;5 :41-2 .
1S. Vassilouthis J, Ambrose JAE : Intraventricular meningioma in a child . Surg Neurol 1978 ;10 :105-7 .