Acta Neurol Belg DOI 10.1007/s13760-015-0494-0
NEURO-IMAGES
Isolated meningeal inflammatory myofibroblastic tumor: an enigmatic tumor ¨ mer Aydın2 • Ayse Sayar3 • Selc¸uk Yılmazlar4 Ramazan Buyukkaya1 • O 2 Bahattin Hakyemez
•
Received: 18 March 2015 / Accepted: 25 May 2015 Ó Belgian Neurological Society 2015
Keywords Inflammatory myofibroblastic tumor Headache Magnetic resonance imaging
Introduction A 41-year-old woman was admitted to our institution with a slowly progressing, severe headache for 2 years. She had no neurological deficits on physical examination, and her laboratory findings were normal. Magnetic resonance imaging (MRI) showed dural thickening, a dural-based mass in the right temporal lobe, and minimal herniation in the occipitoparietal lobe due to the edema surrounding the mass. The dural-based mass had spiculated margins on contrast-enhanced T1-weighted images. Dural thickening, contrast enhancement, and leptomeningeal contrast enhancement were seen. In addition, the contrast-enhanced T1-weighted images showed dural venous sinus thrombosis (Fig. 1). To evaluate venous sinus narrowing or occlusion, digital subtraction angiography (DSA) was performed with carotid artery injection. No contrast filling was observed in ¨ mer Aydın & O [email protected] Ramazan Buyukkaya [email protected] 1
Department of Radiology, School of Medicine, Duzce University, Duzce, Turkey
2
Department of Radiology, School of Medicine, Uludag University, Bursa, Turkey
3
Department of Pathology, School of Medicine, Uludag University, Bursa, Turkey
4
Department of Neurosurgery, School of Medicine, Uludag University, Bursa, Turkey
the superior sagittal sinus, transverse sinus, or deep venous sinuses due to venous thrombosis. For determination of a definitive diagnosis, an open biopsy of the right temporal lobe was performed (Fig. 1f). The pathological examination indicated intense inflammatory cells (Fig. 2). Considering the pathological, radiological, and clinical findings, a meningeal inflammatory myofibroblastic tumor (IMT) was diagnosed. The patient was treated with corticosteroids, and her symptoms decreased during follow-up.
Discussion An IMT is a rare neoplasm that includes myofibroblastic spindle cells and inflammatory cells. Central nervous system (CNS) involvement is extremely rare, and relatively few cases have been reported [1–3]. A CNS–IMT can have a parenchymal [3], meningeal, or intraventricular location, and can settle in the sphenoid sinus or spinal area [1]. The most common sites are dural/meningeal (60 %) and parenchymal (12 %) [4]. In this case, diffuse dural/meningeal and accompanying parenchymal involvement was present. In most previous reports, other soft-tissue IMTs accompanied CNS involvement, while only CNS involvement was present in this case [5]. The most common symptoms of meningeal IMT are headache, visual disturbances, and seizures [1]. Our patient had a headache only. Chronic inflammation is seen in the dura in diffuse meningeal IMT. Venous sinus thrombosis may occur, due to the close anatomical localization of the dural venous sinuses with the dura, as seen in our case. In this case, MRI of the meningeal intracranial IMT showed low-signal intensity on T2-weighted mages, isosignal intensity on T1-weighted images, and diffuse contrast enhancement and dural thickening after contrast
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Acta Neurol Belg
Fig. 1 A 41-year-old woman with an inflammatory myofibroblastic tumor. a, b T2-weighted imaging shows hypointense dural thickening (dashed arrows), a mass (asterisk), and edema (arrow). c–e Contrastenhanced T1-weighted imaging shows diffuse thrombosed dural
sinuses, contrast-enhanced thickened dura (arrowhead), a dural-based mass, areas of edema (asterisk), and suspected leptomeningeal spread of the mass (curved arrow). f postoperative CT shows sinus thrombosis and dural thickening
Fig. 2 Diffuse lymphocyte and plasmacyte infiltration is seen (arrow). The tumor cells exhibited oval nuclei, pale chromatin and a large purple nucleolus
administration. On unenhanced computed tomography (CT) images, increased density of the dural sinus was seen. These findings are not specific for meningeal IMT; similar findings can be seen in fibroblastic en-plaque meningioma, lymphoma, and hypertrophic pachymeningitis. There is no standard treatment. In most cases, complete resection is considered as the first choice of treatment [5]. In summary, radiologists and physicians should be aware that inflammatory myofibroblastic tumors in the
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brain may be mixed with an invasive malignancy. Recent findings suggest that IMT expressing anaplastic lymphoma kinase (ALK), found in some fibrohistiocytic variants might be related to a more aggressive course [6]. In these patients, the radiological and clinical findings are usually incompatible. Knowledge of the clinical and radiological features is extremely important for an early diagnosis and initiation of appropriate treatment for inflammatory myofibroblastic tumors with meningeal involvement.
