- Email: [email protected]
Angioglioma of the Spinal Cord
Case Report
Angioglioma of the Spinal Cord Krishna Chaitanya Joshi1, Kiran Khanapure1, Nishchit Hegde1, Niveditha Ravindra2, Aniruddha T. Jagannatha1, Alangar S. Hegde1
Key words Angioglioma - Spinal tumor -
Abbreviations and Acronyms AVM: Arteriovenous malformation GFAP: Glial fibrillary acidic protein MRC: Medical Research Council MRI: Magnetic resonance imaging WHO: World Health Organization From the 1Department of Neurosurgery, M. S Ramaiah Medical College; and 2Department of Neuropathology, NIMHANS, Bangalore, India To whom correspondence should be addressed: Krishna Chaitanya Joshi, M.Ch. [E-mail: [email protected]] Citation: World Neurosurg. (2016). http://dx.doi.org/10.1016/j.wneu.2016.09.032
- BACKGROUND:
Angiogliomas are rare low-grade glial tumors with significant vascular components. These tumors are usually seen in the brain, and spinal cord angiogliomas have not been reported in the literature until now.
- CASE
DESCRIPTION: We report the case of a 15-year-old boy with an angioglioma of the medulla and cervicodorsal spine, which was completely excised through a combined suboccipital craniotomy and cervicodorsal laminotomy. The patient experienced excellent clinical recovery after the surgery, and follow-up contrast magnetic resonance imaging showed complete excision of the tumor.
- CONCLUSION:
The fact that increased vascularity in a glioma does not always indicate a higher grade is confirmed by the unique histology of angiogliomas. These tumors can present with intratumoral bleeding. Awareness of these entities is extremely important. Complete excision can be attempted, and the postoperative prognosis is very good.
Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
INTRODUCTION An angioglioma is defined as a tumor exhibiting characteristics of a low-grade glioma and a cavernous angioma, arteriovenous malformation (AVM), or hemangioblastoma. In 1914, Councillmann1 coined the term “angioglioma” to describe a cerebellar tumor with a large vascular component. In 1930, Roussey and Oberling2 described angioglioma in their classification of central nervous system neoplasms. Few authors have reported this tumor since then, owing to the controversy over its acceptance as a separate entity. Rubinstein3 suggested restricting the term angioglioma to denote a mixed tumor composed of a hemangioblastoma and an astrocytoma. Bonin et al4 opined that the term be limited to describe only a true mixed tumor of glial and vascular tissue origin. Here we report a 15-year-old boy diagnosed with an angioglioma of the cervical spinal cord, and discuss the various challenges faced in the diagnosis and management of this rare tumor.
CASE REPORT A 15-year-old male presented to us with a complaint of dull, aching neck pain beginning 1 year earlier, which was insidious in onset, progressive in intensity, and present continuously over the last 3 months. One month before consultation, he also began experiencing pain in both upper limbs radiating from the neck, predominately to the left side. The pain was aggravated by raising the arms above shoulder level or by lifting heavy objects. He also complained of weakness in the left upper limb proximally, as well as difficulty gripping objects with his left hand. He had no such symptoms in the lower limbs and no features suggestive of cranial nerve affection or sensory deficit. Bowel and bladder function was normal. He had no significant history of medical illness or any family history of similar tumors. Neurologic examination revealed a lower motor neuron deficit in both upper limbs with a Medical Research Council (MRC) grade of 3/5 and upper motor neuron type weakness in the lower limbs with an MRC grade of 4/5. All sensations were intact, and no cerebellar signs were evident. Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain and
WORLD NEUROSURGERY -: ---, MONTH 2016
cervicodorsal spine showed a large, illdefined, solid cystic intramedullary lesion extending from the lower medulla to the D3 level. The lesion was dorsally placed. The cystic component had fluid-fluid levels, indicating blood in the cystic areas. Hemosiderin caps covered the upper and lower ends of the lesion. The lesion was heterogeneously enhanced, with intense enhancement in the solid component at the midcervical region (Figure 1). A suboccipital craniotomy and cervicodorsal (up to the D4 level) laminotomy were performed. The lesion was noted in the medulla extending to the D3 level. The tumor was highly vascular, partly cystic and partly solid in consistency, and yellow-brown in color, with areas of hemorrhage and necrosis. There was a well-defined pseudogliotic plane around the tumor. An ultrasonic aspirator was used to achieve a gross total removal (Figure 2). Histopathological examination revealed both angioma and pilocytic astrocytoma components (Figure 3). Numerous venous channels of various sizes were evident (Figure 3A), several of which showed ferruginization and hyalinization of their
www.WORLDNEUROSURGERY.org
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CASE REPORT KRISHNA CHAITANYA JOSHI ET AL.
