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Malignant intraventricular meningioma with spinal metastasis through the cerebrospinal fluid
Surg Neural
213
1989;32:2 13-8
Malignant Through Kazuko
Intraventricular the Cerebrospinal
Kamiya,
Departments
Meningioma
M.D., Tetsuji
of Neurosurgery
Inagawa,
Fluid M.D., and Ren Nagasako,
fluid.
A 67-year-old
man developed a malignant meningioma of the right lateral ventricle at the trigone, for which he underwent total removal of the tumor and local irradiation. Six months after the operation, a spinal tumor developed and subtotal removal of the tumor was made. The spinal tumor was considered to be a metastasis via the cerebrospinal fluid in view of its histologic identity with the primary tumor, histologic findings of malignancy, absence of von Recklinghausen’s disease, and lack of tumor attachment to the dura. KEY WORDS: Malignant meningioma; Intraventricular gioma; Metastasis; Cerebrospinal fluid
menin-
Meningioma is a tumor which invariably faces the cerebrospinal fluid (CSF), because it arises from arachnoid villi, tela choroidea, or leptomeninges. Most meningioma cases are benign, but some are malignant. Malignant meningiomas sometimes metastasize to the extraneural organs, but the seeding of meningioma cells through the CSF pathways with the establishment of metastatic implants is extremely rare. This paper presents a case of intraventricular malignant meningioma with metastasis to the spinal cord through the CSF.
Case Report admitted
to Shimane
disturbance tion, and admission,
right-handed
Prefectural
of consciousness,
M.D.
and Clinical Pathology, Shimane Prefectural Central Hospital, Izumo, Shimane, Japan
Kamiya K, Inagawa T, Nagasako R. Malignant intraventricular meningioma with spinal metastasis through the cerebrospinal Surg Neurol 1989;32:213-8.
The case is a 67-year-old,
with Spinal Metastasis
Central
mydriasis,
man who was Hospital
with
ataxic respira-
urinary incontinence. One month before he developed progressive left hemiparesis.
Addressreprint requests to: Kazuko Kamiya, M.D., Department of Neurosurgery, Shimane Prefectural Central Hospital, 1 I6 Imaichicho, Izumo, Shimane, 693, Japan. Received September 9, 1988; accepted March 8, 1989. 0 1989 by Elsevier Science Publishing Co., Inc.
On admission, the patient was confused, with left hemiplegia and urinary incontinence. Plain computed tomography (CT) scanning on admission showed a widespread low density area in the right temporoparietal lobe, dilatation of the posterior horn of the right lateral ventricle, and a high-density area suggesting calcification adjacent to the posterior wall of the right posterior horn. The midline structure was shifted to the left side (Figure 1). Enhanced CT scanning demonstrated enhanced mass approximately 6.5 cm in maximum diameter around the calcification (Figure 2). Carotid angiography showed a coarse stain feeding from the right middle cerebral artery, right posterior cerebral artery, and right anterior cerebral artery corresponding to the enhanced lesion on enhanced CT scanning. A right temporoparietal craniotomy with a transcortical approach was performed and the tumor in the right trigone was totally removed (Simpson 1) IS]. The surgically resected specimens revealed a hypercellular, extensively necrotic meningioma with a high mitotic index. The tumor was diagnosed as malignant meningioma (Figure 3). Postoperatively, local irradiation of 50 Gy was given to a 12 cm X 12 cm field. Thereafter, the patient recovered uneventfully and was discharged with mild disorientation. The patient did well for 6 months after operation, but thereafter had developed progressive weakness of both lower extremities and was readmitted. On physical examination at time of second admission he was noted to have paraplegia, anesthesia of the lower extremities, hypoesthesia below the right T9 level, and hyperalgesia and dysesthesia below the left T9 level. None of the characteristic dermatologic findings of von Recklinghausen’s disease could be observed. A myelogram showed complete obstruction at the TlO-T12 levels. Metrizamide CT cisternography of the spine delineated a filling defect in the right ventral side of the T9 level. Cytology of the CSF revealed no tumor cells, a protein value of 700 mg/dL, and a glucose value of 5 1 mg/dL. The spinal tumor was removed through T9-TlO laminectomy (Simpson 2) [S]. The epidural space and dura mater were not remarkable. The extramedullary tumor, 0090-3019/89/$3.50
