- Email: [email protected]
A diagnostic pitfall: Angiosarcoma of the brain mimicking cavernous angioma
688 Matsuno et al.
A diagnostic pitfall: Angiosarcoma of the brain mimicking cavernous angioma Akira Matsuno1 MD PHD, Tadashi Nagashima1 MD PHD, Yasuo Tajima2 MD PHD, Isamu Sugano2 MD PHD 1 Department of Neurosurgery, 2 Department of Pathology, Teikyo University Ichihara Hospital, Ichihara City, Chiba, Japan
Summary Primary or secondary angiosarcoma in the central nervous system is rarely reported. We present a rare case of cerebral angiosarcoma, which comprised both poorly-differentiated solid areas and well-differentiated areas that led to the misdiagnosis of cavernous angioma. A 79-year old woman presented with an intracerebral hematoma in the left frontal lobe that was misdiagnosed as a hemorrhage from a cavernous angioma at initial operation. At a second surgery, the lesion was diagnosed as angiosarcoma involving the cerebellum, heart, femur, sacro-iliac bones and other locations. An autopsy suggested that the angiosarcoma of the heart was the primary lesion, which was occult at the time of the initial operation. Angiosarcoma may have areas with different degrees of differentiation and when a cavernous angioma is suspected histopathologically, the specimen should also be carefully explored for poorly-differentiated areas and the diagnosis of primary or secondary angiosarcoma considered. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(6), 688–691 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.08.028
Keywords: angiosarcoma, cavernous angioma, brain, heart Received 16 July 2004 Accepted 10 August 2004
Correspondence to: Akira Matsuno MD PhD, Department of Neurosurgery, Teikyo University Ichihara Hospital, 3426-3 Anegasaki, Ichihara City, Chiba 299-0111, Japan. Tel.: +81 436 62 1211, Fax: +81 436 62 1357; E-mail: [email protected]
INTRODUCTION Primary or secondary angiosarcoma of the central nervous system (CNS) is rarely reported 1–22. We describe a rare case of a cerebral angiosarcoma, which comprised both poorly-differentiated solid areas and well-differentiated areas that led to the misdiagnosis of cavernous angioma. This case report and literature review focuses on primary and secondary CNS angiosarcoma and the difficulty of differentiating angiosarcoma from cavernous angioma in the brain.
CASE REPORT A 79-year old woman was hospitalized in 1999 with the sudden onset of a right hemiparesis, motor aphasia, and Gerstmann’s syndrome. MRI of the brain demonstrated a heterogeneous mixed signal intensity lesion on T1-weighted images with a small region of central enhancement in the left fronto-parietal region (Fig. 1a, b). The lesion showed heterogeneous mixed signal intensity on T2-weighted images (Fig. 1c). The left carotid angiogram was normal. Electrocardiography showed left ventricular hypertrophy with no other abnormalities. Although the radiological findings were not typical of a cavernous angioma, we considered the most likely diagnosis to be a hemorrhage from a cavernous angioma. At operation, the hematoma, along with numerous abnormal vessels, was totally removed. The pathological diagnosis was cavernous angioma, composed of numerous vascular channels containing only a few clusters of cells with mild atypia (Fig. 2a, b). The postoperative MRI revealed only a small residual hematoma. However, her motor aphasia deteriorated, and she complained of constipation due to an ileus. MRI demonstrated a recurrent left fronto-parietal heterogeneously enhancing mixed signal intensity
Fig. 1 Preoperative MRI at diagnosis demonstrated a heterogeneous mixed signal intensity lesion on T1-weighted images with a small central enhancement (7 mm) in the left fronto-parietal region (a, b). The lesion showed a heterogeneous mixed signal intensity on T2-weighted images (c). MRI prior to the second operation showed a recurrence of the lesion with a similar appearance (5 · 4.5 · 4 cm) (d, e). A second lesion with a diameter of 1 cm and the same signal characteristics was noted in the left cerebellar hemisphere (f).
Journal of Clinical Neuroscience (2005) 12(6)
ª 2005 Elsevier Ltd. All rights reserved.
