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Extracranial metastasis of glioblastoma with sarcomatous component
Surg Neurol 1985;24:641-5
641
Extracranial Metastasis of Glioblastoma with Sarcomatous Component Hiroaki Yokoyama, M.D., Hirohisa Ono, M.D., Kazuo Mori, M.D., Masao Kishikawa, M.D., and Masataka Kihara, M.D. Departments of Neurosurgery and Pathology, Scientific Data Center of Atomic Bomb Disaster, and Department of Second Division of Pathology, Nagasaki University, Nagasaki, Japan
Yokoyama H, Ono H, Mori K, Kishikawa M, Kihara M. Extracranial metastasis of glioblastoma with sarcomatous component. Surg Neurol 1985;24:641-5.
A case of glioblastoma with sarcomatous component is presented. Craniotomy was performed with total resection of the left occipital tumor. The patient received radiotherapy postoperatively but developed extracranial metastases only two months after the first surgical procedure. KEY WORDS: G l i o b l a s t o m a with sarcomatous c o m p o n e n t ; Extracranial metastasis
It is well known that extracranial metastasis is an unusual occurrence. Some authors attribute the rarity of metastasis of the primary brain tumors to an inability of tumor cells to proliferate in other organs during the short postoperative life span of the patient (8,11). This is a case report of glioblastoma with sarcomatous component with metastases to extracranial organs appearing only two months after resection o f the primary tumor. Case Report The patient, a 22-year-old Japanese woman, was admitted to the Department o f Neurosurgery in Nagasaki University Hospital on October 5, 1981 with a 3-month history of occipital headache, nausea, and vomiting. Neurological examination showed a right homonymous hemianopsia and a right Babinski sign. Left carotid (Figure 1A) and vertebral (Figure 1B) arteriograms furnished evidence of a large tumor mass in the left occipital region. Computed tomography also demonstrated a large mass with marked enchancement (Figure 2).
Address reprint requests to: Dr. Hiroaki Yokoyama, Department of Neurosurgery, Nagasaki Industrial Hospital, 152 Setogoe-cho, Sasebo, Japan 857-01.
© 1985 by Elsevier Science Publishing Co., Inc.
On October 14, 1981, a left parietooccipital craniotomy was performed. The tumor was not adherent to the dura mater and relatively circumscribed with surrounding brain tissue. It was totally resected under direct vision. The postoperative course was uneventful and the visual field defect improved to a right lower quandrantanopsia. Radiotherapy (total dose of 4000 rads) was given over a period of 4 weeks. However, computed tomography on N o v e m b e r 10, 1981, showed recurrence of the brain tumor which gradually increased in size. On December 9, at the termination of radiotherapy, the size of the tumor seemed to be the same as that preoperatively (Figure 3). She was discharged on D e c e m b e r 11. Twenty-two days later, she noted bulging and tenderness over the craniotomy site and was readmitted. Biopsy disclosed a subcutaneous mass with mucinous component and in apparent continuity with the epidural space through the burr holes. X-ray examination of the chest was normal at the time of the second admission. She developed headaches, vomiting, and disturbance in level of consciousness. The size of the occipital subcutaneous mass increased progressively, and left auricular and cervical masses were noted. A third operation was performed on January 11, 1982. Subcutaneous tumor, protruded through the burr holes, invaded the skin and contained very mucinous material. Both epidural and intracerebral tumors contained mucinous materials and were tightly adherent to the dura mater. These tumors were subtotally resected. A cervical tumor was also removed. The postoperative course was smooth until she complained of dyspnea eight days after the third operation. X-ray films of the chest demonstrated complete opacity on the left. Left thoracentesis revealed bloody fluid with malignant tumor cells. She received vincristine, 6-MP, and endoxan but her condition deteriorated progressively, and she died of respiratory insufficiency on February 2, 1982. At autopsy, the brain was edematous, weighed 1330 g, and contained a large tumor with myxomatous com0090-3019/85/$3.30
642
Surg Neurol 1985;24:641-5
Yokoyama et al
Figure 1. Angiograms of the left carotid artery (A) and vertebral artery (B) showed a huge tumor in the left occipital lobe.
Figure 2. Computed tomography scanning demonstrated the low density area in the left occipital lobe with mass effect (top) and marked enchancement after injection of the contrast medium (bottom).