214
Surg Neurol 1989;32:213-8
Kamiya
et al
ular meningiomas in particular are rare [6]. Malignant meningiomas sometimes metastasize to the extraneural organs, but it is strange that seeding of meningioma cells through the CSF pathways with the establishment of metastatic implants is extremely rare though they are in contact with the CSF [4]. In view of the physiologic pathways of the CSF, the risk of intraventricular meningiomas to metastasize should be high. However, metastasis of intraventricular meningiomas via the CSF is rare. Two cases have been reported in the literature, to which we have added our present case (Table 1). When meningioma is observed at multiple sites, it is difficult to distinguish whether the focus is a multiple tumor or a metastatic tumor. The overall incidence of multiple meningiomas is reported to range between 1% and 2 %. Many of the multiple meningiomas have been observed in patients with von Recklinghausen’s disease. Kepes [l) has collected 12 cases of metastatic meningiomas through the CSF pathways, 7 of which were histologically malignant. Six cases had metastatic foci in the spinal region or cauda equina. Miller and Ramsden {3} have suggested that the dynamics of the system might prevent settlement of the cells, but they have assumed that the existence of an antimeningioma immunologic sys-
Figure 1. Plain CT scan on admission shows widespread low-density area in the right temporoparietal lobes, dilatation of the posterior horn of the right lateral ventricle, and high-density area suggesting calcification adjacent to the posterior wall of the right posterior born. The midline structure is shifted to the left side.
which was grayish-violet, soft, approximately 2 cm X 1 cm, and smooth, was encountered without attachment to the dura. The tumor was located at the right side and had invaded the spinal cord (Figure 4). One of the right nerve roots completely attached to the mass was resected with the tumor. The surgically resected specimen of the second operation was diagnosed as malignant meningioma. The histologic features of this mass were identical to those of malignant parts of the primary tumor (Figure 5). Postoperatively, local irradiation of 40 Gy was given to the T7-TlO level of the spine. There was no change in paraplegia, but some improvement in sensation was observed. The patient was referred to another hospital. He thereafter deteriorated and died of pneumonia 6 months after the second operation. Autopsy was not performed.
Discussion Most meningiomas are benign with less than 10% having malignant features. Only 0.7% of the meningiomas occur in the ventricles, and malignant intraventric-
Figure 2. Enhanced CT scan demonstrates an enhanced mass measuring approximately 6.5 cm in diameter around the calcification.
Figure 3. The surgical resection specimen reveals whorl formations of jbroblastic reih with psammoma body and calcification (A),and a hypercellular meningioma with a high mitotic index (B). Hematoxylin and eosin stains ~66.
216
Surg Neural 1989;32:213-8
Kamiya et al
Figure 4. Operative photograph of the spinal tumor of the T9-TIO level. The intradural-extramedullary tumor is located at dorsal side of the spinal CWd.
tern is unlikely within the subarachnoid space. Meningioma cells may not metastasize unless they have formed jointed fragments {3]. In our patient, the spinal tumor was considered to be a metastatic focus via the CSF in view of the findings that the histologic findings of the intraventricular tumor and the spinal tumor were identical with both being malignant, that the spinal tumor did not show any attachment to the dura and was located on the surface of the spinal cord, and that there were no signs of von Recklinghausen’s disease.