Angiosarcoma mimicking cavernous angioma 689
Fig. 2 Histopathological examination of the first surgical specimen suggested a diagnosis of cavernous angioma, composed of numerous vascular channels containing only a few clusters of cells with mild atypia (Hematoxylin and eosin; Original magnification: a x80, b x400). Histopathological examination of the specimen from the second operation confirmed the diagnosis of angiosarcoma, highly cellular with cellular atypia, mitotic figures and irregular immature vascular channels (Hematoxylin and eosin staining; Original magnification: c x400). Histopathological examination of the surgical specimens from the fractured right femur also demonstrated angiosarcoma, with electron microscopic examination showing a Weibel-Palade body (Original magnification: d x400, e: bar = 500 nm).
lesion on T1-weighted images, with heterogeneous mixed signal intensity on T2-weighted images and perifocal high signal intensity (5 · 4.5 · 4 cm) (Fig. 1d, e). A second lesion, 1 cm in diameter, with similar signal characteristics was noted in the left cerebellar hemisphere (Fig. 1f). At a second operation, the abnormal vessels and surrounding large hematoma at the same site as the first surgery were totally removed. The pathological diagnosis on the surgical specimen from the second operation was angiosarcoma, which was highly cellular with cellular atypia (Fig. 2c). On immunohistochemistry, the tumor was positive for vimentin, Factor VIII, Ulex europaeus 1 lectin (UEA-1), and the MIB-1 staining index was high at 44.3%. The patient’s ileus persisted and she suffered a pathological fracture
of the right femur, treated with intramedullary nail fixation. The pathological diagnosis of the femoral lesion was also angiosarcoma, and electron microscope examination showed a WeibelPalade body (Fig. 2d, e). Thoraco-abdominal CT scan revealed a 4 cm diameter mass lesion in the left atrium (Fig. 3a), multiple nodular lesions in the lungs, multiple bone involvement (ribs and sacro-iliac joint) and suspected lesions in both adrenal glands. The patient died of acute heart failure due to an angiosarcoma in the left atrium one month after the second operation. The autopsy, performed 8 hours postmortem, confirmed (1) Primary angiosarcoma of the left atrium, 6.0 · 3.5 · 2.8 cm with central necrosis and hyalinization (Fig. 3b). (2) Metastatic angiosarcoma of the left cerebrum, diameter 3.5 cm, and of the left cerebellum, diameter 2.8 cm. (3) Angiosarcoma of the left upper lobe of the lung, diameter 1 cm and of the adrenal glands with multiple bone metastases and pathologic fractures of the right femur and humerus.
DISCUSSION
Fig. 3 Thoracic computed tomography scan revealed a 4 cm diameter mass lesion in the left atrium (a). Histopathological examination of the autopsied specimens of the heart confirmed the diagnosis of angiosarcoma (b) (Hematoxylin and eosin staining; Original magnification: x80).
ª 2005 Elsevier Ltd. All rights reserved.
Angiosarcoma is a highly malignant neoplasm derived from vascular endothelial cells and is one of the most rare of human tumors. Primary angiosarcoma of the CNS is extremely rare with only 17 reported cases 2,3,5,7,10,12,13,15,22 (Table 1). Metastatic angiosarcoma of the brain is also rare with only 14 cases previously reported 1,4,6,8,9,11,14–21 (Table 2). The most common primary site for a metastatic intracranial angiosarcoma is the heart. Angiosarcomas originating in the heart are more common than those of CNS origin, and most frequently occur in the right atrium (71.4%). Metastatic lesions often manifest prior to the diagnosis of cardiac angiosarcoma (66–89%).11,19 Metastases occur to the lung (51.2%), liver (37.2%), CNS (30.2%) and bone (20.9%). In the case presented, it is likely that the lesion in the brain was a metastatic angiosarcoma from a cardiac primary rather than a Journal of Clinical Neuroscience (2005) 12(6)
