- Email: [email protected]
Intracranial hypertension caused by a meningioma compressing the transverse sinus
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1589–1592 9. Alapatt JP, Kutty RK, Gopi PP, et al. Middle and posterior fossa aspergilloma. Surg Neurol 2006;66:75–9. 10. Sabbah P, Bonardel G, Herve R, et al. CT and MRI findings in primary pituitary abscess: a case report and review of literature. J Neuroradiol 1999;26: 196–9.
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11. Walsh TJ, Pappas P, Winston DJ, et al. Voriconazole compared with liposomal amphotericin B for empirical antifungal therapy in patients with neutropenia and persistent fever. N Engl J Med 2002;346:225–34. 12. Herbrecht R, Denning DW, Patterson TF, et al. Voriconazole versus amphotericin B for primary therapy of invasive aspergillosis. N Engl J Med 2002;347:408–15.
doi:10.1016/j.jocn.2010.04.022
Intracranial hypertension caused by a meningioma compressing the transverse sinus Nicolas Chausson a,*, Jonathan Bocquet b, Mathieu Aveillan b, Stéphane Olindo a, Aïssatou Signaté a, Harold Merle c, Didier Smadja a a b c
Department of Neurology, University Hospital of Fort-de-France, Martinique, France Department of Neuroradiology, University Hospital of Fort-de-France, Martinique, France Department of Ophthalmology, University Hospital of Fort-de-France, Martinique, France
a r t i c l e
i n f o
Article history: Received 11 January 2010 Accepted 10 March 2010
Keywords: Intracranial hypertension Meningioma Stent placement Venous sinus
a b s t r a c t We report a 55-year-old woman with intracranial hypertension due to unilateral extrinsic compression of the left transverse sinus by a meningioma. Because of the high risk of the conventional neurosurgical intervention, she underwent an endovascular procedure consisting of a transstenotic stent placement in the left transverse sinus. One month after stenting, her ophthalmological examination revealed complete regression of the bilateral papilledema, with persistent improvement at 1 year. Cerebral venous-stenting could be a safe alternative for patients suffering from intracranial hypertension caused by extrinsic sinus compression. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Intracranial hypertension (ICH), defined as an increase of the intracranial pressure >20 cm H2O, manifests as headaches, vomiting and visual symptoms (transient visual obscurations, loss of visual acuity, horizontal diplopia).1 This visual loss is usually slow and insidious, but can sometimes be of sudden onset.2 The main causes of ICH are sinus venous thrombosis and drug intake (vitamin A, corticoids) but it can be idiopathic. We describe a woman with ICH caused by a meningioma compressing the left transverse sinus (TS) that was treated with sinus venous-stenting.
showed fluid accumulation in the optic nerve sheaths and reduced ventricle size. There were at least seven intracranial but extraparenchymal lesions, in contact with the meninges, and enhanced by gadolinium injection, highly suggestive of meningiomatosis. The meningioma adjacent to the superior sagittal sinus (SSS) compressed the left TS. Multi-detector CT venography showed a complete segmental obstruction of the left TS. The diagnosis of ICH was confirmed by measuring the cerebrospinal fluid (CSF) pressure at 28 cm H2O; CSF composition was normal. Due to the high risk of damaging the left TS during surgical resection of the compressive meningioma, we opted for radioguided endovascular venous stent insertion to relieve ICH.
2. Case report A 55-year-old Martinican woman, with no remarkable medical history, except for severe myopia, was admitted to the Neurology Department to investigate bilateral papilledema found incidentally during a routine ophthalmological examination. She was not taking any medications. She denied having headaches, reduced visual acuity or transient visual obscurations. She was not overweight (body mass index: 21). Visual acuity was normal under adequate correction (20/20, both eyes). Visual field assessment showed bilateral enlarged blind spots with central scotoma. Fundoscopy confirmed bilateral gradeIV papilledema with juxta-papillary retinal hemorrhages (Fig. 1a). Routine blood analyses were normal. The contrast CT scan of the brain revealed multiple calcified extraparenchymal lesions suggestive of multiple meningiomas (Fig. 2). Brain MRI (Fig. 3)
⇑ Corresponding author. Present address: C/– Kernau, 56450 Vannes, France. Tel.: +33 6 96 01 28 60. E-mail address: [email protected] (N. Chausson).
