Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 7  |  Issue : 6  |  Page : 806-808  

Tumefactive demyelination mimicking neoplasm


Department of Radio-Diagnosis, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D Y Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India

Date of Web Publication18-Nov-2014

Correspondence Address:
Digish Shah
19, Anupam Society, Behind Pizza Inn, Jetalpur Road, Vadodara - 390 007, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.144895

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  Abstract 

Tumefactive demyelination is a large solitary demyelinating lesion, which mimics intracranial brain tumor. This information is particularly valuable to both physicians and surgeons who should be aware that inflammatory demyelinating diseases when present as a mass lesion is difficult to distinguish from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease. Distinguishing tumefactive lesions is necessary from other causes of intracranial space-occupying lesions to avoid unnecessary and expensive surgical intervention. We report a case of demyelinating disease with tumor-like presentation.

Keywords: Cerebral neoplasm, magnetic resonance imaging, tumefactive demyelinating lesion


How to cite this article:
Shah D, Patil A, Patil P, Bhatnagar S, Kulkarni V. Tumefactive demyelination mimicking neoplasm. Med J DY Patil Univ 2014;7:806-8

How to cite this URL:
Shah D, Patil A, Patil P, Bhatnagar S, Kulkarni V. Tumefactive demyelination mimicking neoplasm. Med J DY Patil Univ [serial online] 2014 [cited 2024 Mar 29];7:806-8. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2014/7/6/806/144895


  Introduction Top


Demyelinating diseases can present as a mass lesion, which is indistinguishable from a cerebral neoplasm, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease. This possesses a challenge to the clinician for correctly diagnosing the mass lesion. [1] The tumefactive demyelinating lesions are solitary large lesions (greater than 2 cm) with mass-like features and post-gadolinium magnetic resonance imaging (MRI) generally showing an incomplete ring enhancement. [2],[3] They occur more frequently in women in the second and third decades of life, with an average age of onset of at least 37 years. [4] The incidence of tumefactive demyelinating lesions has been estimated at 0.3 cases per 1,00,000/year. [5] Usual clinical symptoms include headache, cognitive changes, mental confusion, aphasia and apraxia. [3] Sometimes, it is difficult to differentiate large demyelinating lesions from brain abscess or brain tumor and biopsy is needed for final diagnosis. [6] Tumefactive demyelination usually presents with acute symptoms and monophasic course of illness and most of them show complete remission with corticosteroids. [7]


  Case Report Top


This was a case report of a 50-year-old female patient presented with altered behavior (social withdrawal) since last 3 months. She was confused and did not interact much. She was not taking any psychotic drugs. Neurological examination was unremarkable. Her blood pressure was 110/76 mm Hg and blood sugar levels were also normal. Laboratory investigation: Hemoglobin concentration: 12 g/dL, total leukocyte count: 7000/cu.mm with 65% neutrophils Biochemical investigations: Random blood sugar 4.7 mmol/L, serum creatinine level: 70 μmol/L, sodium 142 mmol/L, potassium 3.9 mmol/L and calcium 2.2 mmol/L. Her laboratory and biochemical examination were normal human immunodeficiency virus (HIV) serology was negative. Electroencephalogram was normal. She was advised MRI for further evaluation.

MRI revealed an ill-defined T1 hypointense, T2 hyperintense mass with an incomplete rim of enhancement in the post-contrast scans no other similar mass lesion or focal signal change was visualized [Figure 1] and [Figure 2]. There was no encroachment into the grey matter. Given the large size of the mass, it was observed that the accompanying edema was disproportionately mild. Despite its mass-like appearance, the rim enhancement and relative little perilesional edema were thought to favor a demyelinating lesion over neoplasm. Progressive multifocal leucoencephalopathy was unlikely as the patient was not immunocompromised. HIV serology was also negative. Posterior reversible encephalopathy syndrome was excluded, as there was no history of hypertension.
Figure 1: T2WI showed diffuse symmetrical bilateral hyperintensities around the hippocampal area, peri-ventricular areas around the
occipital horn of the lateral ventricle and parieto-occipital area


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Figure 2: (a) T1WI (b) fl uid attenuated inversion recovery (FLAIR) (c) diffusion-weighted imaging (d) apparent diffusion coeffi cient (ADC) (e) gradient-echo (GRE) (f) T1 post contrast. The lesion appeared hypointense on T1WI, hyperintense on FLAIR and showed peripheral restriction on diffusion and appeared bright on ADC. There was no blooming on GRE and showed open ring like enhancement on contrast study with enhancing ring facing the cortex

Click here to view


The patient was put on corticosteroids therapy and patient fully recovered in 2 months. Follow-up MRI scan was normal confirming our diagnosis of monophasic tumefactive demyelinating lesion [Figure 3].
Figure 3: After treatment with steroids, fl uid attenuated inversion recovery images at the mid brain (a), basal ganglia (b) and centrum semiovale (c) did not reval any abnormality

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  Discussion Top


Tumefactive demyelinating lesions are frequently mistakenly diagnosed as brain tumors. On MRI, tumefactive demyelination appear as an large ill-defined mass having variable amount of cerebral edema causing mass effect and showing variable amount of enhancement on post contrast study. In addition, lesions may show central necrosis, cystic degeneration and variable amount of grey matter involvement. [7],[8]

In a review of 31 cases, Kepes [9] proposed that tumefactive demyelinating lesions represent an intermediate lesion between those typically seen with multiple sclerosis and acute disseminated encephalomyelitis. Pathologically, these lesions are indistinguishable from typical multiple sclerosis plaques and are characterized by infiltrating foamy macrophages intermingled between reactive astrocytes. [10] As a result of myelin breakdown considerable amount of lipid accumulates within the plaques. Recent research has revealed injury to the axons within the multiple sclerosis plaques.

