Tumefactive multiple sclerosis versus high-grade glioma: A diagnostic dilemma

Abstract

Background: Tumefactive demyelinating lesions (TDLs) share similar clinical features and MRI characteristics with high-grade glioma (HGG). This study develops an approach to navigating this diagnostic dilemma, with significant treatment implications as the management of both entities is drastically different. Methods: A retrospective analysis of 41 TDLs and 91 HGG with respect to demographics, presentation, and classical MRI characteristics was performed. A diagnostic pathway was then developed to help diagnose TDLs based on whole neuraxis MRI and cerebrospinal fluid (CSF) examination. Results: The diagnosis of TDL is more likely than HGG in younger females who present with subacute or chronic symptoms. MRI characteristics favoring TDL over HGG include smaller size, open rim enhancement, little or no associated edema or mass effect, and the presence of a T2 hypointense rim. MRI of the whole neuraxis for detection of other lesions typical of multiple sclerosis (MS), in combination with a lumbar puncture (LP) showing positive CSF-specific oligoclonal bands (OCB), was positive in 90% of the TDL cohort. Conclusion: The diagnostic pathway, proposed on the basis of specific clinicoradiological features, should be followed in patients with suspected TDL. If MRI demonstrates other lesions typical of MS and LP demonstrates positive CSF-specific OCBs, then patients should undergo a short course of IV steroids to look for clinical improvement. Patients who continue to deteriorate, do not demonstrate other lesions on MRI or where the LP is negative for CSF-specific OCB, should be considered for biopsy if safe to do so. This pathway will give the patients the best chance at neurological preservation.

Tumefactive Multiple Sclerosis: Challenges With Treatment Modalities

“…A lumbar puncture may reveal oligoclonal protein bands and myelin basic protein in up to 74% of patients. Brain biopsies are reserved for diagnostically challenging patients in whom conservative diagnosis and management fail [ 4 ].…”

Neuroimaging and clinicopathological differences between tumefactive demyelinating lesions and sentinel lesions of primary central nervous system lymphoma

“…In early stage, some patients may only present mental and cognitive impairment such as memory loss, unresponsiveness, apathy, which are ignored and inadvertently take MRI to find large intracranial lesions, even accompanied by peripheral edema and mass or/and enhanced lesions ( 17 ). Mass-occupying lesions are easily diagnosed as a tumor, such as PCNSL or high-grade glioma ( 9 , 14 , 18 , 19 ). During the phase of TDLs is progressing, symptoms may gradually increase or worsen, and reduced vision may also present.…”

Tumefactive multiple sclerosis

“…The symptoms originated by TDL can have an acute or subacute presentation (3) which depends on the location, size of the lesion and its mass effect (6,9). The clinical manifestations of TMS include headache, cognitive abnormalities, mental confusion, seizures, apraxia, aphasia (1,3,4), increased intracranial pressure, vomiting, behavioral disturbances, visual disturbances, optic neuritis and hemiparesis (3,15,16). TDL often causes memory dysfunction, Gerstmann's syndrome and in some patients, encephalopathies are observed (9).…”