3. Stenting procedure and follow-up The stent was inserted under local anesthesia. Clopidogrel was given for 3 days before and was continued in combination with heparin during the endovascular procedure. First, a conventional intra-arterial catheter angiogram was performed. During the venous phase of arteriography, a 30-mm long occlusion of the left TS was seen. We chose the venous access closest to the lesion by direct puncture of the internal jugular vein under ultrasonography guidance. Indeed, this approach provides the maximum ‘‘push” to pass the junction of the jugular vein–transverse sinus and the compressed part of the sinus. A 6F trocar was directed into the TS through a percutaneous puncture of the left internal jugular. A 5F vertebral catheter mounted on a standard guidewire (0.035 Terumo Standard Guide Wire M, Terumo Medical Corporation, Somerset, NJ, USA) was used to pass through the occluded TS. A larger catheter provides more ‘‘push” to get through the transverse sinus obstruction. The angiographic series of the SSS obtained using the catheter confirmed left TS occlusion. A self-expanding stent
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Case Reports / Journal of Clinical Neuroscience 17 (2010) 1589–1592
At the 1-month follow-up visit, fundoscopy showed a clear-cut regression of the papilledema and the disappearance of the peripapillary hemorrhage (Fig. 1b). At 3 months, the regression was complete and the patency of the stent was demonstrated by CT angiography (Fig. 5). Fundoscopy at 1 year confirmed the persistence of the improvement with a stability of the meningioma compressing the left TS on the 1-year CT scan.
4. Discussion
Fig. 1. Fundoscopy (a) before surgery showing bilateral papillary blood congestion accompanied by a peripapillary hemorrhage crossing the 2 o’clock meridian in the left eye: note the moderate bilateral papillary edema; and (b) 1 month after the venous stenting procedure showing the clear-cut regression of the bilateral papillary edema and the disappearance of the peripapillary hemorrhage. (This figure is available in colour at www.sciencedirect.com.)
(8 mm 40 mm Astron Pulsar Stent Device: Biotronik; Berlin, Germany) easily crossed through the compressed zone and spanned the confluence of sinuses into the left TS, causing a transient leftside headache. Angiographic control was satisfactory with no need for additional balloon angioplasty (Fig. 4). The procedure lasted 50 minutes. After TS stenting, the patient was monitored overnight in the intensive care unit and discharged 48 hours later. Clopidogrel was continued for 3 months, the presumed time required for endothelialization of the stent.
Fortuitously discovered papilledema can sometimes be the sign leading to ICH diagnosis,3 especially in children.4 These forms without headaches carry the same vision-loss risk as those with headaches; they can lead insidiously to permanent visual impairment, due to prolonged papilledema with secondary optic atrophy, and thus require treatment.5 We postulated that our patient’s ICH resulted from the unilateral compression of the left TS by the meningioma, causing venous obstruction, but not from the tumor itself because of its small size and the absence of surrounding edema. The elevated pressure in the venous sinus created an inverse pressure gradient at the level of the arachnoid granulation and prevented CSF resorption, leading to ICH. This ICH-triggering mechanism has been previously described in extrinsic median compression of the superior longitudinal sinus by benign or malignant tumors.6,7 But Damak demonstrated that unilateral intrinsic obstruction with isolated lateral sinus thrombosis was sufficient to cause ICH-induced bilateral papilledema in 22% of their 62 patients, particularly when associated with contralateral hypoplasia or extension to the torcular of Herophilus.8 Ideal treatment of our patient would have been complete resection of the compressive meningioma. However, despite advances in microsurgical techniques,9 meningiomas adjacent to the posterior
Fig. 2. Axial contrast-enhanced CT scan showing multiple meningiomas, most of them totally calcified (falx cerebri, right frontal) or partially calcified (right temporal, left temporal, posterior adjacent to confluence of sinuses).
Fig. 3. T1-weighted cerebral MRI showing a well-defined occlusion-causing meningioma, intensely enhanced after intravenous gadolinium injection, next to the left transverse sinus and confluence of the sinuses (left to right: axial, sagittal and coronal).
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1589–1592
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Fig. 4. Venous phase of a conventional angiogram showing: (a) a 30-mm occlusion of the left transverse sinus (TS); (b) the hydrophilic catheter passing through the stenosis; (c) opacification after stent placement and the complete restoration of normal venous flow; and (d) stent placed in the confluence of the sinuses and left TS.
ments unsatisfactory, especially because of their poor long-term tolerance. There are several limits to our report: we did not measure the transstenotic venous pressure gradient during the endovascular procedure and a CSF-pressure decrease was not documented by a new lumbar puncture after stent placement. However, the complete regression of the bilateral papilledema 3 months after the endovascular procedure and maintained at 1 year corroborate the good therapeutic results. 5. Conclusion
Fig. 5. Control contrast-enhanced CT scan (curved reconstruction in stent axis) at 3 months post-intervention showing stent permeability.