690 Matsuno et al.
Table 1 Seventeen reported cases of angiosarcoma originating in the central nervous system Authors
Age (years) 22
17 15 65 32 2 weeks–75 24 26 neonate 39 41
Ziegler (1975) Mena and Garcia (1978)12 Charman et al (1988)3 Cookston et al (1991)5 Mena et al (1991)13 Paulus et al (1991)15 Kirk et al (1992)10 Fuse et al (1995)7 Antoniadis et al (1996)2
Sex
Location in the brain
M F M F M5, F3 M M
Right parietal Left frontal Right parieto-occipital Right occipital 6 cerebral, 1 meninges, 1 unknown Temporal Frontal Right temporal Right temporal Left parietal
M F
Table 2 Fourteen reported cases of metastatic angiosarcoma in the brain Authors Angrish et al (1979)1 Wasmer et al.(1981)20 Seto et al (1988)18 Potter et al (1989)16 Grollier et al (1990)8 Vaquero et al (1990)19 Kiratsu et al (1991)11 Crespo et al (1993)6 Rettmar et al (1993)17 Watanabe et al (1993)21 Chaudhuri et al (1994)4 Hwang et al (1996)9 Nitta et al (1998)15
Age/Sex (years) 38/M 61/M 17/M 27/F 70/F 30/M 31/F 31/M 32/M 44/M 30/M 31/F 17/F 50/M
Location in brain
Primary site
Other metastases
Right temporal Brain Pineal region Multiple Brain Right frontal Left temporal, thalamus, vermis Multiple Multiple Bilateral temporo-parietal Multiple Left fronto-parietal Left fronto-temporo-parietal Not described
Heart Penis Liver Heart Heart (right atrium) Heart Femur Heart Heart Heart Heart Placenta Heart (pericardium) Heart
None Liver, cheek, lung Lung Lung, liver, bone, thyroid, pancreas, peritoneum Liver Lung, bone None Lung, bone, liver None Lung Lung None Multiple Lung
primary angiosarcoma of the brain. CNS angiosarcoma is unlikely to metastasize to the heart. Malignant transformation of a benign cavernous angioma is extremely unusual. The autopsy report suggested that the angiosarcoma of the left atrium was the primary lesion. This conclusion is supported by several findings including the multiple metastases to the brain diagnosed before the second surgery and the presence of multiple systemic metastases. Angiosarcoma arising in the heart is more common than CNS angiosarcoma. Mena et al analyzed the microscopic findings of eight cases of CNS angiosarcoma and reported that all tumors demonstrated a well-differentiated pattern and four also had poorly-differentiated solid areas.13 The brain lesion at the first operation comprised both well- and poorly-differentiated solid areas. The well-differentiated areas were misdiagnosed as a cavernous angioma. Residual poorly-differentiated solid tumor areas after the first operation enlarged rapidly in the left fronto-parietal lobe, and further metastases developed in the cerebellum, femur, sacro-iliac bones and other locations. The primary heart lesion was occult apart from high voltages in ECG suggesting ventricular hypertrophy. Regions of variable differentiation within angiosarcoma may lead to a misdiagnosis of cavernous angioma. When a cavernous angioma is suspected histopathologically, a thorough exploration should be made for poorly-differentiated regions. Rapid growth or recurrence of a cavernous angioma should raise suspicion for a primary or metastatic angiosarcoma.
REFERENCES 1. Angrish K, Manchanda SC, Shankar SK, Chopra P. Primary angiosarcoma of the heart. Jpn Heart J 1979; 20: 375–380. 2. Antoniadis C, Selviaridis P, Zaramboukas T, Fountzilas G. Primary angiosarcoma of the brain: case report. Neurosurgery 1996; 38: 583–585.
Journal of Clinical Neuroscience (2005) 12(6)
3. Charman HP, Lowenstein DH, Cho KG, DeArmond SJ, Wilson CB. Primary cerebral angiosarcoma. Case report. J Neurosurg 1988; 68: 806–810. 4. Chaudhuri KR, Jager R, Bridger J, Fusi L, Thomas DG, Frackowiak RS. Hemiplegia in pregnancy due to metastatic cerebral angiosarcoma. Eur Neurol 1994; 34: 295–297. 5. Cookston M, Cotter GW, Schlitt M, Bastian FO. Primary angiosarcoma of the brain. South Med J 1991; 84: 517–520. 6. Crespo MG, Pulpon LA, Pradas G, et al. Heart transplantation for cardiac angiosarcoma: should its indication be questioned? J Heart Lung Transplant 1993; 12: 527–530. 7. Fuse T, Takagi T, Hirose M. Primary intracranial epithelioid angiosarcoma case report. Neurol Med Chir (Tokyo) 1995; 35: 364–368. 8. Grollier G, Montecot L, Galateau F, et al. Right-left shunt caused by sarcoma of the right atrium. (French) Arch Mal Coeur Vaiss 1990; 83: 1007–1010. 9. Hwang SL, Howng SL, Sun ZM, Kwan AL. Brain metastasis from pericardial angiosarcoma. J Formos Med Assoc 1996; 95: 484–486. 10. Kirk IR, Dominguez R, Castillo M. Congenital primary cerebral angiosarcoma: CT, US, and MR findings. Pediatr Radiol 1992; 22: 134–135. 11. Kuratsu J, Seto H, Kochi M, Itoyama Y, Uemura S, Ushio Y. Metastatic angiosarcoma of the brain. Surg Neurol 1991; 35: 305–309. 12. Mena H, Garcia JH. Primary brain sarcomas: Light and electron microscopic features. Cancer 1978; 42: 1298–1307. 13. Mena H, Ribas JL, Enzinger FM, Parisi JE. Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature. J Neurosurg 1991; 75: 73–76. 14. Nitta R, Sakomura Y, Tanimoto K, et al. Primary cardiac angiosarcoma of the right atrium undiagnosed by transvenous endocardial tumor biopsy. Intern Med 1998; 37: 1023–1026. 15. Paulus W, Slowik F, Jellinger K. Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 1991; 18: 395–402. 16. Potter R, Baumgart P, Greve H, Schnepper E. Primary angiosarcoma of the heart. Thorac Cardiovasc Surg 1989; 37: 374–378. 17. Rettmar K, Stierle U, Sheikhzadeh A, Diederich KW. Primary angiosarcoma of the heart. Report of a case and review of the literature. Jpn Heart J 1993; 34: 667–683. 18. Seto H, Matsukado Y, Kuratsu J, Takaki S, Tomoda K. Angiosarcoma of the liver and pineal region. No Shinkei Geka 1988; 16: 409–413 (Japanese). 19. Vaquero J, Martinez R, Coca S, Oya S, Burgos R. Cerebral metastasis from angiosarcoma of the heart. Case report. J Neurosurg 1990; 73: 633–635. 20. Wasmer JM, Block NL, Politano VA, Tejada F. Penile angiosarcoma presenting in bladder. Urology 1981; 18: 179–180.