Acta Neurol Belg Conflict of interest The authors declare that there is no actual or potential conflict of interest in relation to this article. 3. Ethical standard This article does not contain any studies with human participants performed by any of the authors. 4. Informed consent Informed consent was obtained from all individual participants included in the study. 5.
References 1. Kim JH, Chang KH, Na DG, Park SH, Kim E, Han DH, Kwon HM, Sohn CH, Yim YJ (2009) Imaging features of meningeal inflammatory myofibroblastic tumor. AJNR Am J Neuroradiol 30(6):1261–1267 2. Swain RS, Tihan T, Horvai AE, Di Vizio D, Loda M, Burger PC, Scheithauer BW, Kim GE (2008) Inflammatory myofibroblastic
6.
tumor of the central nervous system and its relationship to inflammatory pseudotumor. Hum Pathol 39(3):410–419 Lin YJ, Yang TM, Lin JW, Song MZ, Lee TC (2009) Posterior fossa intracranial inflammatory pseudotumor: a case report and literature review. Surg Neurol 72(6):712–716 Hausler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B (2003) Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. Hum Pathol 34(3):253–262 Carswell C, Chataway J (2012) The successful long-term management of an intracranial inflammatory myofibroblastic tumor with corticosteroids. Clin Neurol Neurosurg 114(1):77–79 Denis DJ, Elayoubi K, Weil AG, Berthelet F, Bojanowski MW (2013) Inflammatory myofibroblastic tumors of the central nervous system that express anaplastic lymphoma kinase have a high recurrence rate. Surg Neurol Int. 4:70
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NEURO-IMAGES
Isolated meningeal inflammatory myofibroblastic tumor: an enigmatic tumor ¨ mer Aydın2 • Ayse Sayar3 • Selc¸uk Yılmazlar4 Ramazan Buyukkaya1 • O 2 Bahattin Hakyemez
•
Received: 18 March 2015 / Accepted: 25 May 2015 Ó Belgian Neurological Society 2015
Keywords Inflammatory myofibroblastic tumor Headache Magnetic resonance imaging
Introduction A 41-year-old woman was admitted to our institution with a slowly progressing, severe headache for 2 years. She had no neurological deficits on physical examination, and her laboratory findings were normal. Magnetic resonance imaging (MRI) showed dural thickening, a dural-based mass in the right temporal lobe, and minimal herniation in the occipitoparietal lobe due to the edema surrounding the mass. The dural-based mass had spiculated margins on contrast-enhanced T1-weighted images. Dural thickening, contrast enhancement, and leptomeningeal contrast enhancement were seen. In addition, the contrast-enhanced T1-weighted images showed dural venous sinus thrombosis (Fig. 1). To evaluate venous sinus narrowing or occlusion, digital subtraction angiography (DSA) was performed with carotid artery injection. No contrast filling was observed in ¨ mer Aydın & O [email protected] Ramazan Buyukkaya [email protected] 1
Department of Radiology, School of Medicine, Duzce University, Duzce, Turkey
2
Department of Radiology, School of Medicine, Uludag University, Bursa, Turkey
3
Department of Pathology, School of Medicine, Uludag University, Bursa, Turkey
4
Department of Neurosurgery, School of Medicine, Uludag University, Bursa, Turkey
the superior sagittal sinus, transverse sinus, or deep venous sinuses due to venous thrombosis. For determination of a definitive diagnosis, an open biopsy of the right temporal lobe was performed (Fig. 1f). The pathological examination indicated intense inflammatory cells (Fig. 2). Considering the pathological, radiological, and clinical findings, a meningeal inflammatory myofibroblastic tumor (IMT) was diagnosed. The patient was treated with corticosteroids, and her symptoms decreased during follow-up.