ANGIOGLIOMA OF THE SPINAL CORD
walls (Figure 3B). The cyst wall exhibited dense piloid gliosis and Rosenthal fibers (Figure 3C). Old hemorrhage was evident in the form of hemosiderin deposits (Figure 3C). Loose microcystic areas with eosinophilic granular bodies were present in the pilocytic astrocytoma component (Figure 3C, Inset). Based on these features, a histological diagnosis of medullary and cervicodorsal angioglioma, World Health Organization (WHO) grade 1, was made. The patient experienced an unremarkable postoperative recovery and good functional improvement. On clinical and radiologic follow-up at 6 weeks, he was completely independent in performing activities of daily living and exhibited significantly improved power in all limbs. Follow-up MRI revealed no residual tumor or recurrence (Figure 4).
DISCUSSION
Figure 1. Preoperative magnetic resonance imaging of the craniovertebral junction and cervicodorsal spine. (A) T1-weighted image showing a heterogeneous lesion with T1 hyperintensity in the lower medulla to vertebral level T3. (B) T1-weighted contrast-enhanced image showing a heterogeneously enhancing lesion with intense enhancement in the solid component at the cervical vertebral level. (C) Gradient echo sequence showing hemosiderin caps (white arrows) and fluid-fluid level (black arrow).
Figure 2. Intraoperative photograph showing an intramedullary yellow-brown vascular tumor with well-defined margins.
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www.SCIENCEDIRECT.com
Angiogliomas are rare low-grade, highly vascular tumors associated with favorable prognosis. Many authors believe that they need not be considered a separate type of neural tumor. Lombardi et al5 suggested that the term “angioglioma” be abandoned because of the lack of difference in clinical and prognostic features between gliomas with and without angiomatous components. However, other authors recognize these tumors as distinct from gliomas, cavernous angiomas, and hemangioblastomas and have proposed that angioglioma is a subtype of cerebral glial neoplasms. Our extensive review of the literature did not identify any cases of angioglioma reported in the spinal cord. Also striking is the fact that our patient presented with a tumor bleed, which has not been seen in any other cases of cerebral angioglioma reported to date. Angiogliomas as such are a rather puzzling entity without confirmation in the WHO brain tumors classification.6 The most common vascular anomalies in such tumors are AVM7-9 and cavernous angioma.10,11 Nonetheless, some authors have suggested that only true neoplastic vascular neoplasms, such as hemangioblastoma combined with gliomas, should be designated as angiogliomas.11
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2016.09.032
CASE REPORT KRISHNA CHAITANYA JOSHI ET AL.
ANGIOGLIOMA OF THE SPINAL CORD
Figure 3. Photomicrograph of the specimen. (A) Dilated venous channels (V) embedded in a densely fibrillary glial neoplasm (hematoxylin and eosin stain; original magnification, 20). (B) Variably sized ferruginized venous channels along the wall of the cyst (hematoxylin and eosin stain; original magnification, 10).
