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Giant tentorial cavernous hemangioma: Case report and review of literature
Clinical Neurology and Neurosurgery 113 (2011) 937–942
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Giant tentorial cavernous hemangioma: Case report and review of literature Sanjay Bhatia a,∗ , Brent R. O’Neill b , Frank Pu d , Khalid Aziz c a
Department of Neurosurgery, School of Medicine, West Virginia University, Morgantown, WV 26506, United States Neurosurgery, The Children’s Hospital, Aurora, CO, United States c Department of Neurosurgery, Allegheny General Hospital, Pittsburgh, PA, United States d Department of Pathology, Allegheny General Hospital, Pittsburgh, PA, United States b
a r t i c l e
i n f o
Article history: Received 21 September 2010 Received in revised form 20 June 2011 Accepted 1 July 2011 Available online 4 August 2011 Keywords: Cavernous malformation Cavernoma Cavernous hemangioma Dural tumor Tentorial tumor
1. Introduction Cavernous hemangiomas (also known as cavernous malformations, cavernous angiomas, or cavernomas) account for 8–15% of intracranial vascular malformations [1,2]. Histologically, they consist of sinusoidal vascular caverns lined by a single layer of endothelium and separated by fibrous stroma. There are no tight junctions. Mature vascular structures, smooth muscle (pericytes), elastin, and intervening brain tissue are absent while thrombus of varying ages is common [1]. Cavernous hemangiomas near the cavernous sinus are relatively uncommon, accounting for 3% of cavernous sinus lesion, while dural cavernous hemangioma outside of the middle fossa are truly rare with only 28 cases reported in the literature [3–27]. Cavernous hemangiomas regardless of location share the histopathologic features described above, but their clinical and radiographic features vary dramatically by location. Cavernomas located in the brain parenchyma are relatively common (0.4–0.9% of the population) [2], and are typically innocuous. They usually present with seizures, progressive deficits or small bleeds. These bleeds in the supratentorial location are usually small and without sequelae, but in critical locations like the brainstem, may cause severe deficits. Extra-axial cavernomas do not bleed and are usually discovered because of mass effect. While no consensus exists on when to call these lesions
∗ Corresponding author. Tel.: +1 304 293 5041; fax: +1 304 293 4819. E-mail addresses: [email protected], [email protected] (S. Bhatia). 0303-8467/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2011.07.007
“giant”, most authors use this term for lesions 6 cm or more in diameter [27]. We present a case of giant tentorial cavernoma, and review the clinical, imaging, and surgical considerations of this lesion. 2. Case report A 60-year-old male noted gait changes two years prior to presentation at our facility. The patient additionally noted some shaking of his left hand with activity that proved on physical exam to be dysmetria. Other pertinent exam findings include a normal Rhomberg test and some mild truncal ataxia. These symptoms had been progressive over the two-year time course, prompting elective neuroimaging. Non-contrast CT revealed a tentorial-based mass hyperdense to brain (Fig. 1). The lesion measured 6 cm × 6 cm × 4.5 cm, with its bulk primarily in the posterior fossa. A 2.5 cm knuckle extended across the tentorium. MRI showed the mass to be isointense on T1 imaging with avid, faintly speckled uniform contrast enhancement (Fig. 2a–c). On T2 weighted imaging, the mass was markedly hyperintense with fine speckling and faint stripes of lower signal. Nothing on the image suggested hemosiderin or hemorrhage. The mass significantly compressed the surrounding brain, but there was no associated edema. The differential diagnosis included meningioma, hemangiopericytoma, solitary fibrous tumor or metastatic lesion. The patient was taken to surgery. A craniotomy was fashioned spanning the transverse sinus to allow access to both the supra
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Fig. 1. Non-contrasted CT of the head showing a hyperdense tentorial mass with ventriculomegaly. Table 1 Ages and locations of previously reported non-middle fossa dural cavernomas. Author
