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spindle to epithelioid cells, varying amounts of fibrous stroma, calcification, osseous metaplasia, scattered psammoma body and foreign body reaction with giant cells.[6] These findings may not be all together. Mitosis and necrosis have not been reported. Although a wide variety of immunohistochemical stains have been used to characterize CPN, they show highly positive staining with EMA and vimentin and highly negative staining with GFAP and S‑100.[4] Most authors, on the basis of findings of granulomatous inflammation surrounding these, believe that these calcified lesions occur after a reactive process in neuraxis. [2,6,7] Rodriguez et al.[8] presented a CPN case coexisting with ependymomas. They reported that CPN might have developed due to reactive process around ependymomas. Salim et al. [9] reported a CPN in touch with an intra‑axial lipoma and reported that CPN might most likely occur as a result of reactive process. It has been suggested, but not proven, that CPN may develop as a healing response to an array of inciting factors, which can account for the variations in histopathologic features. The causal factors are not yet understood, but response to possible trauma, infection, or inflammation has been proposed. [10] It is impossible to give some diagnostic and treatment algorithms because of rarity of the condition. Imaging methods are insufficient for a definitive diagnosis in growing number of these reported lesions, and they should be considered in the differential diagnosis of calcified lesions. Moreover, these lesions may grow and have recurrence risk. Thus, surgical resection seems to be the rationalistic treatment for now. Surgical removal of lesions is important both in elimination of the patient’s symptoms and obtaining histopathological diagnosis, and besides prevention of aggressive adjuvant treatments. To have enough opinion for the course of the disease, we think that there is a need for a greater number of patients and longer follow‑up period.
Aydemir Fatih, Cekinmez Melih, Kardes Ozgur, Sarica B. Feyzi, Tufan Kadir, Kayaselcuk Fazilet1 Departments of Neurosurgery, and Pathology, Faculty of Medicine, Adana Practice and Research Center, Baskent University, Adana, Turkey E‑mail: [email protected] 1
References 1. Rhodes RH, Davis RL. An unusual fibro‑osseous componentn intracranial lesions. Hum Pathol 1978;9:309‑19. 2. Bertoni F, Unni KK, Dahlin DC, Beabout JW, Onofrio BM. Calcifying pseudoneoplasms of the neural axis. J Neurosurg 1990;72:42‑8. 3. Jun C, Burdick B. An unusual fibro‑osseous lesion of the brain. Case Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4
report. J Neurosurg 1984;60:1308‑11. 4. Kerr EE, Borys E, Bobinski M, Shahlaie K. Posterior fossa calcifyingpseudoneoplasm of the central nervous system. J Neurosurg 2013;118:896‑902. 5. Montibeller GR, Stan AC, Krauss JK, Nakamura M. Calcifying pseudoneoplasm of the inferior colliculus: An unusual location for a rare tumor: Case report. Neurosurgery 2009;65:E1005‑6. 6. Qian J, Rubio A, Powers JM, Rosenblum MK, Pilcher WH, Shrier DA, et al. Fibro‑osseous lesions of the central nervous system: Report of four cases and literature review. Am J Surg Pathol 1999;23:1270‑5. 7. Tatke M, Singh AK, Gupta V. Calcifying pseudoneoplasm of the CNS. Br J Neurosurg 2001;15:521‑3. 8. Rodriguez FJ, Scheithauer BW, Fourney DR, Robinson CA. Ependymoma and intraparenchymal calcifying pseudoneoplasm of the neural axis: İncidental collision or unique reactive phenomenon? Acta Neuropathol 2008;115:363‑6. 9. Salim AA, Wilson PJ, Cherukuri RK, McKenzie S, Buckland ME. An unusual association of calcifying pseudoneoplasm of the neuraxis with interhemispheric lipoma and agenesis of corpus callosum. Pathology 2012;44:657‑9. 10. Aiken AH, Akgun H, Tihan T, Barbaro N, Glastonbury C. Calcifying pseudoneoplasms of the neuraxis: CT, MR imaging, and histologic features. AJNR Am J Neuroradiol 2009;30:1256‑60. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.141255
Received: 11-06-2014 Review completed: 13-06-2014 Accepted: 10-08-2014
Brain metastasis from cardiac angiosarcoma 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 445
[Downloaded free from http://www.neurologyindia.com on Thursday, September 25, 2014, IP: 202.177.173.189] || Click here to download free Android application f this journal Letters to Editor
a
b
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
a
b
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]
Roberto Bezerra Vital, Pedro Tadao Hamamoto Filho, Newton Moreira Lima Neto, Jose Candido Caldeira Xavier Junior1, Daniela Cristina Santos2, Flavio Ramalho Romero3, Marco Antonio Zanini3 Resident of Neurosurgery at Hospital das Clínicas, 1Resident of Pathology at Hospital das Clínicas, 2Pathologist at Hospital das Clínicas, 3Neurosurgeon at Hospital das Clínicas, Botucatu Medical School ‑ São Paulo State University, Botucatu, Brazil E‑mail: [email protected]
References 1. c
d
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)
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. 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 446
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. 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. 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. 4. Kuratsu J, Seto H, Kochi M, Itoyama Y, Uemura S, Ushio Y. Metastatic angiosarcoma of the brain. Surg Neurol 1991;35:305‑9. 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. 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. 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. 8. Hwang SL, Howng SL, Sun ZM, Kwan AL. Brain metastasis from pericardial angiosarcoma. J Formos Med Assoc 1996;95:484‑6. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.141261
Received: 06‑05‑2014 Review completed: 07‑06‑2014 Accepted: 20‑06‑2014 Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
spindle to epithelioid cells, varying amounts of fibrous stroma, calcification, osseous metaplasia, scattered psammoma body and foreign body reaction with giant cells.[6] These findings may not be all together. Mitosis and necrosis have not been reported. Although a wide variety of immunohistochemical stains have been used to characterize CPN, they show highly positive staining with EMA and vimentin and highly negative staining with GFAP and S‑100.[4] Most authors, on the basis of findings of granulomatous inflammation surrounding these, believe that these calcified lesions occur after a reactive process in neuraxis. [2,6,7] Rodriguez et al.[8] presented a CPN case coexisting with ependymomas. They reported that CPN might have developed due to reactive process around ependymomas. Salim et al. [9] reported a CPN in touch with an intra‑axial lipoma and reported that CPN might most likely occur as a result of reactive process. It has been suggested, but not proven, that CPN may develop as a healing response to an array of inciting factors, which can account for the variations in histopathologic features. The causal factors are not yet understood, but response to possible trauma, infection, or inflammation has been proposed. [10] It is impossible to give some diagnostic and treatment algorithms because of rarity of the condition. Imaging methods are insufficient for a definitive diagnosis in growing number of these reported lesions, and they should be considered in the differential diagnosis of calcified lesions. Moreover, these lesions may grow and have recurrence risk. Thus, surgical resection seems to be the rationalistic treatment for now. Surgical removal of lesions is important both in elimination of the patient’s symptoms and obtaining histopathological diagnosis, and besides prevention of aggressive adjuvant treatments. To have enough opinion for the course of the disease, we think that there is a need for a greater number of patients and longer follow‑up period.