ª 2005 Elsevier Ltd. All rights reserved.
Pineal region metastasis of small cell lung cancer 691
21. Watanabe Y, Fukuda T, Araki M, Hiraki S. A case of angiosarcoma of the heart with cardiac tamponade and repeated hemoptysis. Nihon Kyobu Shikkan Gakkai Zasshi 1993; 31: 1485–1488 (Japanese). 22. Ziegler JW. Primary angiosarcoma of the brain: report of a case. J Am Osteopath Assoc 1975; 74: 957–960.
Isolated pineal region metastasis of small cell lung cancer Jung Yong Ahn MD, Young Sun Chung Seong Oh Kwon MD, Ryoong Huh MD, Sang Sup Chung MD
MD,
Department of Neurosurgery, Pundang CHA Hospital, Pochon CHA University, Sungnam, South Korea
Summary The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. Rarely the symptoms of metastatic involvement of the pineal gland precede those of the primary tumor or other metastatic sites. An 83 year-old man presented with gait disturbance and limitation of upward gaze. Brain MRI showed homogeneous enhancement of a solitary mass in the pineal region with obstructive hydrocephalus. A stereotactic biopsy was performed, and small cell carcinoma was diagnosed. A systemic investigation for the primary lesion subsequently revealed small cell carcinoma of the lung. The patient was referred for radiotherapy and chemotherapy. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors, particularly in elderly patients. ª 2005 Elsevier Ltd. All rights reserved.
INTRODUCTION The pineal region is an unusual site for brain metastasis of systemic malignant tumors. Metastasis to the pineal region accounted for only 0.3% of all brain metastasis in a series from Japan1 but ranges from 1.8% to 4% in other series.2–4 A recent literature review identified a total of 87 reported cases. In approximately half of these cases, the pineal gland was the only site of intracranial metastasis.5 Metastases varied in size. The most common site of primary origin is lung carcinoma, followed by breast carcinoma and other malignancies including melanoma, gastric carcinoma, myeloma and tumours of the kidney and colon.6 The mechanism of pineal metastasis is not established, but metastasis via the bloodstream seems most probable. Recently, we treated a patient with a pineal region metastasis, proven to be from a small cell carcinoma of the lung, which was radiosensitive and chemosensitive. Here, we report this patient with an isolated pineal region mass with radiological features mimicking a primary pineal region tumor.
CASE REPORT This 82 year-old man suffered sudden onset severe headache. He had developed progressively worsening gait and visual disturbance 2 months prior to admission. He had a history of hypertension and chronic obstructive pulmonary disease. On examination, he was noted to be hypertensive and confused but obeying commands. Neurological examination revealed cerebellar dysfunction on the left and limitation of upward gaze. A CT scan showed
Journal of Clinical Neuroscience (2005) 12(6), 691–693 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.010
Keywords: pineal region, metastasis, lung carcinoma, small cell carcinoma Received 16 July 2004 Accepted 1 September 2004 Correspondence to: Jung Yong Ahn, Department of Neurosurgery, Pundang CHA Hospital 351, Yatap-dong, Pundang-gu, Sungnam, 463-712, South Korea. Tel.: 82 31 780 5263; Fax: +82 31 780 5269; E-mail: [email protected]
Fig. 1 Axial CT scan showing a 2 · 2 cm hyperdense space-occupying lesion in the pineal region and associated ventricular enlargement.
