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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 6  |  Page : 1333-1335

Primary spinal extradural inflammatory myofibroblastic tumor: A rare cause of paraparesis

1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication11-Nov-2016

Correspondence Address:
Kanwaljeet Garg
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.193804

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How to cite this article:
Mankotia DS, Garg K, Nambirajan A, Suri V, Tandon V, Kumar R, Suri A, Kale SS, Sharma BS. Primary spinal extradural inflammatory myofibroblastic tumor: A rare cause of paraparesis. Neurol India 2016;64:1333-5

How to cite this URL:
Mankotia DS, Garg K, Nambirajan A, Suri V, Tandon V, Kumar R, Suri A, Kale SS, Sharma BS. Primary spinal extradural inflammatory myofibroblastic tumor: A rare cause of paraparesis. Neurol India [serial online] 2016 [cited 2020 Jul 5];64:1333-5. Available from:


A 30-year-old male patient presented to our outpatient department with complaints of progressively worsening upper thoracic back pain and progressive difficulty in walking for the last 2 months. He also complained of tingling and numbness below the level of nipple. Neurological examination was suggestive of thoracic myelopathy with sensory level at T4. His hematological laboratory parameters were within normal limits.

Magnetic resonance imaging (MRI) of the spine [Figure 1] and [Figure 2] revealed an epidural mass lesion in the cervicodorsal spine extending from C7-D3 spinal level with a small extraforaminal extension causing compression and displacement of the spinal cord ventrally and to the right side. The lesion was hypointense on both T1-weighted (W) images and T2 W images and showed restriction of diffusion. The lesion demonstrated homogenous enhancement on intravenous contrast administration. There was no bony abnormality or erosion seen on computed tomography (CT) correlation. Screening MRI of the whole spine and brain did not demonstrate any other lesion. Based on these imaging findings, the possibility of en plaque meningioma, primary spinal lymphoma, and metastasis were considered.
Figure 1: Sagittal magnetic resonance imaging of the spine showing a C7-T1 epidural mass lesion with cord compression. Lesion is hypointense on both T1 (a) and T2 (b) and enhancing homogenously on contrast administration (c)

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Figure 2: Axial magnetic resonance imaging T1 (a) and contrast (b) showing epidural mass lesion with cord compression. No bony involvement could be seen on sagittal (c) and axial (d) CT at the D1 level

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After a detailed workup, the patient was taken up for emergent surgery in view of the progressive myelopathic symptoms. A C7-D4 laminoplasty with excision of the epidural mass was performed. Intraoperatively, a grayish white, moderately vascular, firm lesion was found which was completely extradural. The lesion was firmly adherent to the dura at several places. The cord was severely compressed and pushed toward the right side by the mass lesion. Complete excision of the spinal part could be achieved and cord was well decompressed with appreciable pulsations. There were no signs of bony destruction intraoperatively. Muscle power in lower limbs improved to 5/5 (Medical Research Council grading).

On histopathological examination, the lesion was composed of bland spindle cells arranged in a storiform pattern infiltrating into fibroadipose tissue with dense plasma cell rich infiltrate. Special stains did not reveal any organism. The spindle cells were immunopositive for smooth muscle actin and vimentin and negative for S100 and anaplastic lymphoma kinase (ALK). IgG4 immunostain did not show any appreciable increase in IgG4 positive plasma cells, and the features were suggestive of an inflammatory myofibroblastic tumor [Figure 3].
Figure 3: Sections show a spindle cell tumor (a, ×100) composed of plump-to-spindled fibroblasts in fascicular and storiform patterns (b, ×200) with a dense plasma cell infiltrate (c, ×400). Tumor cells were focally immunopositive for smooth muscle actin (d, ×400)

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Though inflammatory myofibroblastic tumor (IMT) can occur in any part of the body, central nervous system is involved very rarely.[1],[2] The location of IMT in the spine can be intradural extramedullary, intramedullary, or extradural. Spinal extradural IMT or inflammatory pseudo tumor is even rarer, with only seven such cases reported in the English literature so far [Table 1].[1],[3],[4] Many other terms such as pseudogranuloma, pseudosarcomatous myofibroblastic proliferation, and inflammatory myofibrohistiocytic proliferation have been used in the literature to describe these lesions. In 2002, the World Health Organization reclassified this subset of lesions as inflammatory myofibroblastic tumor. No specific age, sex, or location predilection was evident on literature review.[2]
Table 1: Previously reported cases of spinal epidural inflammatory myofibroblastic tumor in world literature