Distinguishing the tumefactive demyelinating lesions from neoplasm is important, since a misdiagnosis can lead to grave consequences. Some MR features which favor the diagnosis of tumefactive demyelinating lesions to brain tumor include incomplete rim enhancement, mixed T2-weighted iso-and hyperintensity of enhanced regions, absence of a mass effect and absence of cortical involvement. [11] The enhancement patterns include ring (single and multiple), heterogeneous, diffuse, punctate and concentric. Ring enhancement is the most frequent. Several studies have suggested that the pattern of open ring enhancement pattern is typically associated with demyelination. In a recent series, authors demonstrated complete ring enhancement (closed ring) pattern in majority of their patients with tumefactive demyelinating lesions. [3]

Demonstration of elevation of the glutamate/glutamine peaks in MRI Spectroscopy is beneficial in the differential diagnosis of tumefactive demyelinating lesions because elevation of these peaks is not seen in neoplastic diseases of the brain [12] Cha et al. in their study have reported the mean relative cerebral blood volume within tumefactive demyelinating lesions was lower than that within the contralateral normal-appearing white matter and substantially less than that found in high-grade glioma and lymphomas. [13]

Thus, recognition of the imaging features of a tumefactive demyelinating lesion would potentially save the patient a biopsy. Although the morbidity rate of a stereotactic biopsy is relatively low at 3.5% and mortality rate of 0.7%. [14]


  Conclusion Top


Monophasic tumefactive demyelination can be misdiagnosed as a intracranial mass lesion. This case report illustrates how the radiological features can aid the diagnosis and save the patient from brain biopsy or harmful aggressive treatment.

 
  References Top

1.
Hayashi T, Kumabe T, Jokura H, Fujihara K, Shiga Y, Watanabe M, et al. Inflammatory demyelinating disease mimicking malignant glioma. J Nucl Med 2003;44:565-9.  Back to cited text no. 1
    
2.
Dagher AP, Smirniotopoulos J. Tumefactive demyelinating lesions. Neuroradiology 1996;38:560-5.  Back to cited text no. 2
    
3.
Lucchinetti CF, Gavrilova RH, Metz I, Parisi JE, Scheithauer BW, Weigand S, et al. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain 2008;131:1759-75.  Back to cited text no. 3
    
4.
Comi G. Multiple sclerosis: Pseudotumoral forms. Neurol Sci 2004;25 Suppl 4:S374-9.  Back to cited text no. 4
[PUBMED]    
5.
Ragel BT, Fassett DR, Baringer JR, Browd SR, Dailey AT. Decompressive hemicraniectomy for tumefactive demyelination with transtentorial herniation: Observation. Surg Neurol 2006;65:582-3.  Back to cited text no. 5
    
6.
Jain D, Rajesh LS, Vasishta RK, Radotra BD, Banerjee AK. Demyelinating disease simulating brain tumours: A histopathologic assessment of seven cases. Indian J Med Sci 2006;60:47-52.  Back to cited text no. 6
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7.
Fallah A, Banglawala S, Ebrahim S, Paulseth JE, Jha NK. Case Series: Tumefactive demyelinating lesions: A diagnostic challenge. Can J Surg 2010;53:69-70.  Back to cited text no. 7
    
8.
Saindane AM, Cha S, Law M, Xue X, Knopp EA, Zagzag D. Proton MR spectroscopy of tumefactive demyelinating lesions. AJNR Am J Neuroradiol 2002;23:1378-86.  Back to cited text no. 8
    
9.
Kepes JJ. Large focal tumor-like demyelinating lesions of the brain: Intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis? A study of 31 patients. Ann Neurol 1993;33:18-27.  Back to cited text no. 9
    
10.
Paley RJ, Persing JA, Doctor A, Westwater JJ, Roberson JP, Edlich RF. Multiple sclerosis and brain tumor: A diagnostic challenge. J Emerg Med 1989;7:241-4.  Back to cited text no. 10
    
11.
Kim DS, Na DG, Kim KH, Kim JH, Kim E, Yun BL, et al. Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: Added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology 2009;251:467-75.  Back to cited text no. 11
    
12.
Cianfoni A, Niku S, Imbesi SG. Metabolite findings in tumefactive demyelinating lesions utilizing short echo time proton magnetic resonance spectroscopy. AJNR Am J Neuroradiol 2007;28:272-7.  Back to cited text no. 12
    
13.
Cha S, Pierce S, Knopp EA, Johnson G, Yang C, Ton A, et al. Dynamic contrast-enhanced T2*-weighted MR imaging of tumefactive demyelinating lesions. AJNR Am J Neuroradiol 2001;22:1109-16.  Back to cited text no. 13
    
14.
Ridderheim PA, Hariz MI, Bergenheim T. Stereotactic craniotomy for brain tumors and cavernous angiomas: Experience with 27 cases. 50 th Annual Meeting of the Scandinavian Neurosurgical Society; 11-14 th June 1998; Oulu, Finland.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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