ICH can be the consequence of unilateral TS compression. Cerebral venous-stenting may be an alternative therapeutic option for patients suffering from ICH caused by tumoral venous compression, when surgical resection of the lesion carries unacceptable risk. Acknowledgment
third of the SSS are challenging to remove completely without significant morbidity (around 10%).9,10 The superficial veins of the cerebrum entering the SSS must be spared during meningioma excision to prevent brain swelling and venous infarction. Stereotactic radiosurgery was an alternative therapeutic option for our patient but was rejected, because of its very delayed effect on tumoral volume and the frequent morbidity linked to post-radiotherapy edema.11,12 Recently, endoluminal venous sinus stenting was proposed as an alternative approach for patients with idiopathic ICH, because bilateral TS stenoses appeared in >90% of these patients (versus 7% for controls) with abnormal intracranial venous pressure in some reported patients.13 Although it is not totally clear whether cranial venous outflow obstruction is the primary or a secondary process in idiopathic ICH,14,15 treatment of focal TS stenotic lesions with transstenotic venous sinus-stenting is a promising therapeutic alternative in this setting: no patient’s clinical status deteriorated after angioplasty, which proved beneficial in >50% of patients.16,17 But the long-term safety and efficacy of this technique has yet to be proven. However, a promising report on stent placement for management of a parasagittal meningioma revealed by isolated ICH demonstrated good stenting efficacy at 14 months, despite a theoretically growing tumor.7 Supported by these recent observations, we opted for a compromise between symptomatic medical treatment and risky surgical management, treating our patient with this original approach of placing a stent in a venous sinus, a technically straightforward intervention. Indeed, the onset of visual field deterioration and the tumoral compression etiology led us to consider medical treat-
The authors thank Janet Jacobson for editorial assistance. References 1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492–5. 2. Thambisetty M, Lavin PJ, Newman NJ, et al. Fulminant idiopathic intracranial hypertension. Neurology 2007;68:229–32. 3. Strominger MB, Weiss GB, Mehler MF. Asymptomatic unilateral papilledema in pseudotumor cerebri. J Clin Neuroophthalmol 1992;12:238–41. 4. Bassan H, Berkner L, Stolovitch C, et al. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 2008;118:251–5. 5. Shah VA, Kardon RH, Lee AG, et al. Long-term follow-up of idiopathic intracranial hypertension: the Iowa experience. Neurology 2008;70:634–40. 6. Kim AW, Trobe JD. Syndrome simulating pseudotumor cerebri caused by partial transverse venous sinus obstruction in metastatic prostate cancer. Am J Ophthalmol 2000;129:254–6. 7. Ganesan D, Higgins JN, Harrower T, et al. Stent placement for management of a small parasagittal meningioma. Technical note. J Neurosurg 2008;108:377–81. 8. Damak M, Crassard I, Wolff V, et al. Isolated lateral sinus thrombosis: a series of 62 patients. Stroke 2009;40:476–81. 9. Sindou MP, Alvernia JE. Results of attempted radical tumor removal and venous repair in 100 consecutive meningiomas involving the major dural sinuses. J Neurosurg 2006;105:514–25. 10. DiMeco F, Li KW, Casali C, et al. Meningiomas invading the superior sagittal sinus: surgical experience in 108 cases. Neurosurgery 2008;62:1124–35. 11. Kondziolka D, Flickinger JC, Perez B. Judicious resection and/or radiosurgery for parasagittal meningiomas: outcomes from a multicenter review. Gamma Knife Meningioma Study Group. Neurosurgery 1998;43:405–13. 12. Higgins JN, Burnet NG, Schwindack CF, et al. Severe brain edema caused by a meningioma obstructing cerebral venous outflow and treated with venous sinus stenting. Case report. J Neurosurg 2008;108:372–6. 13. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003;60: 1418–24. 14. Stienen A, Weinzierl M, Ludolph A, et al . Obstruction of cerebral venous sinus secondary to idiopathic intracranial hypertension. Eur J Neurol 2008;15: 1416–8.
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Case Reports / Journal of Clinical Neuroscience 17 (2010) 1592–1594
15. Corbett JJ, Digre K. Idiopathic intracranial hypertension: an answer to ‘‘the chicken or the egg?”. Neurology 2002;58:5–6. 16. Higgins JN, Cousins C, Owler BK, et al. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. Neurol Neurosurg Psychiatry 2003;74:1662–6.