Gliosarcoma with Extracranial Metastasis
ponents that had replaced the left occipital lobe. The tumor extended through the dura mater and craniotomy site into the occipital skin. Metastases were found in the lungs, pleura, hilar lymph nodes, cervical lymph nodes, sternal bone marrow, and the liver. Microscopic findings were similar in all specimens (material obtained at three operations and at autopsy). The appearance was highly variable: 1) diffuse and sparse proliferations of astrocytes with hyperchromatic pleomorphic nuclei; 2) spindle and pleomorphic sarcomatous cells with abundant collagen; 3) myxomatous areas with giant cells containing bizarre nuclei; 4) transitional areas with sarcomatous appearance; 5) vascular endothelial proliferations; and 6) areas of necrosis and hemorrhage (Figure 4). In phosphotungstic acid-hematoxylin and glial fibril acid protein (GFAP) (Figure 5) preparations, anaplastic cells were noted. The final diagnosis was glioblastoma with sarcomatous components.
Discussion Brain tumors rarely metastasize to extracranial organs. Some authors attribute the rarity of distant metastases
Surg Neurol 1985;24:641-5
643
Figure 3. Computed tomogram on November I0 and December 9 showed the large mass with contrast medium enchancement indicating recurrence of the tumor.
to the absence of intracranial lymphatic channels [1,3], while others believe that postoperative life span is too short to develop enough metastatic masses in other organs [8,11]. It is also believed that surgical procedure plays a major role in entrance of tumor cells into the extracranial blood vessels and lymphatics. The case reported here is very rare because the remote metastases occurred within only 2 months of the first operation. T h e r e are two possibilities: 1) introduction into the venous system by either direct invasion or during surgery; or 2) implantation of tumor cells upon the dura mater and scalp during an operative procedure with subsequent hematogenous or lymphatic spread. In our case, the finding of massive extracranial infiltration of the soft tissue adjacent to the operative site may indicate that tumoral dissemination occurred in the operative wound and led to invasion of the soft tissue, lymphatics, and extracranial vessels. Extracranial metastases o f glioblastoma with sarco-
644
Surg Neurol 1985;24:641-5
Yokoyama et al
Figure 4. Histological examination of brain tumor revealed anaplastic cells and spindle cells. Transitional area from vascular components to sarcomatous ones was also observed (H & E stain, x 37).
matous component, gliosarcoma by some authors [4,5,10], have now been d o c u m e n t e d in 11 cases in the world literatures (Table 1). Smith et al [9] described a series o f seven cases of gliosarcoma that had metastasized to extracranial organs. These seven cases constitute about one-third of the cases of metastasizing glioma at the A r m e d Forces Institute o f Pathology. Morantz et al [7] reported that the ability of the gliosarcoma to metastasize is much greater than that of other malignant tumors. Age distribution in the reported cases ranged from 6.5 to 64 years, with an average of 43.2 years. Eight
Figure 5. Neoplastic cells with GFAPpositive cytoplasm were noted (GFAP stain, × 150).
cases were men and three were women. In all cases the location o f the primary tumor was supratentorial--temporal in eight cases, frontal in two cases, and occipital in the present case. Metastases occurred in the lung (seven cases--64%), liver (six cases--54%), lymph nodes (two c a s e s - - 1 8 % ) , and in the adrenal gland and vertebral body (one case each). Survival time o f gliosarcoma from the time of the first operation ranged from 4 months to 15 months. This is an average of 8.4 months which is shorter than that of other gliomas with distant metastases.
Gliosarcoma with Extracranial Metastasis
Surg Neurol 1985;24:641-5
645
Table 1. Previously Recorded Cases of Glioblastoma with Sarcomatous Component with Extracranial Metastases Length of Patient's
Case
Author(s)
1 2 3 4 5 6 7 8 9 10 11
Ehrenreich et al 2 Feigin et al ~ Garret c' Smith et al v Smith et al v Smith et al 9 Smith et al ') Smith et al 9 Smith et al v Smith et ar ) Present case
(1958) (1958) (1958) (1969) (1969) (1969) (1969) (1969) (1969) (1969) (1983)
Location of
Postoperative
Postoperative
Age & Sex
Primary
Radiation
Survival
44 M 61/2 M 55 F 49 M 44 M 58 M 63 M 64 M 61/2 M 63 F 22 F
R--temporal L--temporal R--temporal R--frontal R--temporal L--temporal R--temporal R--temporal L--temporal L--frontal L--occipital
Yes Yes Yes Yes Yes Yes
Yes
8 15 9 8 6 6 8 10 7 11 4
mos mos mos mos mos mos mos mos mos mos mos
Sites of Metastases Lung Lung Cervical Lymph N o d e Liver Liver Liver Lung, Liver & Adrenal Gland Lung & Liver Lung Vertebral Body & Lung Cervical Lymph Node, Lung & Liver
Abbreviations: mos = months; M ~ male, F = female.