Age (years)
Location
Canevini et al. [3] Dorner et al. [4] Fracasso [5] Goel et al. [6] Hsiang et al. [7] Hyodo et al. [8] Isla et al. [9] Ito et al. [10] Kaga et al. [11] Kunishio et al. [12] Lee et al. [13] Lewis et al. [14]
Neonate 37 ? 60 5 77 37 38 62 61 53 36 33 61 52 54 ? ? Neonate 35 77 63 66 Neonate 61 18 78 36
Convexity Falx Falx Cerebello-pontine Posterior fossa dura Convexity Anterior cranial fossa Convexity Falx Convexity Convexity Convexity Convexity Tentorial Tentorial Tentorial Petrosal sinus Torcula Tentorial Convexity Convexity Tentorial Convexity Convexity Posterior fossa dura Convexity Convexity Tentorial
Matsumo et al. [15] McCormick et al. [16] Meyer et al. [17] Moritake et al. [18] Okada et al. [19] Perry et al. [20] Quattrocchi et al. [21] Revuelta et al. [22] Saldana et al. [23] Sathi et al. [24] Shen et al. [25] Suzuki et al. [26] Van Lindert et al. [27]
and infra-tentorial spaces. The posterior fossa dura was opened initially, and the mass was exposed along its juncture with the tentorium. Its surface was dark purple with a vesicular character, similar to a blackberry. A clear double-arachnoid plane allowed for easy dissection of the lesion from the cerebellum. The mass was initially divided along its tentorial root, to separate the infra-tentorial portion from its blood supply. Constant oozing occurred from the incised areas of the mass, but no large vessels were encountered. This constant venous bleeding proved quite resistant to bipolar coagulation but would stop when the area was packed with a hemostatic agent (oxidized regenerated cellulose). Microscopic examination showed sinusoidal vascular channels lined with a single-cell layer of endothelium without intervening brain tissue. Rich fibrous stroma separated the vascular channels in much greater abundance than is typically seen in parenchymal cavernomas. Immunohistochemistry with epithelial membrane antigen (EMA) was negative for meningothelial cells in the stroma; immunostain with CD31 highlighted the endothelial cells; Verhoeff-van Gieson (VVG) stain was negative for elastic membrane in the vascular wall. These results confirmed the diagnosis of cavernoma (Fig. 3a–c). The patient recovered from surgery, and his neurologic deficits resolved.
S. Bhatia et al. / Clinical Neurology and Neurosurgery 113 (2011) 937–942
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Fig. 2. (a) T2 weighted MRI reveals a mass significantly compressing the brain but causing no associated brain edema. The lack of edema is a typical feature of dural cavernoma that distinguishes it from a meningioma of this size. (b) T1 weighted, contrasted MRI in the coronal plane demonstrates the bright but speckled enhancement. Foci of necrosis, as would be expected from a large meningioma, are absent. (c) Postoperative MRI showing complete resection.
3. Discussion Non-middle fossa dural cavernomas are quite rare. Only 28 cases have been previously reported [3–27] (Table 1). Three of these were identified at autopsy. Only ten of the reported cases underwent MR imaging while an almost equal number underwent angiogram. The imaging features of non-middle fossa dural cavernomas are similar to those of middle fossa dural cavernomas, but very distinct from lesions in the parenchyma. The clinical and surgical characteristics are distinct from cavernomas in all other locations [4]. The MRI appearance of parenchymal cavernomas is distinct enough to allow confident diagnosis on the basis of imaging alone, while dural cavernomas (both near the cavernous sinus and nonmiddle fossa) are usually mistaken for meningioma. Parenchymal cavernomas show a reticulated core with mixed signal intensity, surrounded by a distinct hemosiderin ring [2]. In contrast, dural cavernomas, both at the cavernous sinus and away, lack the features attributable to previous hemorrhage, showing instead a homogeneous mass. Dural cavernomas enhance intensely and uniformly, similar to meningioma. They are dural based, and frequently have an identifiable arachnoid cleft. Dural cavernomas even very large ones have not been reported to cause brain edema, a potentially distinguishing characteristic from meningioma.