The glial components are varied and can range from fibrillary astrocytoma,12 pilocytic astrocytoma,13 astrocytoma with a papillary growth pattern,14 or pleomorphic xanthoastrocytoma15 to oligodendroglioma,8,10 mixed oligoastrocytoma,16 or ganglioneuroma.7 Diverse etiologic hypotheses have been proposed, including genetic predisposition,17 reactive glial response to AVM,5,18 viral origin,19 and exceptional
(C) Prominent Rosenthal fibers (black arrow) and hemosiderin-laden macrophages (black arrowhead) seen on the wall. (Inset) Eosinophilic granular body (black arrow) (hematoxylin and eosin stain; original magnification, 40).
coincidence between an AVM and a glioma.20-22 Imaging studies of these tumors generally show heterogenous enhancement on contrast MRI scans with perilesional edema. MRI also may reveal hypodense rings/caps owing to the paramagnetic effects of hemosiderin.23 In some cases, spinal angiography might be helpful to exclude AVM; however, in cases like our present case, in which a
Figure 4. Postoperative magnetic resonance imaging at 6 weeks after surgery. (A) T2-weighted image showing good decompression and no residual tumor. (B) T1-weighted contrast-enhanced image show no residual enhancing lesion.
WORLD NEUROSURGERY -: ---, MONTH 2016
clear solid component is visualized, angiography might not provide any further useful information. Generally, these tumors exhibit considerable vascularity in multiple areas of the tumor, along with hemorrhagic and necrotic areas, as was seen in our patient. They exhibit a well-defined plane and can be completely excised. Although the histological origin of such tumors is unclear, it has been hypothesized that they may arise from ordinary pilocytic astrocytomas with extensive vascular proliferation or from vascular tumors of hemangioblastoma origin that undergo neoplastic transformation of the glial component.24 On microscopic examination of common pilocytic glial astrocytoma-based angiogliomas, the astroglial component includes spindleshaped pilocytic astrocytes, numerous eosinophilic granular bodies, and Rosenthal fibers, whereas the vascular components are represented by abundant hyalinized capillary-like dilated vessels or an abnormally enlarged vein or artery resembling vessels similar to AVMs with multiple areas of hemosiderin deposits. This picture was seen on histopathological examination of the tumor excised from our patient as well. Areas adjacent to hemangioblastomas/ AVMs also exhibit a strong astroglial reaction; thus, it is important to distinguish the reactive astroglial cells from true neoplastic astrocytes. Immunohistochemical analysis shows the cytoplasm of these cells staining strongly for glial fibrillary acidic protein. This histological and immunohistochemical appearance of the
www.WORLDNEUROSURGERY.org
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CASE REPORT KRISHNA CHAITANYA JOSHI ET AL.
glial component is representative of true pilocytic astrocytoma rather than of reactive astrogliosis.24 In their review of the literature on angiogliomas, Gazzeri et al12,23 concluded that the prognosis of angiogliomatous tumors depends on various factors, including intrinsic biological behavior, malignancy and histological grading, location, extent of resection, and adjuvant therapies. The long-term prognosis of spinal angiogliomas is largely unknown. Although these tumors can masquerade as highly invasive tumors, a gross total excision can portend good prognosis. CONCLUSION Whether angiogliomas are considered a separate category of tumors or just the coincidental admixture of low-grade gliomas with extensive vascular components in contiguous regions, they represent a rare but important differential diagnosis to consider in tumors of the brain or spinal cord of high vascularity, and can be completely excised given their relatively benign nature and good prognosis. REFERENCES 1. Councilman WT. The gliomatous tumors of the brain. Long Island Med J. 1914;8:401-409. 2. Roussy G, Oberling C. Les tumeurs angiomateuses des centres nerveux. Presse Méd. 1930;38: 179-185. 3. Rubinstein LJ, Firminger HI. Tumors of the Central Nervous System. Washington, DC: Armed Forces Institute of Pathology; 1972. 4. Bonnin JM, Peña CE, Rubinstein LJ. Mixed capillary hemangioblastoma and glioma. A redefinition of the “angioglioma”. J Neuropathol Exp Neurol. 1983;42:504-516. 5. Lombardi D, Scheithauer BW, Piepgras D, Meyer FB, Forbes GS. “Angioglioma” and the
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www.SCIENCEDIRECT.com
ANGIOGLIOMA OF THE SPINAL CORD
arteriovenous malformation-glioma association. J Neurosurg. 1991;75:589-596. 6. Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Nervous System. Lyon, France: IARC Press; 2000. 7. Chovanes GI, Truex RC Jr. Association of a ganglioneuroma with an arteriovenous malformation: case report. Neurosurgery. 1987;21:241-243. 8. Crowell RM, DeGirolami U, Sweet WH. Arteriovenous malformation and oligodendroglioma: case report. J Neurosurg. 1975;43:108-111. 9. Goodkin R, Zaias B, Michelsen WJ. Arteriovenous malformation and glioma: coexistent or sequential? Case report. J Neurosurg. 1990;72:798-805. 10. Chee CP, Johnston R, Doyle D, Macpherson P. Oligodendroglioma and cerebral cavernous angioma: case report. J Neurosurg. 1985;62:145-147. 11. Hasegawa H, Bitoh S, Koshino K, Obashi J, Kobayashi Y, Kobayashi M, et al. Mixed cavernous angioma and glioma (angioglioma) in the hypothalamus: case report. Neurol Med Chir (Tokyo). 1995;35:238-242.