Morantz et al [7] reported that the prognosis ofgliosarcoma, in general, is no worse than that of the anaplastic astrocytoma, but they had no cases with distant metastases. We believe that the prognosis is worse than that of other gliomas with extracranial metastases. Although the sarcomatous c o m p o n e n t might play some role in prognosis, the reason is still unknown.
4. Feigin I, Gross SW. Sarcoma arising in glioblastoma of the brain. A m J Pathol 1955;31:633-53.
References
8. Perry RE. Extracranial metastasis in a case of intracranial ependymoma. Arch Pathol 1957;64:337-41.
1. Dubois-Ferriere H. Drainage paths of intracranial fluids (lymph and CSF) and rarity of extracranial metastases of cerebral tumors. Annls Anat Path 1940;16:1081-114.
5. Feigin I, Allen LB, Lipkin L, Gross SW. T h e endothelial hyperplasia of the cerebral blood vessels with brain tumors and its sarcomatous transformation. Cancer 1958;11:264-7. 6. Garret R. Glioblastoma and fibrosarcoma of the brain with extracranial metastases. Cancer 1958; 11:888-894. 7. Morantz RA, Feigin I, R a n s o h o f f J . Gliosarcomas: A clinical and pathological survey of 24 cases. J N e u r o s u r g 1976;45:398-408.
9. Smith DR, H a r d m a n J N , Earle KM. Contiguous glioblastoma multiform and fibrisarcoma with extracranial metastases. Cancer 1969;24:270-7.
2. E h r e n r e i c h T H , DevlinJF. A complex ofglioblastoma and spindle cell sarcoma with pulmonary metastases. Arch Pathol 1958;66:536-49.
10. Stroebe H. U e b e r Entstehung und Bau der Gehirngliome. Beitr Path Anat 1895;18:405-86.
3. Elvidge A, Penfield W, C o n e W. T h e gliomas of the central nervous system. A study of two h u n d r e d and ten verified cases. Res Pubis Ass N e r v Ment Dis 1937;16:107-81.
11. Winkelman N W Jr, Cassel C, Schlesinger B~ lntracranial tumors with extra-cranial metastases. J N e u r o p a t h Exp Neurol 1952;11:149-66.
641
Extracranial Metastasis of Glioblastoma with Sarcomatous Component Hiroaki Yokoyama, M.D., Hirohisa Ono, M.D., Kazuo Mori, M.D., Masao Kishikawa, M.D., and Masataka Kihara, M.D. Departments of Neurosurgery and Pathology, Scientific Data Center of Atomic Bomb Disaster, and Department of Second Division of Pathology, Nagasaki University, Nagasaki, Japan
Yokoyama H, Ono H, Mori K, Kishikawa M, Kihara M. Extracranial metastasis of glioblastoma with sarcomatous component. Surg Neurol 1985;24:641-5.
A case of glioblastoma with sarcomatous component is presented. Craniotomy was performed with total resection of the left occipital tumor. The patient received radiotherapy postoperatively but developed extracranial metastases only two months after the first surgical procedure. KEY WORDS: G l i o b l a s t o m a with sarcomatous c o m p o n e n t ; Extracranial metastasis
It is well known that extracranial metastasis is an unusual occurrence. Some authors attribute the rarity of metastasis of the primary brain tumors to an inability of tumor cells to proliferate in other organs during the short postoperative life span of the patient (8,11). This is a case report of glioblastoma with sarcomatous component with metastases to extracranial organs appearing only two months after resection o f the primary tumor. Case Report The patient, a 22-year-old Japanese woman, was admitted to the Department o f Neurosurgery in Nagasaki University Hospital on October 5, 1981 with a 3-month history of occipital headache, nausea, and vomiting. Neurological examination showed a right homonymous hemianopsia and a right Babinski sign. Left carotid (Figure 1A) and vertebral (Figure 1B) arteriograms furnished evidence of a large tumor mass in the left occipital region. Computed tomography also demonstrated a large mass with marked enchancement (Figure 2).
Address reprint requests to: Dr. Hiroaki Yokoyama, Department of Neurosurgery, Nagasaki Industrial Hospital, 152 Setogoe-cho, Sasebo, Japan 857-01.
© 1985 by Elsevier Science Publishing Co., Inc.