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The pathogenesis of IMT is uncertain and different hypothesis have been proposed including an immunological response to microorganisms, trauma, previous spine surgery, and virus.[1],[2],[5] Our patient did not have any previous history of tuberculosis, spinal trauma, spine surgery, or any chronic illness in the past. The diagnosis of IMT is made only after exclusion of other causes of chronic inflammation such as tuberculosis, fungus, and bacteria.

Spinal en plaque meningioma, lymphoma, metastasis, and myeloma deposits are close differentials and were considered in the current case preoperatively. Usually, these tumors are hypointense on T1 and T2 W images and show homogenous contrast enhancement due to the presence of fibrosis. Han et al., suggested that T2 hypointensity is due to the absence of free water and protons with an associated high cellular content.[6] Lacoste-Collin et al., suggested the association of ALK gene mutation with recurrence.[7] In spinal IMT, surgical excision is the management of choice to relieve cord compression and radiotherapy with steroids are reserved for residual lesions.[3],[4],[5],[8],[9],[10]

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  References Top

Roberts G, Farrell M, Allcutt D. Spinal inflammatory pseudotumours. Br J Neurosurg 2001;15:197-8.  Back to cited text no. 1
Jeon YK, Chang KH, Suh YL, Jung HW, Park SH. Inflammatory myofibroblastic tumor of the central nervous system: Clinicopathologic analysis of 10 cases. J Neuropathol Exp Neurol 2005;64:254-9.  Back to cited text no. 2
Roberts GA, Eldridge PR, Mackenzie JM. Case report: Inflammatory pseudotumour of the spine, with literature review. Br J Neurosurg 1997;11:570-2.  Back to cited text no. 3
Gilliard C, De Coene B, Lahdou JB, Boutsen Y, Noël H, Godfraind C. Cervical epidural pseudotumor and multifocal fibrosclerosis. Case report and review of the literature. J Neurosurg 2000;93 (1 Suppl):152-6.  Back to cited text no. 4
Sailler LJ, Porte L, Ollier SM, Astudillo LM, Couret BG, Catalaa I, et al. Giant cell arteritis and spinal cord compression: An overlap syndrome? Mayo Clin Proc 2006;81:89-91.  Back to cited text no. 5
Han MH, Chi JG, Kim MS, Chang KH, Kim KH, Yeon KM, et al. Fibrosing inflammatory pseudotumors involving the skull base: MR and CT manifestations with histopathologic comparison. AJNR Am J Neuroradiol 1996;17:515-21.  Back to cited text no. 6
Lacoste-Collin L, Roux FE, Gomez-Brouchet A, Despeyroux ML, Uro-Coste E, Coindre JM, et al. Inflammatory myofibroblastic tumor: A spinal case with aggressive clinical course and ALK overexpression. Case report. J Neurosurg 2003;98 (2 Suppl):218-21.  Back to cited text no. 7
Kato S, Murakami H, Demura S, Yoshioka K, Okamoto Y, Hayashi H, et al. Epidural inflammatory pseudotumor in the thoracic spine in a patient with polymyalgia rheumatica. Spine 2012;12:e1-4.  Back to cited text no. 8
Seol HJ, Kim SS, Kim JE, Lee SH, Won JY. Inflammatory pseudotumor in the epidural space of the thoracic spine: A case report and literature review of MR imaging findings. AJNR Am J Neuroradiol 2005;26:2667-70.  Back to cited text no. 9
Kanagaraju V, Rai D, Alluri RV, Prasanna C, Shyam Sundar V, Arvind Kumar SM, et al. An inflammatory pseudotumor in the thoracic epidural space presenting with progressive paraplegia: A histopathological diagnosis with clinical and radiological uncertainty. Case report with literature review. Eur Spine J. 2016;25(Suppl 1):75-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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