Discussion An IMT is a rare neoplasm that includes myofibroblastic spindle cells and inflammatory cells. Central nervous system (CNS) involvement is extremely rare, and relatively few cases have been reported [1–3]. A CNS–IMT can have a parenchymal [3], meningeal, or intraventricular location, and can settle in the sphenoid sinus or spinal area [1]. The most common sites are dural/meningeal (60 %) and parenchymal (12 %) [4]. In this case, diffuse dural/meningeal and accompanying parenchymal involvement was present. In most previous reports, other soft-tissue IMTs accompanied CNS involvement, while only CNS involvement was present in this case [5]. The most common symptoms of meningeal IMT are headache, visual disturbances, and seizures [1]. Our patient had a headache only. Chronic inflammation is seen in the dura in diffuse meningeal IMT. Venous sinus thrombosis may occur, due to the close anatomical localization of the dural venous sinuses with the dura, as seen in our case. In this case, MRI of the meningeal intracranial IMT showed low-signal intensity on T2-weighted mages, isosignal intensity on T1-weighted images, and diffuse contrast enhancement and dural thickening after contrast
123
Acta Neurol Belg
Fig. 1 A 41-year-old woman with an inflammatory myofibroblastic tumor. a, b T2-weighted imaging shows hypointense dural thickening (dashed arrows), a mass (asterisk), and edema (arrow). c–e Contrastenhanced T1-weighted imaging shows diffuse thrombosed dural
sinuses, contrast-enhanced thickened dura (arrowhead), a dural-based mass, areas of edema (asterisk), and suspected leptomeningeal spread of the mass (curved arrow). f postoperative CT shows sinus thrombosis and dural thickening
Fig. 2 Diffuse lymphocyte and plasmacyte infiltration is seen (arrow). The tumor cells exhibited oval nuclei, pale chromatin and a large purple nucleolus
administration. On unenhanced computed tomography (CT) images, increased density of the dural sinus was seen. These findings are not specific for meningeal IMT; similar findings can be seen in fibroblastic en-plaque meningioma, lymphoma, and hypertrophic pachymeningitis. There is no standard treatment. In most cases, complete resection is considered as the first choice of treatment [5]. In summary, radiologists and physicians should be aware that inflammatory myofibroblastic tumors in the
123
brain may be mixed with an invasive malignancy. Recent findings suggest that IMT expressing anaplastic lymphoma kinase (ALK), found in some fibrohistiocytic variants might be related to a more aggressive course [6]. In these patients, the radiological and clinical findings are usually incompatible. Knowledge of the clinical and radiological features is extremely important for an early diagnosis and initiation of appropriate treatment for inflammatory myofibroblastic tumors with meningeal involvement.
Acta Neurol Belg Conflict of interest The authors declare that there is no actual or potential conflict of interest in relation to this article. 3. Ethical standard This article does not contain any studies with human participants performed by any of the authors. 4. Informed consent Informed consent was obtained from all individual participants included in the study. 5.
References 1. Kim JH, Chang KH, Na DG, Park SH, Kim E, Han DH, Kwon HM, Sohn CH, Yim YJ (2009) Imaging features of meningeal inflammatory myofibroblastic tumor. AJNR Am J Neuroradiol 30(6):1261–1267 2. Swain RS, Tihan T, Horvai AE, Di Vizio D, Loda M, Burger PC, Scheithauer BW, Kim GE (2008) Inflammatory myofibroblastic
6.
tumor of the central nervous system and its relationship to inflammatory pseudotumor. Hum Pathol 39(3):410–419 Lin YJ, Yang TM, Lin JW, Song MZ, Lee TC (2009) Posterior fossa intracranial inflammatory pseudotumor: a case report and literature review. Surg Neurol 72(6):712–716 Hausler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B (2003) Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. Hum Pathol 34(3):253–262 Carswell C, Chataway J (2012) The successful long-term management of an intracranial inflammatory myofibroblastic tumor with corticosteroids. Clin Neurol Neurosurg 114(1):77–79 Denis DJ, Elayoubi K, Weil AG, Berthelet F, Bojanowski MW (2013) Inflammatory myofibroblastic tumors of the central nervous system that express anaplastic lymphoma kinase have a high recurrence rate. Surg Neurol Int. 4:70
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