17. Donnet A, Metellus P, Levrier O, et al. Endovascular treatment of idiopathic intracranial hypertension: clinical and radiologic outcome of 10 consecutive patients. Neurology 2008;70:641–7.
doi:10.1016/j.jocn.2010.03.039
Paraspinal malignant ossifying fibromyxoid tumor with spinal involvement Jaypal Reddy Sangala a, Paul Park a,⇑, Mila Blaivas b, Frank LaMarca a b
a
Department of Neurosurgery, University of Michigan Health System, 1500 E. Medical Center Drive, Room 3552 TC, Ann Arbor, Michigan 48109-5338, USA Department of Pathology, University of Michigan Health System, Ann Arbor, Michigan, USA
a r t i c l e
i n f o
Article history: Received 18 January 2010 Accepted 25 April 2010
Keywords: Ossifying tumors Soft tissue tumors Spine tumors Vertebral tumors
a b s t r a c t Ossifying fibromyxoid tumors (OFT), first described in 1989 by Enzinger et al., are rare lesions; malignant OFT (MOFT) are even rarer. We report a large recurrent paraspinal MOFT invading the spine and causing epidural compression in a 70-year-old male, despite prior debulking and radiotherapy. Paraspinal involvement of these tumors has been reported only twice before. We describe its imaging, pathology, and also review the pertinent literature. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Ossifying fibromyxoid tumors (OFT) are rare lesions arising from soft tissues. Approximately 220 such tumors have been reported.1 OFT were first described by Enzinger et al. in 1989.2 Malignant OFT (MOFT) are a subtype of these lesions that demonstrate malignant or atypical features.3 Although there has been one case report of an OFT infiltrating the spine, a MOFT invading the spine causing epidural compression has not been reported. We report such a lesion, and describe its imaging and pathological importance. The pertinent literature is also reviewed. 2. Case report A 73-year-old man presented to a regional hospital with lower back and hip pain radiating down his right anterior thigh in 2007. MRI revealed a large posterior paraspinal mass adjacent to the right L2 vertebra involving the right pedicle, lamina, and transverse process. The mass measured about 7 6 8 cm, extending superiorly to L1 and inferiorly to S1. There was an L2 intraspinal and extradural extension of the lesion via the right neural foramen, which was enlarged. This lesion was surgically debulked in March 2007. Histopathology of the tumor indicated a MOFT with an infiltrative growth pattern. Postoperative imaging revealed a decreased but still substantial amount of tumor. The patient received 70 Gy of radiation therapy. In October 2008, the patient presented to our institution with recurrent severe mechanical low back pain, right anterior thigh pain, and neurogenic claudication. MRI revealed an abnormally enhancing heterogeneous soft tissue mass centered upon the right-sided posterior elements of the L2 and L3 vertebra (Fig. 1).
⇑ Corresponding author. Tel.: +1 734 615 2627; fax: +1 734 936 9294. E-mail address: [email protected] (P. Park).
There was effacement of the central canal at the level of the tumor. Severe central canal stenosis was present at L3–L4 due to degenerative changes and tumor. Infiltrative changes were also identified within the right posterior paraspinal musculature. Substantial degenerative changes of the lumbar spine were identified including severe central canal narrowing at L4–L5. The tumor was a poorly circumscribed, rubbery mass located in the right paraspinal muscle with significant intracanalicular extension. In view of the diffuse nature of the tumor, complete surgical excision of the lesion was not attempted. The paraspinal tumor was debulked, and the nerve roots were decompressed. A T12–L4 instrumented posterior spinal fusion was also performed. At the 1-month follow-up, the patient had a significant reduction in both back pain and limb pain. At 4 months, the patient had a repeat MRI of the lumbosacral spine that showed recurrent/residual tumor without significant neural compression. The patient refused palliative chemotherapy in a clinical trial offered by the oncology service. One month later, the patient presented to the emergency room with significant weakness in both legs. Imaging revealed recurrent tumor with significant extension into the spinal canal. Various treatment options were discussed, but the patient refused further treatment. 2.1. Histopathology Microscopically, the tumor demonstrated features of OFT consisting of lobules of ovoid mesenchymal cells within a fibromyxoid to hyalinized matrix with areas of bone formation (Supplementary Fig. 1). Very brisk mitotic activity in areas of high cellularity was noted. In addition, there were focal areas of high-grade nuclear atypia. The specimen had an infiltrative growth pattern into the surrounding skeletal muscle and adipose tissue and involved the margins of excision fairly extensively. Based on the presence of high cellularity and high mitotic activity, the tumor was determined to be a MOFT, according to the criteria proposed by Folpe and Weiss.3
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11. Walsh TJ, Pappas P, Winston DJ, et al. Voriconazole compared with liposomal amphotericin B for empirical antifungal therapy in patients with neutropenia and persistent fever. N Engl J Med 2002;346:225–34. 12. Herbrecht R, Denning DW, Patterson TF, et al. Voriconazole versus amphotericin B for primary therapy of invasive aspergillosis. N Engl J Med 2002;347:408–15.