Dural cavernomas are isodense to hyperdense on CT, and excepting a single case of acute subdural hematoma (described in further detail below), have not been reported to show any hemorrhage. Angiogram typically reveals a tumor blush or avascular mass effect. One exceptional case did show significant vascularity. Successful pre-operative embolization has never been reported, and in our opinion is unnecessary. Most non-middle fossa dural cavernomas identified on CT or MRI were mistaken for meningioma. In reviewing the imaging of our case and the extant literature, some clues to distinguishing this rare lesion from meningioma can be gleaned. First, the T2 MRI appearance in our case was remarkably hyperintense and uniform. This raised our suspicion that the lesion might not be a meningioma, but this finding has not been present in all cases reported in the literature. The remaining characteristics are most helpful for large lesions. As in our case, the large non-middle fossa dural cavernomas described in the literature have an irregular border, but no associated brain edema. Giant meningiomas with irregular borders are commonly WHO grade 2 or 3 histologically, and usually have significant associated edema. Higher-grade meningiomas also commonly have non-uniform enhancement, reflective of necrotic areas of tumor, whereas all of the reported giant dural cavernomas showed avid, uniform enhancement without necrosis.
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Fig. 2. (Continued)
Dural cavernous hemangiomas outside the middle cranial fossa have presented in two ways – mass effect, or incidental discovery [4]. About half of the reported non-middle fossa cavernomas were discovered incidentally, many at autopsy. Those identified incidentally on imaging were presumed to be meningioma. This high likelihood of incidental discovery matches the pre-
sentation of parenchymal cavernomas, but the lack of distinct imaging characteristics may result in under-recognition of this entity. The second presentation of non-middle fossa dural cavernomas, as in our case, is mass effect. Contrary to parenchymal and middle fossa cavernomas, a high percentage of non-middle fossa dural cavernomas have been of very large size. The slow growth, the rarity
S. Bhatia et al. / Clinical Neurology and Neurosurgery 113 (2011) 937–942
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Fig. 3. (a) H and E stained section showing dilated vascular channels lined with a single layer of endothelium separated by an abundance of benign fibrous stroma, far more than typically seen in parenchymal cavernomas. (b) Verhoeff-van Gieson (VVG) stain showing absence of elastin within the vasculature, ruling out AVM. (c) CD 31 stained section highlighting the single-cell layer of endothelium.
of hemorrhage, and the lack of associated brain edema probably afford these lesions the ability to grow to very large size asymptomatically. A few giant non-middle fossa dural cavernomas have presented in infants. Some have been detected on pre-natal ultrasound, while others have presented with large head and bulging fontanel. Non-middle fossa dural cavernomas are usually amenable to complete removal. Some reports of cavernous sinus cavernoma note that pre-operative field radiation decreases lesion size and operative bleeding, allowing for more complete resection. While we feel that this adjunct is typically unnecessary for lesions outside the middle cranial fossa, it may be considered in difficult to access lesions or patients with high risk of operative morbidity. Genetically cerebral intraparenchymal cavernous malformations are known to be associated with three cerebral cavernous malformation (CCM) genes, CCM-1, CCM-2 and CCM-3. The disease is autosomal dominant and almost all mutations encountered in the CCM genes are loss of function mutations. It has been suggested that a ‘second hit’ in a patient with an existing embryonal nonfunctioning CCM gene results in complete loss of function and proliferation of endothelial cells [28]. Similar mechanisms are likely to be operating in extraparenchymal cavernous malformations. In
this respect these lesions may be regarded more like endothelial cell tumors than vascular malformations. Further supporting this is the fact that these lesions may arise de novo, may enlarge, and are frequently seen after cranial radiation [1].
4. Conclusion Dural based cavernomas outside the middle fossa are rare lesions with imaging and clinical characteristics distinct from parenchymal cavernomas. Most reach a very large size before causing symptoms, and most are amenable to complete surgical resection. Imaging characteristics can give clues to proper preoperative diagnosis and management.
References [1] Allen JC, Miller DC, Budzilovich GN, Epstein FJ. Brain and spinal cord hemorrhage in long term survivors of malignant pediatric brain tumors: a possible late effect of therapy. Neurology 1991;41:148–50. [2] Bertalanffy H, Benes L, Miyazawa T, Olaf A, Siegel A, Sure U. Cerebral cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients. Neurosurg Rev 2001;25:1–53. [3] Canevini P, Farneti A, Flauto U. Report of a case of cavernous hemangioma of the dura mater in a 2-day old newborn. Folia Hered Pathol (Milano) 1963;12:163–6.