Table
1. lntvaventricular
with Metastases
Primary
Authors Kleinschmidt-DeMasters and Avakian {2] Strenger et al [6J Kamiya et al [present
Meningiomas
study]
site
Does surgery have an effect on the spread of meningioma by the CSF pathways? In view of the large number of meningioma cases in which surgical removal was made, the incidence of metastatic meningiomas through the CSF is extremely small, even if majority of benignancy contribute to this incidence. Contrary, malignant meningiomas can spread without surgical intervention. Four of Kepes’ Cl] 12 cases had no surgery. Surgical intervention may not be an important factor in the pathogenesis of metastasis of meningiomas through the CSF.
to Distant Areas of the Central
Nervous System via the Cerebrospinal
Surgery
Site of metastases
Left lateral ventricle
+
Third ventricle Right lateral ventricle
+ +
Medulla, pineal gland, cerebellum, spinal cord Cerebrum (multiple) Spinal cord
Duration
to metastasis
Fluid
Histology
20 months
Malignant
6 weeks 7 months
Malignant Malignant
B Figure 5. The surgically resected specimen of the second tumor of the spinal cord. It ir virturllly identical to that of the malignant Hematoxylin and eosin stains (A) ~80, (B) x25. intrauentricuhr tumor (A),and it shows extensive necrosis (B).
part of the primav
218
Kamiya
Surg Neurol 1989;32:213-8
References
metastases and seeding of the subarachnoid cles. Path Europ 1972;7:167-75.
Kepes JJ. Meningiomas. Biology, pathology, and differential nosis. New York: Masson, 1982;27-8, 190-200. Kleinschmidt-DeMasters caused by CSF metastasis ropathol 1985;4:2 14-9. Miller
AA, Ramsden
BK, Avakian JJ. WaUenberg from malignant meningioma.
F. Malignant
meningioma
diag-
syndrome Clin Neu-
with extracranial
Russell system.
et
al
space and the ventri-
DS, Rubinstein LJ. Pathology of tumors of the nervous Baltimore: Williams & Wilkins, 1977;68-74, 89-91.
Simpson D. The recurrence of intracranial meningioma after surgical treatment. J Neural Neurosur & Psychiat 1957;20:22-39. Strenger SW, Huang YP, within the third ventricle: 20:465-8.
Sachdev VP. Malignant meningioma a case report. Neurosurgery 1987;
213
1989;32:2 13-8
Malignant Through Kazuko
Intraventricular the Cerebrospinal
Kamiya,
Departments
Meningioma
M.D., Tetsuji
of Neurosurgery
Inagawa,
Fluid M.D., and Ren Nagasako,
fluid.
A 67-year-old
man developed a malignant meningioma of the right lateral ventricle at the trigone, for which he underwent total removal of the tumor and local irradiation. Six months after the operation, a spinal tumor developed and subtotal removal of the tumor was made. The spinal tumor was considered to be a metastasis via the cerebrospinal fluid in view of its histologic identity with the primary tumor, histologic findings of malignancy, absence of von Recklinghausen’s disease, and lack of tumor attachment to the dura. KEY WORDS: Malignant meningioma; Intraventricular gioma; Metastasis; Cerebrospinal fluid
menin-
Meningioma is a tumor which invariably faces the cerebrospinal fluid (CSF), because it arises from arachnoid villi, tela choroidea, or leptomeninges. Most meningioma cases are benign, but some are malignant. Malignant meningiomas sometimes metastasize to the extraneural organs, but the seeding of meningioma cells through the CSF pathways with the establishment of metastatic implants is extremely rare. This paper presents a case of intraventricular malignant meningioma with metastasis to the spinal cord through the CSF.
Case Report admitted
to Shimane
disturbance tion, and admission,
right-handed
Prefectural
of consciousness,
M.D.
and Clinical Pathology, Shimane Prefectural Central Hospital, Izumo, Shimane, Japan
Kamiya K, Inagawa T, Nagasako R. Malignant intraventricular meningioma with spinal metastasis through the cerebrospinal Surg Neurol 1989;32:213-8.
The case is a 67-year-old,
with Spinal Metastasis
Central
mydriasis,
man who was Hospital
with
ataxic respira-
urinary incontinence. One month before he developed progressive left hemiparesis.