17. White RJ, Kernohan JW, Wood MW. A study of intracranial vascular tumors found incidentally at necropsy. J Neuropathol Exp Neurol. 1958;17:392-398. 18. Nazek M, Mandybur TI, Kashiwagi S. Oligodendroglial proliferative abnormality associated with arteriovenous malformation: report of three cases with review of the literature. Neurosurgery. 1988;23: 781-785. 19. Fischer EG, Sotrel A, Welch K. Cerebral hemangioma with glial neoplasia (angioglioma?): report of two cases. J Neurosurg. 1982;56:430-434. 20. Heffner RR Jr, Porro RS, Deck MD. Benign astrocytoma associated with arteriovenous malformation: case report. J Neurosurg. 1971;35:229-233. 21. Licata C, Pasqualin A, Freschini A, Barone G, Da Pian R. Management of associated primary cerebral neoplasms and vascular malformations, 2: intracranial arterio-venous malformations. Acta Neurochir (Wien). 1986;83:38-46. 22. Spetzler RF. Comment to Nazek. Neurosurgery. 1988;23:781-785.
12. Malcolm GP, Symon L, Tan LC, Pires M. Astrocytoma and associated arteriovenous malformation. Surg Neurol. 1991;36:59-62.
23. Gazzeri R, De Bonis C, Carotenuto V, Catapano D, d’Angelo V, Galarza M. Association between cavernous angioma and cerebral glioma: report of two cases and literature review of so-called angiogliomas. Neurocirugia (Astur). 2011;22:562-566.
13. Suzuki H, Uenohara H, Utsunomiya A, Kurihara N, Suzuki S, Tadokoro M, et al. A case of angioglioma composed of astrocytoma with a papillary growth pattern: immunohistochemical and ultrastructural studies. Brain Tumor Pathol. 2002;19:111-116.
24. Matyja E, Grajkowska W, Taraszewska A, Marchel A, Bojarski P, Nauman P. Advanced reactive astrogliosis associated with hemangioblastoma versus astroglial-vascular neoplasm (“angioglioma”). Folia Neuropathol. 2007;45: 120-125.
14. Jurco S 3rd, Nadji M, Harvey DG, Parker JC Jr, Font RL, Morales AR. Hemangioblastomas: histogenesis of the stromal cell studied by immunocytochemistry. Hum Pathol. 1982;13:13-18. 15. Lee TT, Landy HJ, Bruce JH. Arteriovenous malformation associated with pleomorphic xanthoastrocytoma. Acta Neurochir (Wien). 1996;138: 590-591.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Received 22 July 2016; accepted 8 September 2016 Citation: World Neurosurg. (2016). http://dx.doi.org/10.1016/j.wneu.2016.09.032 Journal homepage: www.WORLDNEUROSURGERY.org
16. Palma L, Mastronardi L, Celli P, d’Addetta R. Cavernous angioma associated with oligoastrocytoma-like proliferation. Acta Neurochir (Wien). 1995;133:169-173.
Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2016.09.032
Angioglioma of the Spinal Cord Krishna Chaitanya Joshi1, Kiran Khanapure1, Nishchit Hegde1, Niveditha Ravindra2, Aniruddha T. Jagannatha1, Alangar S. Hegde1
Key words Angioglioma - Spinal tumor -
Abbreviations and Acronyms AVM: Arteriovenous malformation GFAP: Glial fibrillary acidic protein MRC: Medical Research Council MRI: Magnetic resonance imaging WHO: World Health Organization From the 1Department of Neurosurgery, M. S Ramaiah Medical College; and 2Department of Neuropathology, NIMHANS, Bangalore, India To whom correspondence should be addressed: Krishna Chaitanya Joshi, M.Ch. [E-mail: [email protected]] Citation: World Neurosurg. (2016). http://dx.doi.org/10.1016/j.wneu.2016.09.032
- BACKGROUND:
Angiogliomas are rare low-grade glial tumors with significant vascular components. These tumors are usually seen in the brain, and spinal cord angiogliomas have not been reported in the literature until now.
- CASE
DESCRIPTION: We report the case of a 15-year-old boy with an angioglioma of the medulla and cervicodorsal spine, which was completely excised through a combined suboccipital craniotomy and cervicodorsal laminotomy. The patient experienced excellent clinical recovery after the surgery, and follow-up contrast magnetic resonance imaging showed complete excision of the tumor.
- CONCLUSION:
The fact that increased vascularity in a glioma does not always indicate a higher grade is confirmed by the unique histology of angiogliomas. These tumors can present with intratumoral bleeding. Awareness of these entities is extremely important. Complete excision can be attempted, and the postoperative prognosis is very good.
Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2016 Elsevier Inc. All rights reserved.
INTRODUCTION An angioglioma is defined as a tumor exhibiting characteristics of a low-grade glioma and a cavernous angioma, arteriovenous malformation (AVM), or hemangioblastoma. In 1914, Councillmann1 coined the term “angioglioma” to describe a cerebellar tumor with a large vascular component. In 1930, Roussey and Oberling2 described angioglioma in their classification of central nervous system neoplasms. Few authors have reported this tumor since then, owing to the controversy over its acceptance as a separate entity. Rubinstein3 suggested restricting the term angioglioma to denote a mixed tumor composed of a hemangioblastoma and an astrocytoma. Bonin et al4 opined that the term be limited to describe only a true mixed tumor of glial and vascular tissue origin. Here we report a 15-year-old boy diagnosed with an angioglioma of the cervical spinal cord, and discuss the various challenges faced in the diagnosis and management of this rare tumor.
CASE REPORT A 15-year-old male presented to us with a complaint of dull, aching neck pain beginning 1 year earlier, which was insidious in onset, progressive in intensity, and present continuously over the last 3 months. One month before consultation, he also began experiencing pain in both upper limbs radiating from the neck, predominately to the left side. The pain was aggravated by raising the arms above shoulder level or by lifting heavy objects. He also complained of weakness in the left upper limb proximally, as well as difficulty gripping objects with his left hand. He had no such symptoms in the lower limbs and no features suggestive of cranial nerve affection or sensory deficit. Bowel and bladder function was normal. He had no significant history of medical illness or any family history of similar tumors. Neurologic examination revealed a lower motor neuron deficit in both upper limbs with a Medical Research Council (MRC) grade of 3/5 and upper motor neuron type weakness in the lower limbs with an MRC grade of 4/5. All sensations were intact, and no cerebellar signs were evident. Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain and
WORLD NEUROSURGERY -: ---, MONTH 2016
cervicodorsal spine showed a large, illdefined, solid cystic intramedullary lesion extending from the lower medulla to the D3 level. The lesion was dorsally placed. The cystic component had fluid-fluid levels, indicating blood in the cystic areas. Hemosiderin caps covered the upper and lower ends of the lesion. The lesion was heterogeneously enhanced, with intense enhancement in the solid component at the midcervical region (Figure 1). A suboccipital craniotomy and cervicodorsal (up to the D4 level) laminotomy were performed. The lesion was noted in the medulla extending to the D3 level. The tumor was highly vascular, partly cystic and partly solid in consistency, and yellow-brown in color, with areas of hemorrhage and necrosis. There was a well-defined pseudogliotic plane around the tumor. An ultrasonic aspirator was used to achieve a gross total removal (Figure 2). Histopathological examination revealed both angioma and pilocytic astrocytoma components (Figure 3). Numerous venous channels of various sizes were evident (Figure 3A), several of which showed ferruginization and hyalinization of their
www.WORLDNEUROSURGERY.org
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CASE REPORT KRISHNA CHAITANYA JOSHI ET AL.