On October 14, 1981, a left parietooccipital craniotomy was performed. The tumor was not adherent to the dura mater and relatively circumscribed with surrounding brain tissue. It was totally resected under direct vision. The postoperative course was uneventful and the visual field defect improved to a right lower quandrantanopsia. Radiotherapy (total dose of 4000 rads) was given over a period of 4 weeks. However, computed tomography on N o v e m b e r 10, 1981, showed recurrence of the brain tumor which gradually increased in size. On December 9, at the termination of radiotherapy, the size of the tumor seemed to be the same as that preoperatively (Figure 3). She was discharged on D e c e m b e r 11. Twenty-two days later, she noted bulging and tenderness over the craniotomy site and was readmitted. Biopsy disclosed a subcutaneous mass with mucinous component and in apparent continuity with the epidural space through the burr holes. X-ray examination of the chest was normal at the time of the second admission. She developed headaches, vomiting, and disturbance in level of consciousness. The size of the occipital subcutaneous mass increased progressively, and left auricular and cervical masses were noted. A third operation was performed on January 11, 1982. Subcutaneous tumor, protruded through the burr holes, invaded the skin and contained very mucinous material. Both epidural and intracerebral tumors contained mucinous materials and were tightly adherent to the dura mater. These tumors were subtotally resected. A cervical tumor was also removed. The postoperative course was smooth until she complained of dyspnea eight days after the third operation. X-ray films of the chest demonstrated complete opacity on the left. Left thoracentesis revealed bloody fluid with malignant tumor cells. She received vincristine, 6-MP, and endoxan but her condition deteriorated progressively, and she died of respiratory insufficiency on February 2, 1982. At autopsy, the brain was edematous, weighed 1330 g, and contained a large tumor with myxomatous com0090-3019/85/$3.30
642
Surg Neurol 1985;24:641-5
Yokoyama et al
Figure 1. Angiograms of the left carotid artery (A) and vertebral artery (B) showed a huge tumor in the left occipital lobe.
Figure 2. Computed tomography scanning demonstrated the low density area in the left occipital lobe with mass effect (top) and marked enchancement after injection of the contrast medium (bottom).
Gliosarcoma with Extracranial Metastasis
ponents that had replaced the left occipital lobe. The tumor extended through the dura mater and craniotomy site into the occipital skin. Metastases were found in the lungs, pleura, hilar lymph nodes, cervical lymph nodes, sternal bone marrow, and the liver. Microscopic findings were similar in all specimens (material obtained at three operations and at autopsy). The appearance was highly variable: 1) diffuse and sparse proliferations of astrocytes with hyperchromatic pleomorphic nuclei; 2) spindle and pleomorphic sarcomatous cells with abundant collagen; 3) myxomatous areas with giant cells containing bizarre nuclei; 4) transitional areas with sarcomatous appearance; 5) vascular endothelial proliferations; and 6) areas of necrosis and hemorrhage (Figure 4). In phosphotungstic acid-hematoxylin and glial fibril acid protein (GFAP) (Figure 5) preparations, anaplastic cells were noted. The final diagnosis was glioblastoma with sarcomatous components.
Discussion Brain tumors rarely metastasize to extracranial organs. Some authors attribute the rarity of distant metastases
Surg Neurol 1985;24:641-5
643
Figure 3. Computed tomogram on November I0 and December 9 showed the large mass with contrast medium enchancement indicating recurrence of the tumor.
to the absence of intracranial lymphatic channels [1,3], while others believe that postoperative life span is too short to develop enough metastatic masses in other organs [8,11]. It is also believed that surgical procedure plays a major role in entrance of tumor cells into the extracranial blood vessels and lymphatics. The case reported here is very rare because the remote metastases occurred within only 2 months of the first operation. T h e r e are two possibilities: 1) introduction into the venous system by either direct invasion or during surgery; or 2) implantation of tumor cells upon the dura mater and scalp during an operative procedure with subsequent hematogenous or lymphatic spread. In our case, the finding of massive extracranial infiltration of the soft tissue adjacent to the operative site may indicate that tumoral dissemination occurred in the operative wound and led to invasion of the soft tissue, lymphatics, and extracranial vessels. Extracranial metastases o f glioblastoma with sarco-
644
Surg Neurol 1985;24:641-5
Yokoyama et al
Figure 4. Histological examination of brain tumor revealed anaplastic cells and spindle cells. Transitional area from vascular components to sarcomatous ones was also observed (H & E stain, x 37).