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[4] Dorner L, Buhl R, Hugo HH, Jansen O, Barth H, Mehdorn HM. Unusual locations for cavernous hemangiomas: report of two cases and review of the literature. Acta Neurochir (Wien) 2005;147:1091–6. [5] Fracasso L. A case of hemangioma of the falx. Riv Patol Nerv Ment 1947;68:214–26. [6] Goel A, Achwal S, Nagpal RD. Dural cavernous haemangioma of posterior cranial fossa. J Postgrad Med 1993;39:222–3. [7] Hsiang JN, Ng HK, Tsang RKY, Poon WS. Dural cavernous angioma in a child. Pediatr Neurosurg 1996;25:105–8. [8] Hyodo A, Yanaka K, Higuchi O, Tomono Y, Nose T. Giant interdural cavernous hemangioma at the convexity. Case illustration. J Neurosurg 2000;92:503. [9] Isla A, Roda JM, Alvarez F, Munoz J, Garcia E, Blazquez MG. Intracranial cavernous angioma in the dura. Neurosurgery 1989;25(4):657–9. [10] Ito J, Konno K, Sato I, Kamenyama S, Takeda S. Convexity cavernous hemangioma, its angiographic and CT findings. Report of a case. NoTo Shinkei 1997;30:737–47. [11] Kaga A, Isono M, Mori T, Kusakabe T, Okada H, Hori S. Cavernous angioma of falx cerebri; case report. No Shinkei Geka 1991;19:1079–83. [12] Kunishio K, Sunami N, Yamamoto Y, Satoh T, Asari S, Ohtsuki Y. A case of convexity cavernous hemangioma associated with meningioma. No Shinkei Geka 1986;14:1487–91. [13] Lee AG, Parrish RG, Goodman JC. Homonymous hemianopsia due to dural cavernous hemangioma. J Neuroopthalmol 1998;18:250–4. [14] Lewis AI, Tew Jr JM, Payner TD, Yeh HS. Cavernous angiomas outside the middle cranial fossa: a report of two cases. Neurosurgery 1994;35:498–504, discussion 504. [15] Matsumoto M, Nagata I, Kichuchi H, Yamagata S. A case of tentorial cavernous angioma. No Shinkei Geka 1988;16:403–7. [16] McCormick W, Boulter T. Vascular malformations (Angiomas) of the dura mater: report of two cases. J Neurosurg 1966;25:309–11.
[17] Meyer FB, Lombardi D, Scheithauer B, Nichols DA. Extra-axial cavernous hemangiomas involving the dural sinuses. J Neurosurg 1990;73(2): 187–92. [18] Moritake K, Handa H, Nozaki K, Tomiwa K. Tentorial cavernous angioma with calcification in a neonate. Neurosurgery 1985;16:207–11. [19] Okada J, Hara M, Takeuchi K. Dural haemangioma with extracranial component. Acta Neurochir (Wien) 1977;36:111–5. [20] Perry JR, Tucker WS, Chui M, Bilbao JM. Dural cavernous hemangioma: an under-recognized lesion mimicking meningioma. Can J Neurol Sci 1993;20:230–3. [21] Quattrocchi KB, Kissel P, Ellis WG, Frank EH. Cavernous angioma of the tentorium cerebelli. J Neurosurg 1989;71(6):935–7. [22] Revuelta R, Teixeira F, Rojas R, Juambelz P, Romero V, Valdes J. Cavernous hemangiomas of the dura mater at the convexity. Report of a case and therapeutical considerations. Neurosurg Rev 1994;17:309–11. [23] Saldana CJ, Zimman H, Alonso P, Mata PR. Neonatal cavernous hemangioma of the dura mater: case report. Neurosurgery 1991;29:602–5. [24] Sathi S, Folkerth R, Madsen JR. Cavernous angioma of the posterior fossa dura mimicking a meningioma: case report and review of literature. Surg Neurol 1992;38:257–60. [25] Shen WC, Chenn CA, Hsue CT, Lin TY. Dural cavernous angioma mimicking a meningioma and causing facial pain. J Neuroimaging 2000;10:183–5. [26] Suzuki K, Kamezaki T, Tsuboi K, Kobayashi E. Dural cavernous angioma causing acute subdural hemorrhage—case report. Neurol Med Chir (Tokyo) 1996;36:580–2. [27] Van Lindert EJ, Tan TC, Grotenhuis JA, Wesseling P. Giant cavernous hemangioma: report of three cases. Neurosurg Rev 2007;30:83–92. [28] Riant F, Bergametti F, Ayrignac X, Boulday G, Tournier-Lasserve E. Recent insights into cerebral cavernous malformations: the molecular genetics of CCM. FEBS J 2010;277:1070–5.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Giant tentorial cavernous hemangioma: Case report and review of literature Sanjay Bhatia a,∗ , Brent R. O’Neill b , Frank Pu d , Khalid Aziz c a