Fig. 2 MRI scans showing the tumor, which is isointense on the T1-weighted sagittal image (a), uniformly hypointense on the T2-weighted axial image (b), and shows homogeneous enhancement after administration of gadolinium (c).
ª 2005 Elsevier Ltd. All rights reserved.
Journal of Clinical Neuroscience (2005) 12(6)
A diagnostic pitfall: Angiosarcoma of the brain mimicking cavernous angioma Akira Matsuno1 MD PHD, Tadashi Nagashima1 MD PHD, Yasuo Tajima2 MD PHD, Isamu Sugano2 MD PHD 1 Department of Neurosurgery, 2 Department of Pathology, Teikyo University Ichihara Hospital, Ichihara City, Chiba, Japan
Summary Primary or secondary angiosarcoma in the central nervous system is rarely reported. We present a rare case of cerebral angiosarcoma, which comprised both poorly-differentiated solid areas and well-differentiated areas that led to the misdiagnosis of cavernous angioma. A 79-year old woman presented with an intracerebral hematoma in the left frontal lobe that was misdiagnosed as a hemorrhage from a cavernous angioma at initial operation. At a second surgery, the lesion was diagnosed as angiosarcoma involving the cerebellum, heart, femur, sacro-iliac bones and other locations. An autopsy suggested that the angiosarcoma of the heart was the primary lesion, which was occult at the time of the initial operation. Angiosarcoma may have areas with different degrees of differentiation and when a cavernous angioma is suspected histopathologically, the specimen should also be carefully explored for poorly-differentiated areas and the diagnosis of primary or secondary angiosarcoma considered. ª 2005 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2005) 12(6), 688–691 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.08.028
Keywords: angiosarcoma, cavernous angioma, brain, heart Received 16 July 2004 Accepted 10 August 2004
Correspondence to: Akira Matsuno MD PhD, Department of Neurosurgery, Teikyo University Ichihara Hospital, 3426-3 Anegasaki, Ichihara City, Chiba 299-0111, Japan. Tel.: +81 436 62 1211, Fax: +81 436 62 1357; E-mail: [email protected]
INTRODUCTION Primary or secondary angiosarcoma of the central nervous system (CNS) is rarely reported 1–22. We describe a rare case of a cerebral angiosarcoma, which comprised both poorly-differentiated solid areas and well-differentiated areas that led to the misdiagnosis of cavernous angioma. This case report and literature review focuses on primary and secondary CNS angiosarcoma and the difficulty of differentiating angiosarcoma from cavernous angioma in the brain.
CASE REPORT A 79-year old woman was hospitalized in 1999 with the sudden onset of a right hemiparesis, motor aphasia, and Gerstmann’s syndrome. MRI of the brain demonstrated a heterogeneous mixed signal intensity lesion on T1-weighted images with a small region of central enhancement in the left fronto-parietal region (Fig. 1a, b). The lesion showed heterogeneous mixed signal intensity on T2-weighted images (Fig. 1c). The left carotid angiogram was normal. Electrocardiography showed left ventricular hypertrophy with no other abnormalities. Although the radiological findings were not typical of a cavernous angioma, we considered the most likely diagnosis to be a hemorrhage from a cavernous angioma. At operation, the hematoma, along with numerous abnormal vessels, was totally removed. The pathological diagnosis was cavernous angioma, composed of numerous vascular channels containing only a few clusters of cells with mild atypia (Fig. 2a, b). The postoperative MRI revealed only a small residual hematoma. However, her motor aphasia deteriorated, and she complained of constipation due to an ileus. MRI demonstrated a recurrent left fronto-parietal heterogeneously enhancing mixed signal intensity
Fig. 1 Preoperative MRI at diagnosis demonstrated a heterogeneous mixed signal intensity lesion on T1-weighted images with a small central enhancement (7 mm) in the left fronto-parietal region (a, b). The lesion showed a heterogeneous mixed signal intensity on T2-weighted images (c). MRI prior to the second operation showed a recurrence of the lesion with a similar appearance (5 · 4.5 · 4 cm) (d, e). A second lesion with a diameter of 1 cm and the same signal characteristics was noted in the left cerebellar hemisphere (f).
Journal of Clinical Neuroscience (2005) 12(6)
ª 2005 Elsevier Ltd. All rights reserved.