matous component, gliosarcoma by some authors [4,5,10], have now been d o c u m e n t e d in 11 cases in the world literatures (Table 1). Smith et al [9] described a series o f seven cases of gliosarcoma that had metastasized to extracranial organs. These seven cases constitute about one-third of the cases of metastasizing glioma at the A r m e d Forces Institute o f Pathology. Morantz et al [7] reported that the ability of the gliosarcoma to metastasize is much greater than that of other malignant tumors. Age distribution in the reported cases ranged from 6.5 to 64 years, with an average of 43.2 years. Eight
Figure 5. Neoplastic cells with GFAPpositive cytoplasm were noted (GFAP stain, × 150).
cases were men and three were women. In all cases the location o f the primary tumor was supratentorial--temporal in eight cases, frontal in two cases, and occipital in the present case. Metastases occurred in the lung (seven cases--64%), liver (six cases--54%), lymph nodes (two c a s e s - - 1 8 % ) , and in the adrenal gland and vertebral body (one case each). Survival time o f gliosarcoma from the time of the first operation ranged from 4 months to 15 months. This is an average of 8.4 months which is shorter than that of other gliomas with distant metastases.
Gliosarcoma with Extracranial Metastasis
Surg Neurol 1985;24:641-5
645
Table 1. Previously Recorded Cases of Glioblastoma with Sarcomatous Component with Extracranial Metastases Length of Patient's
Case
Author(s)
1 2 3 4 5 6 7 8 9 10 11
Ehrenreich et al 2 Feigin et al ~ Garret c' Smith et al v Smith et al v Smith et al 9 Smith et al ') Smith et al 9 Smith et al v Smith et ar ) Present case
(1958) (1958) (1958) (1969) (1969) (1969) (1969) (1969) (1969) (1969) (1983)
Location of
Postoperative
Postoperative
Age & Sex
Primary
Radiation
Survival
44 M 61/2 M 55 F 49 M 44 M 58 M 63 M 64 M 61/2 M 63 F 22 F
R--temporal L--temporal R--temporal R--frontal R--temporal L--temporal R--temporal R--temporal L--temporal L--frontal L--occipital
Yes Yes Yes Yes Yes Yes
Yes
8 15 9 8 6 6 8 10 7 11 4
mos mos mos mos mos mos mos mos mos mos mos
Sites of Metastases Lung Lung Cervical Lymph N o d e Liver Liver Liver Lung, Liver & Adrenal Gland Lung & Liver Lung Vertebral Body & Lung Cervical Lymph Node, Lung & Liver
Abbreviations: mos = months; M ~ male, F = female.
Morantz et al [7] reported that the prognosis ofgliosarcoma, in general, is no worse than that of the anaplastic astrocytoma, but they had no cases with distant metastases. We believe that the prognosis is worse than that of other gliomas with extracranial metastases. Although the sarcomatous c o m p o n e n t might play some role in prognosis, the reason is still unknown.
4. Feigin I, Gross SW. Sarcoma arising in glioblastoma of the brain. A m J Pathol 1955;31:633-53.
References
8. Perry RE. Extracranial metastasis in a case of intracranial ependymoma. Arch Pathol 1957;64:337-41.
1. Dubois-Ferriere H. Drainage paths of intracranial fluids (lymph and CSF) and rarity of extracranial metastases of cerebral tumors. Annls Anat Path 1940;16:1081-114.
5. Feigin I, Allen LB, Lipkin L, Gross SW. T h e endothelial hyperplasia of the cerebral blood vessels with brain tumors and its sarcomatous transformation. Cancer 1958;11:264-7. 6. Garret R. Glioblastoma and fibrosarcoma of the brain with extracranial metastases. Cancer 1958; 11:888-894. 7. Morantz RA, Feigin I, R a n s o h o f f J . Gliosarcomas: A clinical and pathological survey of 24 cases. J N e u r o s u r g 1976;45:398-408.
9. Smith DR, H a r d m a n J N , Earle KM. Contiguous glioblastoma multiform and fibrisarcoma with extracranial metastases. Cancer 1969;24:270-7.
2. E h r e n r e i c h T H , DevlinJF. A complex ofglioblastoma and spindle cell sarcoma with pulmonary metastases. Arch Pathol 1958;66:536-49.
10. Stroebe H. U e b e r Entstehung und Bau der Gehirngliome. Beitr Path Anat 1895;18:405-86.
3. Elvidge A, Penfield W, C o n e W. T h e gliomas of the central nervous system. A study of two h u n d r e d and ten verified cases. Res Pubis Ass N e r v Ment Dis 1937;16:107-81.
11. Winkelman N W Jr, Cassel C, Schlesinger B~ lntracranial tumors with extra-cranial metastases. J N e u r o p a t h Exp Neurol 1952;11:149-66.