Department of Neurosurgery, School of Medicine, West Virginia University, Morgantown, WV 26506, United States Neurosurgery, The Children’s Hospital, Aurora, CO, United States c Department of Neurosurgery, Allegheny General Hospital, Pittsburgh, PA, United States d Department of Pathology, Allegheny General Hospital, Pittsburgh, PA, United States b
a r t i c l e
i n f o
Article history: Received 21 September 2010 Received in revised form 20 June 2011 Accepted 1 July 2011 Available online 4 August 2011 Keywords: Cavernous malformation Cavernoma Cavernous hemangioma Dural tumor Tentorial tumor
1. Introduction Cavernous hemangiomas (also known as cavernous malformations, cavernous angiomas, or cavernomas) account for 8–15% of intracranial vascular malformations [1,2]. Histologically, they consist of sinusoidal vascular caverns lined by a single layer of endothelium and separated by fibrous stroma. There are no tight junctions. Mature vascular structures, smooth muscle (pericytes), elastin, and intervening brain tissue are absent while thrombus of varying ages is common [1]. Cavernous hemangiomas near the cavernous sinus are relatively uncommon, accounting for 3% of cavernous sinus lesion, while dural cavernous hemangioma outside of the middle fossa are truly rare with only 28 cases reported in the literature [3–27]. Cavernous hemangiomas regardless of location share the histopathologic features described above, but their clinical and radiographic features vary dramatically by location. Cavernomas located in the brain parenchyma are relatively common (0.4–0.9% of the population) [2], and are typically innocuous. They usually present with seizures, progressive deficits or small bleeds. These bleeds in the supratentorial location are usually small and without sequelae, but in critical locations like the brainstem, may cause severe deficits. Extra-axial cavernomas do not bleed and are usually discovered because of mass effect. While no consensus exists on when to call these lesions
∗ Corresponding author. Tel.: +1 304 293 5041; fax: +1 304 293 4819. E-mail addresses: [email protected], [email protected] (S. Bhatia). 0303-8467/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2011.07.007
“giant”, most authors use this term for lesions 6 cm or more in diameter [27]. We present a case of giant tentorial cavernoma, and review the clinical, imaging, and surgical considerations of this lesion. 2. Case report A 60-year-old male noted gait changes two years prior to presentation at our facility. The patient additionally noted some shaking of his left hand with activity that proved on physical exam to be dysmetria. Other pertinent exam findings include a normal Rhomberg test and some mild truncal ataxia. These symptoms had been progressive over the two-year time course, prompting elective neuroimaging. Non-contrast CT revealed a tentorial-based mass hyperdense to brain (Fig. 1). The lesion measured 6 cm × 6 cm × 4.5 cm, with its bulk primarily in the posterior fossa. A 2.5 cm knuckle extended across the tentorium. MRI showed the mass to be isointense on T1 imaging with avid, faintly speckled uniform contrast enhancement (Fig. 2a–c). On T2 weighted imaging, the mass was markedly hyperintense with fine speckling and faint stripes of lower signal. Nothing on the image suggested hemosiderin or hemorrhage. The mass significantly compressed the surrounding brain, but there was no associated edema. The differential diagnosis included meningioma, hemangiopericytoma, solitary fibrous tumor or metastatic lesion. The patient was taken to surgery. A craniotomy was fashioned spanning the transverse sinus to allow access to both the supra
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Fig. 1. Non-contrasted CT of the head showing a hyperdense tentorial mass with ventriculomegaly. Table 1 Ages and locations of previously reported non-middle fossa dural cavernomas. Author
Age (years)
Location
Canevini et al. [3] Dorner et al. [4] Fracasso [5] Goel et al. [6] Hsiang et al. [7] Hyodo et al. [8] Isla et al. [9] Ito et al. [10] Kaga et al. [11] Kunishio et al. [12] Lee et al. [13] Lewis et al. [14]