ANGIOGLIOMA OF THE SPINAL CORD
walls (Figure 3B). The cyst wall exhibited dense piloid gliosis and Rosenthal fibers (Figure 3C). Old hemorrhage was evident in the form of hemosiderin deposits (Figure 3C). Loose microcystic areas with eosinophilic granular bodies were present in the pilocytic astrocytoma component (Figure 3C, Inset). Based on these features, a histological diagnosis of medullary and cervicodorsal angioglioma, World Health Organization (WHO) grade 1, was made. The patient experienced an unremarkable postoperative recovery and good functional improvement. On clinical and radiologic follow-up at 6 weeks, he was completely independent in performing activities of daily living and exhibited significantly improved power in all limbs. Follow-up MRI revealed no residual tumor or recurrence (Figure 4).
DISCUSSION
Figure 1. Preoperative magnetic resonance imaging of the craniovertebral junction and cervicodorsal spine. (A) T1-weighted image showing a heterogeneous lesion with T1 hyperintensity in the lower medulla to vertebral level T3. (B) T1-weighted contrast-enhanced image showing a heterogeneously enhancing lesion with intense enhancement in the solid component at the cervical vertebral level. (C) Gradient echo sequence showing hemosiderin caps (white arrows) and fluid-fluid level (black arrow).
Figure 2. Intraoperative photograph showing an intramedullary yellow-brown vascular tumor with well-defined margins.
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www.SCIENCEDIRECT.com
Angiogliomas are rare low-grade, highly vascular tumors associated with favorable prognosis. Many authors believe that they need not be considered a separate type of neural tumor. Lombardi et al5 suggested that the term “angioglioma” be abandoned because of the lack of difference in clinical and prognostic features between gliomas with and without angiomatous components. However, other authors recognize these tumors as distinct from gliomas, cavernous angiomas, and hemangioblastomas and have proposed that angioglioma is a subtype of cerebral glial neoplasms. Our extensive review of the literature did not identify any cases of angioglioma reported in the spinal cord. Also striking is the fact that our patient presented with a tumor bleed, which has not been seen in any other cases of cerebral angioglioma reported to date. Angiogliomas as such are a rather puzzling entity without confirmation in the WHO brain tumors classification.6 The most common vascular anomalies in such tumors are AVM7-9 and cavernous angioma.10,11 Nonetheless, some authors have suggested that only true neoplastic vascular neoplasms, such as hemangioblastoma combined with gliomas, should be designated as angiogliomas.11
WORLD NEUROSURGERY, http://dx.doi.org/10.1016/j.wneu.2016.09.032
CASE REPORT KRISHNA CHAITANYA JOSHI ET AL.
ANGIOGLIOMA OF THE SPINAL CORD
Figure 3. Photomicrograph of the specimen. (A) Dilated venous channels (V) embedded in a densely fibrillary glial neoplasm (hematoxylin and eosin stain; original magnification, 20). (B) Variably sized ferruginized venous channels along the wall of the cyst (hematoxylin and eosin stain; original magnification, 10).