doi:10.1016/j.jocn.2010.04.022
Intracranial hypertension caused by a meningioma compressing the transverse sinus Nicolas Chausson a,*, Jonathan Bocquet b, Mathieu Aveillan b, Stéphane Olindo a, Aïssatou Signaté a, Harold Merle c, Didier Smadja a a b c
Department of Neurology, University Hospital of Fort-de-France, Martinique, France Department of Neuroradiology, University Hospital of Fort-de-France, Martinique, France Department of Ophthalmology, University Hospital of Fort-de-France, Martinique, France
a r t i c l e
i n f o
Article history: Received 11 January 2010 Accepted 10 March 2010
Keywords: Intracranial hypertension Meningioma Stent placement Venous sinus
a b s t r a c t We report a 55-year-old woman with intracranial hypertension due to unilateral extrinsic compression of the left transverse sinus by a meningioma. Because of the high risk of the conventional neurosurgical intervention, she underwent an endovascular procedure consisting of a transstenotic stent placement in the left transverse sinus. One month after stenting, her ophthalmological examination revealed complete regression of the bilateral papilledema, with persistent improvement at 1 year. Cerebral venous-stenting could be a safe alternative for patients suffering from intracranial hypertension caused by extrinsic sinus compression. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Intracranial hypertension (ICH), defined as an increase of the intracranial pressure >20 cm H2O, manifests as headaches, vomiting and visual symptoms (transient visual obscurations, loss of visual acuity, horizontal diplopia).1 This visual loss is usually slow and insidious, but can sometimes be of sudden onset.2 The main causes of ICH are sinus venous thrombosis and drug intake (vitamin A, corticoids) but it can be idiopathic. We describe a woman with ICH caused by a meningioma compressing the left transverse sinus (TS) that was treated with sinus venous-stenting.
showed fluid accumulation in the optic nerve sheaths and reduced ventricle size. There were at least seven intracranial but extraparenchymal lesions, in contact with the meninges, and enhanced by gadolinium injection, highly suggestive of meningiomatosis. The meningioma adjacent to the superior sagittal sinus (SSS) compressed the left TS. Multi-detector CT venography showed a complete segmental obstruction of the left TS. The diagnosis of ICH was confirmed by measuring the cerebrospinal fluid (CSF) pressure at 28 cm H2O; CSF composition was normal. Due to the high risk of damaging the left TS during surgical resection of the compressive meningioma, we opted for radioguided endovascular venous stent insertion to relieve ICH.
2. Case report A 55-year-old Martinican woman, with no remarkable medical history, except for severe myopia, was admitted to the Neurology Department to investigate bilateral papilledema found incidentally during a routine ophthalmological examination. She was not taking any medications. She denied having headaches, reduced visual acuity or transient visual obscurations. She was not overweight (body mass index: 21). Visual acuity was normal under adequate correction (20/20, both eyes). Visual field assessment showed bilateral enlarged blind spots with central scotoma. Fundoscopy confirmed bilateral gradeIV papilledema with juxta-papillary retinal hemorrhages (Fig. 1a). Routine blood analyses were normal. The contrast CT scan of the brain revealed multiple calcified extraparenchymal lesions suggestive of multiple meningiomas (Fig. 2). Brain MRI (Fig. 3)
⇑ Corresponding author. Present address: C/– Kernau, 56450 Vannes, France. Tel.: +33 6 96 01 28 60. E-mail address: [email protected] (N. Chausson).