Neonate 37 ? 60 5 77 37 38 62 61 53 36 33 61 52 54 ? ? Neonate 35 77 63 66 Neonate 61 18 78 36
Convexity Falx Falx Cerebello-pontine Posterior fossa dura Convexity Anterior cranial fossa Convexity Falx Convexity Convexity Convexity Convexity Tentorial Tentorial Tentorial Petrosal sinus Torcula Tentorial Convexity Convexity Tentorial Convexity Convexity Posterior fossa dura Convexity Convexity Tentorial
Matsumo et al. [15] McCormick et al. [16] Meyer et al. [17] Moritake et al. [18] Okada et al. [19] Perry et al. [20] Quattrocchi et al. [21] Revuelta et al. [22] Saldana et al. [23] Sathi et al. [24] Shen et al. [25] Suzuki et al. [26] Van Lindert et al. [27]
and infra-tentorial spaces. The posterior fossa dura was opened initially, and the mass was exposed along its juncture with the tentorium. Its surface was dark purple with a vesicular character, similar to a blackberry. A clear double-arachnoid plane allowed for easy dissection of the lesion from the cerebellum. The mass was initially divided along its tentorial root, to separate the infra-tentorial portion from its blood supply. Constant oozing occurred from the incised areas of the mass, but no large vessels were encountered. This constant venous bleeding proved quite resistant to bipolar coagulation but would stop when the area was packed with a hemostatic agent (oxidized regenerated cellulose). Microscopic examination showed sinusoidal vascular channels lined with a single-cell layer of endothelium without intervening brain tissue. Rich fibrous stroma separated the vascular channels in much greater abundance than is typically seen in parenchymal cavernomas. Immunohistochemistry with epithelial membrane antigen (EMA) was negative for meningothelial cells in the stroma; immunostain with CD31 highlighted the endothelial cells; Verhoeff-van Gieson (VVG) stain was negative for elastic membrane in the vascular wall. These results confirmed the diagnosis of cavernoma (Fig. 3a–c). The patient recovered from surgery, and his neurologic deficits resolved.
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Fig. 2. (a) T2 weighted MRI reveals a mass significantly compressing the brain but causing no associated brain edema. The lack of edema is a typical feature of dural cavernoma that distinguishes it from a meningioma of this size. (b) T1 weighted, contrasted MRI in the coronal plane demonstrates the bright but speckled enhancement. Foci of necrosis, as would be expected from a large meningioma, are absent. (c) Postoperative MRI showing complete resection.
3. Discussion Non-middle fossa dural cavernomas are quite rare. Only 28 cases have been previously reported [3–27] (Table 1). Three of these were identified at autopsy. Only ten of the reported cases underwent MR imaging while an almost equal number underwent angiogram. The imaging features of non-middle fossa dural cavernomas are similar to those of middle fossa dural cavernomas, but very distinct from lesions in the parenchyma. The clinical and surgical characteristics are distinct from cavernomas in all other locations [4]. The MRI appearance of parenchymal cavernomas is distinct enough to allow confident diagnosis on the basis of imaging alone, while dural cavernomas (both near the cavernous sinus and nonmiddle fossa) are usually mistaken for meningioma. Parenchymal cavernomas show a reticulated core with mixed signal intensity, surrounded by a distinct hemosiderin ring [2]. In contrast, dural cavernomas, both at the cavernous sinus and away, lack the features attributable to previous hemorrhage, showing instead a homogeneous mass. Dural cavernomas enhance intensely and uniformly, similar to meningioma. They are dural based, and frequently have an identifiable arachnoid cleft. Dural cavernomas even very large ones have not been reported to cause brain edema, a potentially distinguishing characteristic from meningioma.