The glial components are varied and can range from fibrillary astrocytoma,12 pilocytic astrocytoma,13 astrocytoma with a papillary growth pattern,14 or pleomorphic xanthoastrocytoma15 to oligodendroglioma,8,10 mixed oligoastrocytoma,16 or ganglioneuroma.7 Diverse etiologic hypotheses have been proposed, including genetic predisposition,17 reactive glial response to AVM,5,18 viral origin,19 and exceptional
(C) Prominent Rosenthal fibers (black arrow) and hemosiderin-laden macrophages (black arrowhead) seen on the wall. (Inset) Eosinophilic granular body (black arrow) (hematoxylin and eosin stain; original magnification, 40).
coincidence between an AVM and a glioma.20-22 Imaging studies of these tumors generally show heterogenous enhancement on contrast MRI scans with perilesional edema. MRI also may reveal hypodense rings/caps owing to the paramagnetic effects of hemosiderin.23 In some cases, spinal angiography might be helpful to exclude AVM; however, in cases like our present case, in which a
Figure 4. Postoperative magnetic resonance imaging at 6 weeks after surgery. (A) T2-weighted image showing good decompression and no residual tumor. (B) T1-weighted contrast-enhanced image show no residual enhancing lesion.
WORLD NEUROSURGERY -: ---, MONTH 2016
clear solid component is visualized, angiography might not provide any further useful information. Generally, these tumors exhibit considerable vascularity in multiple areas of the tumor, along with hemorrhagic and necrotic areas, as was seen in our patient. They exhibit a well-defined plane and can be completely excised. Although the histological origin of such tumors is unclear, it has been hypothesized that they may arise from ordinary pilocytic astrocytomas with extensive vascular proliferation or from vascular tumors of hemangioblastoma origin that undergo neoplastic transformation of the glial component.24 On microscopic examination of common pilocytic glial astrocytoma-based angiogliomas, the astroglial component includes spindleshaped pilocytic astrocytes, numerous eosinophilic granular bodies, and Rosenthal fibers, whereas the vascular components are represented by abundant hyalinized capillary-like dilated vessels or an abnormally enlarged vein or artery resembling vessels similar to AVMs with multiple areas of hemosiderin deposits. This picture was seen on histopathological examination of the tumor excised from our patient as well. Areas adjacent to hemangioblastomas/ AVMs also exhibit a strong astroglial reaction; thus, it is important to distinguish the reactive astroglial cells from true neoplastic astrocytes. Immunohistochemical analysis shows the cytoplasm of these cells staining strongly for glial fibrillary acidic protein. This histological and immunohistochemical appearance of the
www.WORLDNEUROSURGERY.org
1.E3
CASE REPORT KRISHNA CHAITANYA JOSHI ET AL.
glial component is representative of true pilocytic astrocytoma rather than of reactive astrogliosis.24 In their review of the literature on angiogliomas, Gazzeri et al12,23 concluded that the prognosis of angiogliomatous tumors depends on various factors, including intrinsic biological behavior, malignancy and histological grading, location, extent of resection, and adjuvant therapies. The long-term prognosis of spinal angiogliomas is largely unknown. Although these tumors can masquerade as highly invasive tumors, a gross total excision can portend good prognosis. CONCLUSION Whether angiogliomas are considered a separate category of tumors or just the coincidental admixture of low-grade gliomas with extensive vascular components in contiguous regions, they represent a rare but important differential diagnosis to consider in tumors of the brain or spinal cord of high vascularity, and can be completely excised given their relatively benign nature and good prognosis. REFERENCES 1. Councilman WT. The gliomatous tumors of the brain. Long Island Med J. 1914;8:401-409. 2. Roussy G, Oberling C. Les tumeurs angiomateuses des centres nerveux. Presse Méd. 1930;38: 179-185. 3. Rubinstein LJ, Firminger HI. Tumors of the Central Nervous System. Washington, DC: Armed Forces Institute of Pathology; 1972. 4. Bonnin JM, Peña CE, Rubinstein LJ. Mixed capillary hemangioblastoma and glioma. A redefinition of the “angioglioma”. J Neuropathol Exp Neurol. 1983;42:504-516. 5. Lombardi D, Scheithauer BW, Piepgras D, Meyer FB, Forbes GS. “Angioglioma” and the
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www.SCIENCEDIRECT.com
ANGIOGLIOMA OF THE SPINAL CORD
arteriovenous malformation-glioma association. J Neurosurg. 1991;75:589-596. 6. Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Nervous System. Lyon, France: IARC Press; 2000. 7. Chovanes GI, Truex RC Jr. Association of a ganglioneuroma with an arteriovenous malformation: case report. Neurosurgery. 1987;21:241-243. 8. Crowell RM, DeGirolami U, Sweet WH. Arteriovenous malformation and oligodendroglioma: case report. J Neurosurg. 1975;43:108-111. 9. Goodkin R, Zaias B, Michelsen WJ. Arteriovenous malformation and glioma: coexistent or sequential? Case report. J Neurosurg. 1990;72:798-805. 10. Chee CP, Johnston R, Doyle D, Macpherson P. Oligodendroglioma and cerebral cavernous angioma: case report. J Neurosurg. 1985;62:145-147. 11. Hasegawa H, Bitoh S, Koshino K, Obashi J, Kobayashi Y, Kobayashi M, et al. Mixed cavernous angioma and glioma (angioglioma) in the hypothalamus: case report. Neurol Med Chir (Tokyo). 1995;35:238-242.
