종괴형성병변을 보인 2증례: 탈수초질환과 뇌종양

We studied 21 cases of pathologically confirmed tumefactive demyelinating lesions and reviewed the spectrum of tumefactive demyelinating lesions in the literature. Radiological features and clinical data were reviewed to characterize the lesions as consistent with a known demyelinating disease, most notably multiple sclerosis. Atypical clinical or radiological features (other than tumefaction) were noted. Most lesions were part of a clinical and/or radiological picture consistent with multiple sclerosis. No case strongly suggestive of variants or related diseases, such as Schilder's disease or Balo's concentric sclerosis, were found. There was one case suggestive of acute disseminated encephalomyelitis. Features which help distinguish the lesions from tumour are discussed.

Tumefactive demyelinating lesions.

Establishing the diagnosis of a brain tumour is not always a straightforward process. Many non-neoplastic neurological diseases can mimic brain neoplasms on neuroimaging or on histological examination, including multiple sclerosis, stroke, pyogenic abscess, toxoplasmosis, tuberculosis, cysticercosis, fungal infections, syphilis, sarcoidosis, Behcet disease, radiation necrosis, venous thrombosis, and others. Conversely, several types of brain neoplasms, such as glioblastomas, low-grade gliomas, CNS lymphomas, and brain metastases, can present in the absence of typical tumefactive lesions, posing significant diagnostic challenges. In this Review, we discuss the process of accurately establishing the diagnosis of brain tumours, focusing on pitfalls commonly encountered in clinical practice. We also discuss the rational use and limitations of new diagnostic techniques, such as diffusion-weighted MRI, perfusion-weighted MRI, magnetic resonance spectroscopy, single-photon emission tomography, and positron emission tomography, as well as new tools for histological examination, such as immunohistochemistry and molecular genetics analysis.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(06)70597-X.pdf

Pitfalls in the diagnosis of brain tumours

The authors describe a patient with an isolated, gadolinium-enhancing, biopsy-proven focus of tumefactive demyelination. There was marked clinical improvement with plasma exchange after failure of high-dose i.v. corticosteroids. The post-treatment clinical course correlated with decreasing enhancement and lesion size on MRI. This patient's rapid clinical and MRI response suggests that plasma exchange may be beneficial in this disorder, and could perhaps serve as a diagnostic tool to avoid the need for brain biopsy.

Treatment of steroid-unresponsive tumefactive demyelinating disease with plasma exchange

A retrospective analysis of 14 patients who presented with a progressively expanding mass lesion(s) shown at biopsy/autopsy to represent acute demyelination was carried out. The aims of this study were to determine the optimal neuropathological approach to diagnosis and to determine the clinical evolution of this condition. Subsequent investigations and clinical outcome studies confirmed MS in 10 cases. Two patients had received an incorrect neuropathologic diagnosis of astrocytoma resulting in cranial irradiation. Key histologic parameters in establishing a diagnosis of acute demyelination were a predominance of lipid filled macrophages, macrophage alignment along axons, and an absence of oligodendroglial inclusions. Axonal injury was present in all cases and a lymphocytic/plasma cell infiltrate was sparse in areas of demyelination. Neuroimaging revealed single lesions in 10 patients and multiple lesions in 4 patients. Two patients were lost to follow-up, 3 died within 18 months of diagnosis, 8 had a relapsing remitting clinical course, and 1 patient had a chronic progressive course.

In conclusion, a dense lymphocytic and plasma cell infiltrate is unusual in acute human demyelination. Although axonal injury is a frequent histologic finding in acute demyelination, it does not preclude a favorable clinical outcome.

Acute demyelination, neuropathological diagnosis, and clinical evolution.

BackgroundTumefactive demyelination (TD) is a relatively uncommon entity which mimics other focal intracranial lesions. Conventional radiological findings in tumefactive demyelination have been well described. However, DTI and MRS findings in TD have not been studied in detail.PurposeTo evaluate the usefulness of conventional magnetic resonance imaging (MRI), multivoxel 1H spectroscopy (MRS) and diffusion tensor imaging (DTI) in diagnosis and follow-up of TD of the brain.Material and MethodsClinical and imaging findings of 18 patients were reviewed. MR imaging data which included conventional imaging as well as MRS and DTI were reviewed. At TE 135ms MRS various metabolite ratios were calculated at different depths of the demyelinating lesions. At TE 30 ms, glutamate-glutamine (GLX-2.1–2.5 ppm) was compared in the lesion to the contralateral normal side. DTI data were available for 15 patients and Dav (mean diffusivity) and trace values were recorded from central and peripheral layers of the index lesion. ...

종괴형성병변을 보인 2증례: 탈수초질환과 뇌종양

References

Tumefactive demyelinating lesions.

Pitfalls in the diagnosis of brain tumours

Treatment of steroid-unresponsive tumefactive demyelinating disease with plasma exchange

Acute demyelination, neuropathological diagnosis, and clinical evolution.

Conventional and advanced magnetic resonance imaging in tumefactive demyelination