Angiosarcoma mimicking cavernous angioma 689
Fig. 2 Histopathological examination of the first surgical specimen suggested a diagnosis of cavernous angioma, composed of numerous vascular channels containing only a few clusters of cells with mild atypia (Hematoxylin and eosin; Original magnification: a x80, b x400). Histopathological examination of the specimen from the second operation confirmed the diagnosis of angiosarcoma, highly cellular with cellular atypia, mitotic figures and irregular immature vascular channels (Hematoxylin and eosin staining; Original magnification: c x400). Histopathological examination of the surgical specimens from the fractured right femur also demonstrated angiosarcoma, with electron microscopic examination showing a Weibel-Palade body (Original magnification: d x400, e: bar = 500 nm).
lesion on T1-weighted images, with heterogeneous mixed signal intensity on T2-weighted images and perifocal high signal intensity (5 · 4.5 · 4 cm) (Fig. 1d, e). A second lesion, 1 cm in diameter, with similar signal characteristics was noted in the left cerebellar hemisphere (Fig. 1f). At a second operation, the abnormal vessels and surrounding large hematoma at the same site as the first surgery were totally removed. The pathological diagnosis on the surgical specimen from the second operation was angiosarcoma, which was highly cellular with cellular atypia (Fig. 2c). On immunohistochemistry, the tumor was positive for vimentin, Factor VIII, Ulex europaeus 1 lectin (UEA-1), and the MIB-1 staining index was high at 44.3%. The patient’s ileus persisted and she suffered a pathological fracture
of the right femur, treated with intramedullary nail fixation. The pathological diagnosis of the femoral lesion was also angiosarcoma, and electron microscope examination showed a WeibelPalade body (Fig. 2d, e). Thoraco-abdominal CT scan revealed a 4 cm diameter mass lesion in the left atrium (Fig. 3a), multiple nodular lesions in the lungs, multiple bone involvement (ribs and sacro-iliac joint) and suspected lesions in both adrenal glands. The patient died of acute heart failure due to an angiosarcoma in the left atrium one month after the second operation. The autopsy, performed 8 hours postmortem, confirmed (1) Primary angiosarcoma of the left atrium, 6.0 · 3.5 · 2.8 cm with central necrosis and hyalinization (Fig. 3b). (2) Metastatic angiosarcoma of the left cerebrum, diameter 3.5 cm, and of the left cerebellum, diameter 2.8 cm. (3) Angiosarcoma of the left upper lobe of the lung, diameter 1 cm and of the adrenal glands with multiple bone metastases and pathologic fractures of the right femur and humerus.
DISCUSSION
Fig. 3 Thoracic computed tomography scan revealed a 4 cm diameter mass lesion in the left atrium (a). Histopathological examination of the autopsied specimens of the heart confirmed the diagnosis of angiosarcoma (b) (Hematoxylin and eosin staining; Original magnification: x80).
ª 2005 Elsevier Ltd. All rights reserved.
Angiosarcoma is a highly malignant neoplasm derived from vascular endothelial cells and is one of the most rare of human tumors. Primary angiosarcoma of the CNS is extremely rare with only 17 reported cases 2,3,5,7,10,12,13,15,22 (Table 1). Metastatic angiosarcoma of the brain is also rare with only 14 cases previously reported 1,4,6,8,9,11,14–21 (Table 2). The most common primary site for a metastatic intracranial angiosarcoma is the heart. Angiosarcomas originating in the heart are more common than those of CNS origin, and most frequently occur in the right atrium (71.4%). Metastatic lesions often manifest prior to the diagnosis of cardiac angiosarcoma (66–89%).11,19 Metastases occur to the lung (51.2%), liver (37.2%), CNS (30.2%) and bone (20.9%). In the case presented, it is likely that the lesion in the brain was a metastatic angiosarcoma from a cardiac primary rather than a Journal of Clinical Neuroscience (2005) 12(6)