3. Stenting procedure and follow-up The stent was inserted under local anesthesia. Clopidogrel was given for 3 days before and was continued in combination with heparin during the endovascular procedure. First, a conventional intra-arterial catheter angiogram was performed. During the venous phase of arteriography, a 30-mm long occlusion of the left TS was seen. We chose the venous access closest to the lesion by direct puncture of the internal jugular vein under ultrasonography guidance. Indeed, this approach provides the maximum ‘‘push” to pass the junction of the jugular vein–transverse sinus and the compressed part of the sinus. A 6F trocar was directed into the TS through a percutaneous puncture of the left internal jugular. A 5F vertebral catheter mounted on a standard guidewire (0.035 Terumo Standard Guide Wire M, Terumo Medical Corporation, Somerset, NJ, USA) was used to pass through the occluded TS. A larger catheter provides more ‘‘push” to get through the transverse sinus obstruction. The angiographic series of the SSS obtained using the catheter confirmed left TS occlusion. A self-expanding stent
1590
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1589–1592
At the 1-month follow-up visit, fundoscopy showed a clear-cut regression of the papilledema and the disappearance of the peripapillary hemorrhage (Fig. 1b). At 3 months, the regression was complete and the patency of the stent was demonstrated by CT angiography (Fig. 5). Fundoscopy at 1 year confirmed the persistence of the improvement with a stability of the meningioma compressing the left TS on the 1-year CT scan.
4. Discussion
Fig. 1. Fundoscopy (a) before surgery showing bilateral papillary blood congestion accompanied by a peripapillary hemorrhage crossing the 2 o’clock meridian in the left eye: note the moderate bilateral papillary edema; and (b) 1 month after the venous stenting procedure showing the clear-cut regression of the bilateral papillary edema and the disappearance of the peripapillary hemorrhage. (This figure is available in colour at www.sciencedirect.com.)
(8 mm 40 mm Astron Pulsar Stent Device: Biotronik; Berlin, Germany) easily crossed through the compressed zone and spanned the confluence of sinuses into the left TS, causing a transient leftside headache. Angiographic control was satisfactory with no need for additional balloon angioplasty (Fig. 4). The procedure lasted 50 minutes. After TS stenting, the patient was monitored overnight in the intensive care unit and discharged 48 hours later. Clopidogrel was continued for 3 months, the presumed time required for endothelialization of the stent.
Fortuitously discovered papilledema can sometimes be the sign leading to ICH diagnosis,3 especially in children.4 These forms without headaches carry the same vision-loss risk as those with headaches; they can lead insidiously to permanent visual impairment, due to prolonged papilledema with secondary optic atrophy, and thus require treatment.5 We postulated that our patient’s ICH resulted from the unilateral compression of the left TS by the meningioma, causing venous obstruction, but not from the tumor itself because of its small size and the absence of surrounding edema. The elevated pressure in the venous sinus created an inverse pressure gradient at the level of the arachnoid granulation and prevented CSF resorption, leading to ICH. This ICH-triggering mechanism has been previously described in extrinsic median compression of the superior longitudinal sinus by benign or malignant tumors.6,7 But Damak demonstrated that unilateral intrinsic obstruction with isolated lateral sinus thrombosis was sufficient to cause ICH-induced bilateral papilledema in 22% of their 62 patients, particularly when associated with contralateral hypoplasia or extension to the torcular of Herophilus.8 Ideal treatment of our patient would have been complete resection of the compressive meningioma. However, despite advances in microsurgical techniques,9 meningiomas adjacent to the posterior
Fig. 2. Axial contrast-enhanced CT scan showing multiple meningiomas, most of them totally calcified (falx cerebri, right frontal) or partially calcified (right temporal, left temporal, posterior adjacent to confluence of sinuses).
Fig. 3. T1-weighted cerebral MRI showing a well-defined occlusion-causing meningioma, intensely enhanced after intravenous gadolinium injection, next to the left transverse sinus and confluence of the sinuses (left to right: axial, sagittal and coronal).
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1589–1592
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Fig. 4. Venous phase of a conventional angiogram showing: (a) a 30-mm occlusion of the left transverse sinus (TS); (b) the hydrophilic catheter passing through the stenosis; (c) opacification after stent placement and the complete restoration of normal venous flow; and (d) stent placed in the confluence of the sinuses and left TS.
ments unsatisfactory, especially because of their poor long-term tolerance. There are several limits to our report: we did not measure the transstenotic venous pressure gradient during the endovascular procedure and a CSF-pressure decrease was not documented by a new lumbar puncture after stent placement. However, the complete regression of the bilateral papilledema 3 months after the endovascular procedure and maintained at 1 year corroborate the good therapeutic results. 5. Conclusion
Fig. 5. Control contrast-enhanced CT scan (curved reconstruction in stent axis) at 3 months post-intervention showing stent permeability.