Dural cavernomas are isodense to hyperdense on CT, and excepting a single case of acute subdural hematoma (described in further detail below), have not been reported to show any hemorrhage. Angiogram typically reveals a tumor blush or avascular mass effect. One exceptional case did show significant vascularity. Successful pre-operative embolization has never been reported, and in our opinion is unnecessary. Most non-middle fossa dural cavernomas identified on CT or MRI were mistaken for meningioma. In reviewing the imaging of our case and the extant literature, some clues to distinguishing this rare lesion from meningioma can be gleaned. First, the T2 MRI appearance in our case was remarkably hyperintense and uniform. This raised our suspicion that the lesion might not be a meningioma, but this finding has not been present in all cases reported in the literature. The remaining characteristics are most helpful for large lesions. As in our case, the large non-middle fossa dural cavernomas described in the literature have an irregular border, but no associated brain edema. Giant meningiomas with irregular borders are commonly WHO grade 2 or 3 histologically, and usually have significant associated edema. Higher-grade meningiomas also commonly have non-uniform enhancement, reflective of necrotic areas of tumor, whereas all of the reported giant dural cavernomas showed avid, uniform enhancement without necrosis.
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Fig. 2. (Continued)
Dural cavernous hemangiomas outside the middle cranial fossa have presented in two ways – mass effect, or incidental discovery [4]. About half of the reported non-middle fossa cavernomas were discovered incidentally, many at autopsy. Those identified incidentally on imaging were presumed to be meningioma. This high likelihood of incidental discovery matches the pre-
sentation of parenchymal cavernomas, but the lack of distinct imaging characteristics may result in under-recognition of this entity. The second presentation of non-middle fossa dural cavernomas, as in our case, is mass effect. Contrary to parenchymal and middle fossa cavernomas, a high percentage of non-middle fossa dural cavernomas have been of very large size. The slow growth, the rarity
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Fig. 3. (a) H and E stained section showing dilated vascular channels lined with a single layer of endothelium separated by an abundance of benign fibrous stroma, far more than typically seen in parenchymal cavernomas. (b) Verhoeff-van Gieson (VVG) stain showing absence of elastin within the vasculature, ruling out AVM. (c) CD 31 stained section highlighting the single-cell layer of endothelium.
of hemorrhage, and the lack of associated brain edema probably afford these lesions the ability to grow to very large size asymptomatically. A few giant non-middle fossa dural cavernomas have presented in infants. Some have been detected on pre-natal ultrasound, while others have presented with large head and bulging fontanel. Non-middle fossa dural cavernomas are usually amenable to complete removal. Some reports of cavernous sinus cavernoma note that pre-operative field radiation decreases lesion size and operative bleeding, allowing for more complete resection. While we feel that this adjunct is typically unnecessary for lesions outside the middle cranial fossa, it may be considered in difficult to access lesions or patients with high risk of operative morbidity. Genetically cerebral intraparenchymal cavernous malformations are known to be associated with three cerebral cavernous malformation (CCM) genes, CCM-1, CCM-2 and CCM-3. The disease is autosomal dominant and almost all mutations encountered in the CCM genes are loss of function mutations. It has been suggested that a ‘second hit’ in a patient with an existing embryonal nonfunctioning CCM gene results in complete loss of function and proliferation of endothelial cells [28]. Similar mechanisms are likely to be operating in extraparenchymal cavernous malformations. In
this respect these lesions may be regarded more like endothelial cell tumors than vascular malformations. Further supporting this is the fact that these lesions may arise de novo, may enlarge, and are frequently seen after cranial radiation [1].
4. Conclusion Dural based cavernomas outside the middle fossa are rare lesions with imaging and clinical characteristics distinct from parenchymal cavernomas. Most reach a very large size before causing symptoms, and most are amenable to complete surgical resection. Imaging characteristics can give clues to proper preoperative diagnosis and management.
References [1] Allen JC, Miller DC, Budzilovich GN, Epstein FJ. Brain and spinal cord hemorrhage in long term survivors of malignant pediatric brain tumors: a possible late effect of therapy. Neurology 1991;41:148–50. [2] Bertalanffy H, Benes L, Miyazawa T, Olaf A, Siegel A, Sure U. Cerebral cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients. Neurosurg Rev 2001;25:1–53. [3] Canevini P, Farneti A, Flauto U. Report of a case of cavernous hemangioma of the dura mater in a 2-day old newborn. Folia Hered Pathol (Milano) 1963;12:163–6.
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