690 Matsuno et al.
Table 1 Seventeen reported cases of angiosarcoma originating in the central nervous system Authors
Age (years) 22
17 15 65 32 2 weeks–75 24 26 neonate 39 41
Ziegler (1975) Mena and Garcia (1978)12 Charman et al (1988)3 Cookston et al (1991)5 Mena et al (1991)13 Paulus et al (1991)15 Kirk et al (1992)10 Fuse et al (1995)7 Antoniadis et al (1996)2
Sex
Location in the brain
M F M F M5, F3 M M
Right parietal Left frontal Right parieto-occipital Right occipital 6 cerebral, 1 meninges, 1 unknown Temporal Frontal Right temporal Right temporal Left parietal
M F
Table 2 Fourteen reported cases of metastatic angiosarcoma in the brain Authors Angrish et al (1979)1 Wasmer et al.(1981)20 Seto et al (1988)18 Potter et al (1989)16 Grollier et al (1990)8 Vaquero et al (1990)19 Kiratsu et al (1991)11 Crespo et al (1993)6 Rettmar et al (1993)17 Watanabe et al (1993)21 Chaudhuri et al (1994)4 Hwang et al (1996)9 Nitta et al (1998)15
Age/Sex (years) 38/M 61/M 17/M 27/F 70/F 30/M 31/F 31/M 32/M 44/M 30/M 31/F 17/F 50/M
Location in brain
Primary site
Other metastases
Right temporal Brain Pineal region Multiple Brain Right frontal Left temporal, thalamus, vermis Multiple Multiple Bilateral temporo-parietal Multiple Left fronto-parietal Left fronto-temporo-parietal Not described
Heart Penis Liver Heart Heart (right atrium) Heart Femur Heart Heart Heart Heart Placenta Heart (pericardium) Heart
None Liver, cheek, lung Lung Lung, liver, bone, thyroid, pancreas, peritoneum Liver Lung, bone None Lung, bone, liver None Lung Lung None Multiple Lung
primary angiosarcoma of the brain. CNS angiosarcoma is unlikely to metastasize to the heart. Malignant transformation of a benign cavernous angioma is extremely unusual. The autopsy report suggested that the angiosarcoma of the left atrium was the primary lesion. This conclusion is supported by several findings including the multiple metastases to the brain diagnosed before the second surgery and the presence of multiple systemic metastases. Angiosarcoma arising in the heart is more common than CNS angiosarcoma. Mena et al analyzed the microscopic findings of eight cases of CNS angiosarcoma and reported that all tumors demonstrated a well-differentiated pattern and four also had poorly-differentiated solid areas.13 The brain lesion at the first operation comprised both well- and poorly-differentiated solid areas. The well-differentiated areas were misdiagnosed as a cavernous angioma. Residual poorly-differentiated solid tumor areas after the first operation enlarged rapidly in the left fronto-parietal lobe, and further metastases developed in the cerebellum, femur, sacro-iliac bones and other locations. The primary heart lesion was occult apart from high voltages in ECG suggesting ventricular hypertrophy. Regions of variable differentiation within angiosarcoma may lead to a misdiagnosis of cavernous angioma. When a cavernous angioma is suspected histopathologically, a thorough exploration should be made for poorly-differentiated regions. Rapid growth or recurrence of a cavernous angioma should raise suspicion for a primary or metastatic angiosarcoma.
REFERENCES 1. Angrish K, Manchanda SC, Shankar SK, Chopra P. Primary angiosarcoma of the heart. Jpn Heart J 1979; 20: 375–380. 2. Antoniadis C, Selviaridis P, Zaramboukas T, Fountzilas G. Primary angiosarcoma of the brain: case report. Neurosurgery 1996; 38: 583–585.
Journal of Clinical Neuroscience (2005) 12(6)
3. Charman HP, Lowenstein DH, Cho KG, DeArmond SJ, Wilson CB. Primary cerebral angiosarcoma. Case report. J Neurosurg 1988; 68: 806–810. 4. Chaudhuri KR, Jager R, Bridger J, Fusi L, Thomas DG, Frackowiak RS. Hemiplegia in pregnancy due to metastatic cerebral angiosarcoma. Eur Neurol 1994; 34: 295–297. 5. Cookston M, Cotter GW, Schlitt M, Bastian FO. Primary angiosarcoma of the brain. South Med J 1991; 84: 517–520. 6. Crespo MG, Pulpon LA, Pradas G, et al. Heart transplantation for cardiac angiosarcoma: should its indication be questioned? J Heart Lung Transplant 1993; 12: 527–530. 7. Fuse T, Takagi T, Hirose M. Primary intracranial epithelioid angiosarcoma case report. Neurol Med Chir (Tokyo) 1995; 35: 364–368. 8. Grollier G, Montecot L, Galateau F, et al. Right-left shunt caused by sarcoma of the right atrium. (French) Arch Mal Coeur Vaiss 1990; 83: 1007–1010. 9. Hwang SL, Howng SL, Sun ZM, Kwan AL. Brain metastasis from pericardial angiosarcoma. J Formos Med Assoc 1996; 95: 484–486. 10. Kirk IR, Dominguez R, Castillo M. Congenital primary cerebral angiosarcoma: CT, US, and MR findings. Pediatr Radiol 1992; 22: 134–135. 11. Kuratsu J, Seto H, Kochi M, Itoyama Y, Uemura S, Ushio Y. Metastatic angiosarcoma of the brain. Surg Neurol 1991; 35: 305–309. 12. Mena H, Garcia JH. Primary brain sarcomas: Light and electron microscopic features. Cancer 1978; 42: 1298–1307. 13. Mena H, Ribas JL, Enzinger FM, Parisi JE. Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature. J Neurosurg 1991; 75: 73–76. 14. Nitta R, Sakomura Y, Tanimoto K, et al. Primary cardiac angiosarcoma of the right atrium undiagnosed by transvenous endocardial tumor biopsy. Intern Med 1998; 37: 1023–1026. 15. Paulus W, Slowik F, Jellinger K. Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 1991; 18: 395–402. 16. Potter R, Baumgart P, Greve H, Schnepper E. Primary angiosarcoma of the heart. Thorac Cardiovasc Surg 1989; 37: 374–378. 17. Rettmar K, Stierle U, Sheikhzadeh A, Diederich KW. Primary angiosarcoma of the heart. Report of a case and review of the literature. Jpn Heart J 1993; 34: 667–683. 18. Seto H, Matsukado Y, Kuratsu J, Takaki S, Tomoda K. Angiosarcoma of the liver and pineal region. No Shinkei Geka 1988; 16: 409–413 (Japanese). 19. Vaquero J, Martinez R, Coca S, Oya S, Burgos R. Cerebral metastasis from angiosarcoma of the heart. Case report. J Neurosurg 1990; 73: 633–635. 20. Wasmer JM, Block NL, Politano VA, Tejada F. Penile angiosarcoma presenting in bladder. Urology 1981; 18: 179–180.