ICH can be the consequence of unilateral TS compression. Cerebral venous-stenting may be an alternative therapeutic option for patients suffering from ICH caused by tumoral venous compression, when surgical resection of the lesion carries unacceptable risk. Acknowledgment
third of the SSS are challenging to remove completely without significant morbidity (around 10%).9,10 The superficial veins of the cerebrum entering the SSS must be spared during meningioma excision to prevent brain swelling and venous infarction. Stereotactic radiosurgery was an alternative therapeutic option for our patient but was rejected, because of its very delayed effect on tumoral volume and the frequent morbidity linked to post-radiotherapy edema.11,12 Recently, endoluminal venous sinus stenting was proposed as an alternative approach for patients with idiopathic ICH, because bilateral TS stenoses appeared in >90% of these patients (versus 7% for controls) with abnormal intracranial venous pressure in some reported patients.13 Although it is not totally clear whether cranial venous outflow obstruction is the primary or a secondary process in idiopathic ICH,14,15 treatment of focal TS stenotic lesions with transstenotic venous sinus-stenting is a promising therapeutic alternative in this setting: no patient’s clinical status deteriorated after angioplasty, which proved beneficial in >50% of patients.16,17 But the long-term safety and efficacy of this technique has yet to be proven. However, a promising report on stent placement for management of a parasagittal meningioma revealed by isolated ICH demonstrated good stenting efficacy at 14 months, despite a theoretically growing tumor.7 Supported by these recent observations, we opted for a compromise between symptomatic medical treatment and risky surgical management, treating our patient with this original approach of placing a stent in a venous sinus, a technically straightforward intervention. Indeed, the onset of visual field deterioration and the tumoral compression etiology led us to consider medical treat-
The authors thank Janet Jacobson for editorial assistance. References 1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492–5. 2. Thambisetty M, Lavin PJ, Newman NJ, et al. Fulminant idiopathic intracranial hypertension. Neurology 2007;68:229–32. 3. Strominger MB, Weiss GB, Mehler MF. Asymptomatic unilateral papilledema in pseudotumor cerebri. J Clin Neuroophthalmol 1992;12:238–41. 4. Bassan H, Berkner L, Stolovitch C, et al. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 2008;118:251–5. 5. Shah VA, Kardon RH, Lee AG, et al. Long-term follow-up of idiopathic intracranial hypertension: the Iowa experience. Neurology 2008;70:634–40. 6. Kim AW, Trobe JD. Syndrome simulating pseudotumor cerebri caused by partial transverse venous sinus obstruction in metastatic prostate cancer. Am J Ophthalmol 2000;129:254–6. 7. Ganesan D, Higgins JN, Harrower T, et al. Stent placement for management of a small parasagittal meningioma. Technical note. J Neurosurg 2008;108:377–81. 8. Damak M, Crassard I, Wolff V, et al. Isolated lateral sinus thrombosis: a series of 62 patients. Stroke 2009;40:476–81. 9. Sindou MP, Alvernia JE. Results of attempted radical tumor removal and venous repair in 100 consecutive meningiomas involving the major dural sinuses. J Neurosurg 2006;105:514–25. 10. DiMeco F, Li KW, Casali C, et al. Meningiomas invading the superior sagittal sinus: surgical experience in 108 cases. Neurosurgery 2008;62:1124–35. 11. Kondziolka D, Flickinger JC, Perez B. Judicious resection and/or radiosurgery for parasagittal meningiomas: outcomes from a multicenter review. Gamma Knife Meningioma Study Group. Neurosurgery 1998;43:405–13. 12. Higgins JN, Burnet NG, Schwindack CF, et al. Severe brain edema caused by a meningioma obstructing cerebral venous outflow and treated with venous sinus stenting. Case report. J Neurosurg 2008;108:372–6. 13. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003;60: 1418–24. 14. Stienen A, Weinzierl M, Ludolph A, et al . Obstruction of cerebral venous sinus secondary to idiopathic intracranial hypertension. Eur J Neurol 2008;15: 1416–8.
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Case Reports / Journal of Clinical Neuroscience 17 (2010) 1592–1594
15. Corbett JJ, Digre K. Idiopathic intracranial hypertension: an answer to ‘‘the chicken or the egg?”. Neurology 2002;58:5–6. 16. Higgins JN, Cousins C, Owler BK, et al. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. Neurol Neurosurg Psychiatry 2003;74:1662–6.