Aydemir Fatih, Cekinmez Melih, Kardes Ozgur, Sarica B. Feyzi, Tufan Kadir, Kayaselcuk Fazilet1 Departments of Neurosurgery, and Pathology, Faculty of Medicine, Adana Practice and Research Center, Baskent University, Adana, Turkey E‑mail: [email protected] 1
References 1. Rhodes RH, Davis RL. An unusual fibro‑osseous componentn intracranial lesions. Hum Pathol 1978;9:309‑19. 2. Bertoni F, Unni KK, Dahlin DC, Beabout JW, Onofrio BM. Calcifying pseudoneoplasms of the neural axis. J Neurosurg 1990;72:42‑8. 3. Jun C, Burdick B. An unusual fibro‑osseous lesion of the brain. Case Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4
report. J Neurosurg 1984;60:1308‑11. 4. Kerr EE, Borys E, Bobinski M, Shahlaie K. Posterior fossa calcifyingpseudoneoplasm of the central nervous system. J Neurosurg 2013;118:896‑902. 5. Montibeller GR, Stan AC, Krauss JK, Nakamura M. Calcifying pseudoneoplasm of the inferior colliculus: An unusual location for a rare tumor: Case report. Neurosurgery 2009;65:E1005‑6. 6. Qian J, Rubio A, Powers JM, Rosenblum MK, Pilcher WH, Shrier DA, et al. Fibro‑osseous lesions of the central nervous system: Report of four cases and literature review. Am J Surg Pathol 1999;23:1270‑5. 7. Tatke M, Singh AK, Gupta V. Calcifying pseudoneoplasm of the CNS. Br J Neurosurg 2001;15:521‑3. 8. Rodriguez FJ, Scheithauer BW, Fourney DR, Robinson CA. Ependymoma and intraparenchymal calcifying pseudoneoplasm of the neural axis: İncidental collision or unique reactive phenomenon? Acta Neuropathol 2008;115:363‑6. 9. Salim AA, Wilson PJ, Cherukuri RK, McKenzie S, Buckland ME. An unusual association of calcifying pseudoneoplasm of the neuraxis with interhemispheric lipoma and agenesis of corpus callosum. Pathology 2012;44:657‑9. 10. Aiken AH, Akgun H, Tihan T, Barbaro N, Glastonbury C. Calcifying pseudoneoplasms of the neuraxis: CT, MR imaging, and histologic features. AJNR Am J Neuroradiol 2009;30:1256‑60. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.141255
Received: 11-06-2014 Review completed: 13-06-2014 Accepted: 10-08-2014
Brain metastasis from cardiac angiosarcoma 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 445
[Downloaded free from http://www.neurologyindia.com on Thursday, September 25, 2014, IP: 202.177.173.189] || Click here to download free Android application f this journal Letters to Editor
a
b
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
a
b
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]
Roberto Bezerra Vital, Pedro Tadao Hamamoto Filho, Newton Moreira Lima Neto, Jose Candido Caldeira Xavier Junior1, Daniela Cristina Santos2, Flavio Ramalho Romero3, Marco Antonio Zanini3 Resident of Neurosurgery at Hospital das Clínicas, 1Resident of Pathology at Hospital das Clínicas, 2Pathologist at Hospital das Clínicas, 3Neurosurgeon at Hospital das Clínicas, Botucatu Medical School ‑ São Paulo State University, Botucatu, Brazil E‑mail: [email protected]
References 1. c
d
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)
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. 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 446
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. 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. 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. 4. Kuratsu J, Seto H, Kochi M, Itoyama Y, Uemura S, Ushio Y. Metastatic angiosarcoma of the brain. Surg Neurol 1991;35:305‑9. 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. 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. 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. 8. Hwang SL, Howng SL, Sun ZM, Kwan AL. Brain metastasis from pericardial angiosarcoma. J Formos Med Assoc 1996;95:484‑6. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.141261
Received: 06‑05‑2014 Review completed: 07‑06‑2014 Accepted: 20‑06‑2014 Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