17. White RJ, Kernohan JW, Wood MW. A study of intracranial vascular tumors found incidentally at necropsy. J Neuropathol Exp Neurol. 1958;17:392-398. 18. Nazek M, Mandybur TI, Kashiwagi S. Oligodendroglial proliferative abnormality associated with arteriovenous malformation: report of three cases with review of the literature. Neurosurgery. 1988;23: 781-785. 19. Fischer EG, Sotrel A, Welch K. Cerebral hemangioma with glial neoplasia (angioglioma?): report of two cases. J Neurosurg. 1982;56:430-434. 20. Heffner RR Jr, Porro RS, Deck MD. Benign astrocytoma associated with arteriovenous malformation: case report. J Neurosurg. 1971;35:229-233. 21. Licata C, Pasqualin A, Freschini A, Barone G, Da Pian R. Management of associated primary cerebral neoplasms and vascular malformations, 2: intracranial arterio-venous malformations. Acta Neurochir (Wien). 1986;83:38-46. 22. Spetzler RF. Comment to Nazek. Neurosurgery. 1988;23:781-785.
12. Malcolm GP, Symon L, Tan LC, Pires M. Astrocytoma and associated arteriovenous malformation. Surg Neurol. 1991;36:59-62.
23. Gazzeri R, De Bonis C, Carotenuto V, Catapano D, d’Angelo V, Galarza M. Association between cavernous angioma and cerebral glioma: report of two cases and literature review of so-called angiogliomas. Neurocirugia (Astur). 2011;22:562-566.
13. Suzuki H, Uenohara H, Utsunomiya A, Kurihara N, Suzuki S, Tadokoro M, et al. A case of angioglioma composed of astrocytoma with a papillary growth pattern: immunohistochemical and ultrastructural studies. Brain Tumor Pathol. 2002;19:111-116.
24. Matyja E, Grajkowska W, Taraszewska A, Marchel A, Bojarski P, Nauman P. Advanced reactive astrogliosis associated with hemangioblastoma versus astroglial-vascular neoplasm (“angioglioma”). Folia Neuropathol. 2007;45: 120-125.
14. Jurco S 3rd, Nadji M, Harvey DG, Parker JC Jr, Font RL, Morales AR. Hemangioblastomas: histogenesis of the stromal cell studied by immunocytochemistry. Hum Pathol. 1982;13:13-18. 15. Lee TT, Landy HJ, Bruce JH. Arteriovenous malformation associated with pleomorphic xanthoastrocytoma. Acta Neurochir (Wien). 1996;138: 590-591.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Received 22 July 2016; accepted 8 September 2016 Citation: World Neurosurg. (2016). http://dx.doi.org/10.1016/j.wneu.2016.09.032 Journal homepage: www.WORLDNEUROSURGERY.org
16. Palma L, Mastronardi L, Celli P, d’Addetta R. Cavernous angioma associated with oligoastrocytoma-like proliferation. Acta Neurochir (Wien). 1995;133:169-173.
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