17. Donnet A, Metellus P, Levrier O, et al. Endovascular treatment of idiopathic intracranial hypertension: clinical and radiologic outcome of 10 consecutive patients. Neurology 2008;70:641–7.
doi:10.1016/j.jocn.2010.03.039
Paraspinal malignant ossifying fibromyxoid tumor with spinal involvement Jaypal Reddy Sangala a, Paul Park a,⇑, Mila Blaivas b, Frank LaMarca a b
a
Department of Neurosurgery, University of Michigan Health System, 1500 E. Medical Center Drive, Room 3552 TC, Ann Arbor, Michigan 48109-5338, USA Department of Pathology, University of Michigan Health System, Ann Arbor, Michigan, USA
a r t i c l e
i n f o
Article history: Received 18 January 2010 Accepted 25 April 2010
Keywords: Ossifying tumors Soft tissue tumors Spine tumors Vertebral tumors
a b s t r a c t Ossifying fibromyxoid tumors (OFT), first described in 1989 by Enzinger et al., are rare lesions; malignant OFT (MOFT) are even rarer. We report a large recurrent paraspinal MOFT invading the spine and causing epidural compression in a 70-year-old male, despite prior debulking and radiotherapy. Paraspinal involvement of these tumors has been reported only twice before. We describe its imaging, pathology, and also review the pertinent literature. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Ossifying fibromyxoid tumors (OFT) are rare lesions arising from soft tissues. Approximately 220 such tumors have been reported.1 OFT were first described by Enzinger et al. in 1989.2 Malignant OFT (MOFT) are a subtype of these lesions that demonstrate malignant or atypical features.3 Although there has been one case report of an OFT infiltrating the spine, a MOFT invading the spine causing epidural compression has not been reported. We report such a lesion, and describe its imaging and pathological importance. The pertinent literature is also reviewed. 2. Case report A 73-year-old man presented to a regional hospital with lower back and hip pain radiating down his right anterior thigh in 2007. MRI revealed a large posterior paraspinal mass adjacent to the right L2 vertebra involving the right pedicle, lamina, and transverse process. The mass measured about 7 6 8 cm, extending superiorly to L1 and inferiorly to S1. There was an L2 intraspinal and extradural extension of the lesion via the right neural foramen, which was enlarged. This lesion was surgically debulked in March 2007. Histopathology of the tumor indicated a MOFT with an infiltrative growth pattern. Postoperative imaging revealed a decreased but still substantial amount of tumor. The patient received 70 Gy of radiation therapy. In October 2008, the patient presented to our institution with recurrent severe mechanical low back pain, right anterior thigh pain, and neurogenic claudication. MRI revealed an abnormally enhancing heterogeneous soft tissue mass centered upon the right-sided posterior elements of the L2 and L3 vertebra (Fig. 1).
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There was effacement of the central canal at the level of the tumor. Severe central canal stenosis was present at L3–L4 due to degenerative changes and tumor. Infiltrative changes were also identified within the right posterior paraspinal musculature. Substantial degenerative changes of the lumbar spine were identified including severe central canal narrowing at L4–L5. The tumor was a poorly circumscribed, rubbery mass located in the right paraspinal muscle with significant intracanalicular extension. In view of the diffuse nature of the tumor, complete surgical excision of the lesion was not attempted. The paraspinal tumor was debulked, and the nerve roots were decompressed. A T12–L4 instrumented posterior spinal fusion was also performed. At the 1-month follow-up, the patient had a significant reduction in both back pain and limb pain. At 4 months, the patient had a repeat MRI of the lumbosacral spine that showed recurrent/residual tumor without significant neural compression. The patient refused palliative chemotherapy in a clinical trial offered by the oncology service. One month later, the patient presented to the emergency room with significant weakness in both legs. Imaging revealed recurrent tumor with significant extension into the spinal canal. Various treatment options were discussed, but the patient refused further treatment. 2.1. Histopathology Microscopically, the tumor demonstrated features of OFT consisting of lobules of ovoid mesenchymal cells within a fibromyxoid to hyalinized matrix with areas of bone formation (Supplementary Fig. 1). Very brisk mitotic activity in areas of high cellularity was noted. In addition, there were focal areas of high-grade nuclear atypia. The specimen had an infiltrative growth pattern into the surrounding skeletal muscle and adipose tissue and involved the margins of excision fairly extensively. Based on the presence of high cellularity and high mitotic activity, the tumor was determined to be a MOFT, according to the criteria proposed by Folpe and Weiss.3