Addressreprint requests to: Kazuko Kamiya, M.D., Department of Neurosurgery, Shimane Prefectural Central Hospital, 1 I6 Imaichicho, Izumo, Shimane, 693, Japan. Received September 9, 1988; accepted March 8, 1989. 0 1989 by Elsevier Science Publishing Co., Inc.
On admission, the patient was confused, with left hemiplegia and urinary incontinence. Plain computed tomography (CT) scanning on admission showed a widespread low density area in the right temporoparietal lobe, dilatation of the posterior horn of the right lateral ventricle, and a high-density area suggesting calcification adjacent to the posterior wall of the right posterior horn. The midline structure was shifted to the left side (Figure 1). Enhanced CT scanning demonstrated enhanced mass approximately 6.5 cm in maximum diameter around the calcification (Figure 2). Carotid angiography showed a coarse stain feeding from the right middle cerebral artery, right posterior cerebral artery, and right anterior cerebral artery corresponding to the enhanced lesion on enhanced CT scanning. A right temporoparietal craniotomy with a transcortical approach was performed and the tumor in the right trigone was totally removed (Simpson 1) IS]. The surgically resected specimens revealed a hypercellular, extensively necrotic meningioma with a high mitotic index. The tumor was diagnosed as malignant meningioma (Figure 3). Postoperatively, local irradiation of 50 Gy was given to a 12 cm X 12 cm field. Thereafter, the patient recovered uneventfully and was discharged with mild disorientation. The patient did well for 6 months after operation, but thereafter had developed progressive weakness of both lower extremities and was readmitted. On physical examination at time of second admission he was noted to have paraplegia, anesthesia of the lower extremities, hypoesthesia below the right T9 level, and hyperalgesia and dysesthesia below the left T9 level. None of the characteristic dermatologic findings of von Recklinghausen’s disease could be observed. A myelogram showed complete obstruction at the TlO-T12 levels. Metrizamide CT cisternography of the spine delineated a filling defect in the right ventral side of the T9 level. Cytology of the CSF revealed no tumor cells, a protein value of 700 mg/dL, and a glucose value of 5 1 mg/dL. The spinal tumor was removed through T9-TlO laminectomy (Simpson 2) [S]. The epidural space and dura mater were not remarkable. The extramedullary tumor, 0090-3019/89/$3.50
214
Surg Neurol 1989;32:213-8
Kamiya
et al
ular meningiomas in particular are rare [6]. Malignant meningiomas sometimes metastasize to the extraneural organs, but it is strange that seeding of meningioma cells through the CSF pathways with the establishment of metastatic implants is extremely rare though they are in contact with the CSF [4]. In view of the physiologic pathways of the CSF, the risk of intraventricular meningiomas to metastasize should be high. However, metastasis of intraventricular meningiomas via the CSF is rare. Two cases have been reported in the literature, to which we have added our present case (Table 1). When meningioma is observed at multiple sites, it is difficult to distinguish whether the focus is a multiple tumor or a metastatic tumor. The overall incidence of multiple meningiomas is reported to range between 1% and 2 %. Many of the multiple meningiomas have been observed in patients with von Recklinghausen’s disease. Kepes [l) has collected 12 cases of metastatic meningiomas through the CSF pathways, 7 of which were histologically malignant. Six cases had metastatic foci in the spinal region or cauda equina. Miller and Ramsden {3} have suggested that the dynamics of the system might prevent settlement of the cells, but they have assumed that the existence of an antimeningioma immunologic sys-
Figure 1. Plain CT scan on admission shows widespread low-density area in the right temporoparietal lobes, dilatation of the posterior horn of the right lateral ventricle, and high-density area suggesting calcification adjacent to the posterior wall of the right posterior born. The midline structure is shifted to the left side.
which was grayish-violet, soft, approximately 2 cm X 1 cm, and smooth, was encountered without attachment to the dura. The tumor was located at the right side and had invaded the spinal cord (Figure 4). One of the right nerve roots completely attached to the mass was resected with the tumor. The surgically resected specimen of the second operation was diagnosed as malignant meningioma. The histologic features of this mass were identical to those of malignant parts of the primary tumor (Figure 5). Postoperatively, local irradiation of 40 Gy was given to the T7-TlO level of the spine. There was no change in paraplegia, but some improvement in sensation was observed. The patient was referred to another hospital. He thereafter deteriorated and died of pneumonia 6 months after the second operation. Autopsy was not performed.
