Tumefactive demyelinating lesions: nine cases and a review of the literature

Xia, Lei; Lin, Song; Wang, Zhongcheng; Li, Shaowu; Xu, Li; Wu, Jing; Hao, Shuyu; Gao, Chuan-chuan

doi:10.1007/s10143-009-0185-5

Cited by 49 publications

(45 citation statements)

References 36 publications

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“…However, our case may indicate that patients with TD should have a close and careful clinical follow-up with regard to concomitant or developing autoimmune or neoplastic disorder. In line with other reports [8,9], all lesions of our patient responded to dexamethasone. In unresponsive cases plasma exchange can be effective [10].…”

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confidence: 94%

Recurrent tumefactive demyelination without evidence of multiple sclerosis or brain tumour

et al. 2010

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The differential diagnosis of single space-occupying lesions comprises multiple sclerosis (MS) in younger patients and primary or secondary brain tumour at any age. We describe a 70-year-old man with recurrent tumour-like intracerebral masses, which were diagnosed as tumefactive demyelination (TD). He presented with confusion, headache and hemineglect. Past medical history included coronary heart disease and atrial fibrillation. Magnetic resonance imaging (MRI) of the brain revealed a single right parieto-occipital lesion (Fig. 1a, d). Search for primary neoplasm (lymph nodes, blood cell analysis, computer tomography of the thorax and abdomen, and whole body deoxyglucose positron emission tomography) was negative. The cerebrospinal fluid including fluorescenceactivated cell sorter showed mild lymphocytic pleocytosis (27 cells/ll) and elevated protein (0.71 g/l), but no oligoclonal bands or tumour cells. Biopsy of the cerebral lesion showed foamy macrophages and focal perivascular lymphocytic cuffs, which are indicative of demyelination ( Fig. 1g-j). There was no evidence of a neoplasm. Following biopsy the patient rapidly improved to a course of oral dexamethasone starting with 12 mg per day. Six and 12 weeks later his MRI showed substantial regression of the lesion with residual non-space-occupying hyperintensity on T2-weighted MRI. Eight, 23 and 29 months later, the patient developed single symptomatic lesions again (representative images are shown in Fig. 1b, c, e and f). He again responded to oral dexamethasone, and clinical improvement was paralleled by a decrease of lesion volume and contrast enhancement on MRI. Repeated extensive search for a primary tumour was negative. Ten months after the initial presentation of TD the patient presented with adynamia, anaemia and leukopaenia. Bone marrow examination revealed myelodysplastic syndrome (MDS). Eleven months later the patient suffered from haematuria, which led to the diagnosis of renal cell carcinoma. He died 34 months after the first manifestation of TD due to metastatic renal cancer. Brain autopsy findings were similar to those of the biopsy. Again, there was no evidence of lymphoma or any other neoplastic process. Our final diagnosis was recurrent TD.Our patient developed four single tumour-like lesions at various sites of the brain over 3 years. There was no preceding viral infection or vaccination. From the initial appearance on MRI, central nervous system lymphoma was suggested. However, neither biopsy nor autopsy revealed tumour. Age, clinical course with recurrent single lesions and negative oligoclonal bands in our patient suggest that at least a subset of TD cases may represent a distinct entity rather than a manifestation of MS [1]. In our case the diagnosis of MS appears very unlikely. Based on the

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confidence: 94%

Recurrent tumefactive demyelination without evidence of multiple sclerosis or brain tumour

et al. 2010

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“…Despite several reports [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16], including some large series of tumefactive demyelinating disease [7,9,11,12], no consensus has been reached about this rare spectrum. Common MRI findings include single or multiple large white matter lesions with variable mass effect and edema, complete or open ring enhancement, T2W hypointense rim, and a perilesional restriction on DWI [9][10][11][12][13][14][15][16]. However the prevalence of these radiological features is highly variable, and it is still controversial whether tumefactive CNS IDD represent a variant of MS or a unique form of isolated demyelinating disease.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with TDLs, often presenting with brain tumor mimicking clinical symptoms and space occupying lesions, have been described in previous reports [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]. However, a variety of radiographic and clinical characteristics were reported in different studies; some emphasized the unifocal and clinically isolated features [7,8], while others highlighted the predominant multifocal lesions and a high trend of conversion into multiple sclerosis (MS) by follow-up [9,10].…”

Section: Introductionmentioning

confidence: 91%

Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: Follow-up study

Yao

Huang

Gui

et al. 2015

Journal of the Neurological Sciences

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“…Quick frozen sections are often misdiagnosed as astrocytoma because it is difficult to distinguish macrophages and swollen astrocytes. Therefore, Xia et al (19) suggested that intraoperative frozen section analysis was inconclusive in the diagnosis of intracranial demyelinating pseudotumor. Immunohistochemical techniques should be applied when the case is suspicious or uncertain (3).…”

Section: █ Discussionmentioning

confidence: 99%

Intracranial demyelinating pseudotumor: a case report and review of the literature

Ning

Zhao²,

Wang³

et al. 2014

Turkish Neurosurgery

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ABSTRACTpseudotumor in the clinic is relative low, delayed diagnosis or misdiagnosis may cause delays in treatment of these patients and subsequently may lead to physical and mental damage (14). The differential diagnosis of demyelinating pseudotumor and brain tumors, especially of low-grade astrocytomas, should arouse more attention to exclude the possibility of a demyelinating process. Despite this, only few reported cases of intracranial demyelinating pseudotumor are known in the literature. In this case report, we present the diagnosis and treatment of a demyelinating pseudotumor patient who was misdiagnosed with astrocytoma. █ CASE REPORTA female 40-year-old patient was hospitalized due to a progressive disability of the left limb that severely increased over one week. The patient did not have a recent history █ INTRODUCTION D emyelinating pseudotumor of the brain, also called tumor-like demyelinating lesions (TDLs) or inflammatory demyelinating pseudotumor (IDP), is a rare inflammatory demyelinating disease of the central nervous system (CNS) (3,4,7,8). The exact etiology of demyelinating pseudotumor is unclear, however it may associate with multiple sclerosis (MS) (5). Based on the clinical presentation and computed tomography (CT) and magnetic resonance imaging (MRI) findings, it is difficult to distinguish between brain tumors, abscesses and demyelinating pseudotumors (13). Unlike brain tumors, demyelinating pseudotumors respond well to steroid hormones (6). Therefore, a reliable distinction between brain tumors and intracranial demyelinating pseudotumors is important for a targeted therapy. Although the incidence of demyelinating Demyelinating pseudotumor is a rare inflammatory demyelinating disease of the central nervous system (CNS) that has a similar clinical presentation and computed tomography (CT) and magnetic resonance imaging (MRI) imaging findings as brain tumors or abscesses. Unlike brain tumors, demyelinating pseudotumors respond well to steroid hormones. There are only a few reported cases of intracranial demyelinating pseudotumors in the literature. In this case report, we present the diagnosis and treatment of demyelinating pseudotumor in a patient whose condition was initially misdiagnosed as an astrocytoma. Based on the literature and our case, we formulated an outline for the differential diagnosis of demyelinating pseudotumor and astrocytoma. A timely and correct diagnosis of demyelinating pseudotumor would avoid blind surgery, radiotherapy and chemotherapy, which are used to treat brain tumors.

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Tumefactive demyelinating lesions: nine cases and a review of the literature

Cited by 49 publications

References 36 publications

Recurrent tumefactive demyelination without evidence of multiple sclerosis or brain tumour

Recurrent tumefactive demyelination without evidence of multiple sclerosis or brain tumour

Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: Follow-up study

Intracranial demyelinating pseudotumor: a case report and review of the literature

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