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Isolated pineal region metastasis of small cell lung cancer Jung Yong Ahn MD, Young Sun Chung Seong Oh Kwon MD, Ryoong Huh MD, Sang Sup Chung MD
MD,
Department of Neurosurgery, Pundang CHA Hospital, Pochon CHA University, Sungnam, South Korea
Summary The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. Rarely the symptoms of metastatic involvement of the pineal gland precede those of the primary tumor or other metastatic sites. An 83 year-old man presented with gait disturbance and limitation of upward gaze. Brain MRI showed homogeneous enhancement of a solitary mass in the pineal region with obstructive hydrocephalus. A stereotactic biopsy was performed, and small cell carcinoma was diagnosed. A systemic investigation for the primary lesion subsequently revealed small cell carcinoma of the lung. The patient was referred for radiotherapy and chemotherapy. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors, particularly in elderly patients. ª 2005 Elsevier Ltd. All rights reserved.
INTRODUCTION The pineal region is an unusual site for brain metastasis of systemic malignant tumors. Metastasis to the pineal region accounted for only 0.3% of all brain metastasis in a series from Japan1 but ranges from 1.8% to 4% in other series.2–4 A recent literature review identified a total of 87 reported cases. In approximately half of these cases, the pineal gland was the only site of intracranial metastasis.5 Metastases varied in size. The most common site of primary origin is lung carcinoma, followed by breast carcinoma and other malignancies including melanoma, gastric carcinoma, myeloma and tumours of the kidney and colon.6 The mechanism of pineal metastasis is not established, but metastasis via the bloodstream seems most probable. Recently, we treated a patient with a pineal region metastasis, proven to be from a small cell carcinoma of the lung, which was radiosensitive and chemosensitive. Here, we report this patient with an isolated pineal region mass with radiological features mimicking a primary pineal region tumor.
CASE REPORT This 82 year-old man suffered sudden onset severe headache. He had developed progressively worsening gait and visual disturbance 2 months prior to admission. He had a history of hypertension and chronic obstructive pulmonary disease. On examination, he was noted to be hypertensive and confused but obeying commands. Neurological examination revealed cerebellar dysfunction on the left and limitation of upward gaze. A CT scan showed
Journal of Clinical Neuroscience (2005) 12(6), 691–693 0967-5868/$ - see front matter ª 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2004.09.010
Keywords: pineal region, metastasis, lung carcinoma, small cell carcinoma Received 16 July 2004 Accepted 1 September 2004 Correspondence to: Jung Yong Ahn, Department of Neurosurgery, Pundang CHA Hospital 351, Yatap-dong, Pundang-gu, Sungnam, 463-712, South Korea. Tel.: 82 31 780 5263; Fax: +82 31 780 5269; E-mail: [email protected]
Fig. 1 Axial CT scan showing a 2 · 2 cm hyperdense space-occupying lesion in the pineal region and associated ventricular enlargement.
Fig. 2 MRI scans showing the tumor, which is isointense on the T1-weighted sagittal image (a), uniformly hypointense on the T2-weighted axial image (b), and shows homogeneous enhancement after administration of gadolinium (c).
ª 2005 Elsevier Ltd. All rights reserved.
Journal of Clinical Neuroscience (2005) 12(6)