Discussion Most meningiomas are benign with less than 10% having malignant features. Only 0.7% of the meningiomas occur in the ventricles, and malignant intraventric-
Figure 2. Enhanced CT scan demonstrates an enhanced mass measuring approximately 6.5 cm in diameter around the calcification.
Figure 3. The surgical resection specimen reveals whorl formations of jbroblastic reih with psammoma body and calcification (A),and a hypercellular meningioma with a high mitotic index (B). Hematoxylin and eosin stains ~66.
216
Surg Neural 1989;32:213-8
Kamiya et al
Figure 4. Operative photograph of the spinal tumor of the T9-TIO level. The intradural-extramedullary tumor is located at dorsal side of the spinal CWd.
tern is unlikely within the subarachnoid space. Meningioma cells may not metastasize unless they have formed jointed fragments {3]. In our patient, the spinal tumor was considered to be a metastatic focus via the CSF in view of the findings that the histologic findings of the intraventricular tumor and the spinal tumor were identical with both being malignant, that the spinal tumor did not show any attachment to the dura and was located on the surface of the spinal cord, and that there were no signs of von Recklinghausen’s disease.
Table
1. lntvaventricular
with Metastases
Primary
Authors Kleinschmidt-DeMasters and Avakian {2] Strenger et al [6J Kamiya et al [present
Meningiomas
study]
site
Does surgery have an effect on the spread of meningioma by the CSF pathways? In view of the large number of meningioma cases in which surgical removal was made, the incidence of metastatic meningiomas through the CSF is extremely small, even if majority of benignancy contribute to this incidence. Contrary, malignant meningiomas can spread without surgical intervention. Four of Kepes’ Cl] 12 cases had no surgery. Surgical intervention may not be an important factor in the pathogenesis of metastasis of meningiomas through the CSF.
to Distant Areas of the Central
Nervous System via the Cerebrospinal
Surgery
Site of metastases
Left lateral ventricle
+
Third ventricle Right lateral ventricle
+ +
Medulla, pineal gland, cerebellum, spinal cord Cerebrum (multiple) Spinal cord
Duration
to metastasis
Fluid
Histology
20 months
Malignant
6 weeks 7 months
Malignant Malignant
B Figure 5. The surgically resected specimen of the second tumor of the spinal cord. It ir virturllly identical to that of the malignant Hematoxylin and eosin stains (A) ~80, (B) x25. intrauentricuhr tumor (A),and it shows extensive necrosis (B).
part of the primav
218
Kamiya
Surg Neurol 1989;32:213-8
References
metastases and seeding of the subarachnoid cles. Path Europ 1972;7:167-75.
Kepes JJ. Meningiomas. Biology, pathology, and differential nosis. New York: Masson, 1982;27-8, 190-200. Kleinschmidt-DeMasters caused by CSF metastasis ropathol 1985;4:2 14-9. Miller
AA, Ramsden
BK, Avakian JJ. WaUenberg from malignant meningioma.
F. Malignant
meningioma
diag-
syndrome Clin Neu-
with extracranial
Russell system.
et
al
space and the ventri-
DS, Rubinstein LJ. Pathology of tumors of the nervous Baltimore: Williams & Wilkins, 1977;68-74, 89-91.
Simpson D. The recurrence of intracranial meningioma after surgical treatment. J Neural Neurosur & Psychiat 1957;20:22-39. Strenger SW, Huang YP, within the third ventricle: 20:465-8.
Sachdev VP. Malignant meningioma a case report. Neurosurgery 1987;