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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 451-452

Brain metastasis from cardiac angiosarcoma


1 Resident of Neurosurgery at Hospital das Clínicas, Botucatu, Brazil
2 Resident of Pathology at Hospital das Clínicas, Botucatu, Brazil
3 Pathologist at Hospital das Clínicas, Botucatu, Brazil
4 Neurosurgeon at Hospital das Clínicas, Botucatu Medical School São Paulo State University, Botucatu, Brazil

Date of Web Publication19-Sep-2014

Correspondence Address:
Roberto Bezerra Vital
Resident of Neurosurgery at Hospital das Clínicas, Botucatu
Brazil
Roberto Bezerra Vital
Resident of Neurosurgery at Hospital das Clínicas, Botucatu
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.141261

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How to cite this article:
Vital RB, Hamamoto Filho PT, Lima Neto NM, Caldeira Xavier JC, Santos DC, Romero FR, Zanini MA, Vital RB, Hamamoto Filho PT, Lima Neto NM, Caldeira Xavier JC, Santos DC, Romero FR, Zanini MA. Brain metastasis from cardiac angiosarcoma. Neurol India 2014;62:451-2

How to cite this URL:
Vital RB, Hamamoto Filho PT, Lima Neto NM, Caldeira Xavier JC, Santos DC, Romero FR, Zanini MA, Vital RB, Hamamoto Filho PT, Lima Neto NM, Caldeira Xavier JC, Santos DC, Romero FR, Zanini MA. Brain metastasis from cardiac angiosarcoma. Neurol India [serial online] 2014 [cited 2020 Oct 24];62:451-2. Available from: https://www.neurologyindia.com/text.asp?2014/62/4/451/141261


Sir,

Angiosarcomas are rare malignant tumors, accounting for less than 1% of all sarcomas, [1] and are the second most common histological type of cardiac sarcoma. These tumors are more frequent in men and usually occur in the third to fifth decades of life. [2] Involvement of brain, either primary or metastatic, is extremely rare. [3]

A 28-year-old male presented with headache and vomiting of 15 days duration. Neurologic examination was essentially normal. Magnetic resonance imaging (MRI) revealed a left frontal mass with heterogeneous contrast enhancement and peri-lesional edema [Figure 1]. Thirteen months before this admission, he was evaluated for progressive chest pain and was found to have a right atrial mass. On that occasion, the cardiac mass could not be completely resected because of invasion of the vena cava. Histopathology revealed cardiac angiosarcoma with positive staining for CD31 and CD34. The Ki-67 labeling index was about 80% [Figure 2]. Patient received chemotherapy with adriamycin and ifosfamide (6 cycles), followed by a rescue regimen with paclitaxel for bone dissemination (ribs). Since the patient showed good clinical health, despite the uncontrolled disease, he was taken up for surgery and complete resection of brain mass was performed. On postoperative day-3, he was discharged without neurological deficits. He had an uneventful history until 1.5 month of brain surgery, when he was brought to the emergency room and died from cardiac arrest.
Figure 1: (a) T1-weighted axial plane gadolinium-enhanced MRI scan showing an expansive lesion in the left frontal lobe with heterogeneous paramagnetic enhancement and a large hypointense vasogenic edema.
(b) Sagittal FLAIR sequence showing the lesion at the left frontal pole and a large hyperintense area of edema


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Figure 2: (a) Moderately differentiated metastatic angiosarcoma expanding into the gray-white junction accompanied by marked peritumoral edema (H and E, x200) (b) Cardiac angiosarcoma in the right atrium (H and E, x100). The vascular channels are lined with variably pleomorphic, hyperchromatic endothelial cells which frequently show multilayering. Normal and abnormal mitoses are found. (c) Immunohistochemical staining for CD34, which is usually positive (x400). (d) Immunohistochemical staining for Ki-67, with a labeling index of about 80% (x400)

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Angiosarcomas of the heart are rare and dissemination to the brain is even rarer. The sites affected by metastasis in descending order of frequency are: Cervical lymph nodes, lung, liver, and spleen. [4] Till 2005, only 14 cases of cerebral metastases have been reported. [5] Radiologically, cerebral angiosarcomas have a frequent tendency of hemorrhage with marked peri-lesional edema on computed tomography (CT) and MRI. Contrast enhancement is partial. [6] Angiosarcomas are positive for CD31 and CD34. Factor VIII is usually positive, except in cases of poorly differentiated or undifferentiated growth. This may explain the prolongation of prothrombin time, partial thromboplastin time and factor VIII, increasing the tendency to bleed. [7] The prognosis of cardiac sarcomas is still poor, with a median survival of 10 to 24 months despite more aggressive treatment with radiotherapy and chemotherapy. Moreover, while the development of new treatment protocols has improved the survival of patients with cardiac sarcomas, the incidence of cerebral metastasis seems to increase, probably because the tumor has more time to spread. [8]

 
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1.Regel JP, Pospiech J, Baume B, van de Nes JA. Cerebral metastasis from an undifferentiated sarcoma of the left atrium. Acta Neurochir (Wien) 2006;148:595-6.  Back to cited text no. 1
    
2.Fernandes F, Soufen HN, Ianni BM, Arteaga E, Ramires FJ, Mady C. Primary neoplasms of the heart. Clinical and histological presentation of 50 cases. Arq Bras Cardiol 2001;76:231-7.  Back to cited text no. 2
    
3.Jung SH, Jung TY, Joo SP, Kim HS. Rapid clinical course of cerebral metastatic angiosarcoma from the heart. J Korean Neurosurg Soc 2012;51:47-50.  Back to cited text no. 3
    
4.Kuratsu J, Seto H, Kochi M, Itoyama Y, Uemura S, Ushio Y. Metastatic angiosarcoma of the brain. Surg Neurol 1991;35:305-9.  Back to cited text no. 4
    
5.Matsuno A, Nagashima T, Tajima Y, Sugano I. A diagnostic pitfall: Angiosarcoma of the brain mimicking cavernous angioma. J Clin Neurosci 2005;12:688-91.  Back to cited text no. 5
    
6.Vaquero J, Martinez R, Coca S, Oya S, Burgos R. Cerebral metastasis from angiosarcoma of the heart. Case report. J Neurosurg 1990;73:633-5.  Back to cited text no. 6
    
7.Donsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier JF, Loire R. Primary cardiac sarcomas: An immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology 1999;34:295-304.  Back to cited text no. 7
    
8.Hwang SL, Howng SL, Sun ZM, Kwan AL. Brain metastasis from pericardial angiosarcoma. J Formos Med Assoc 1996;95:484-6.  Back to cited text no. 8
    


    Figures

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