Focal Plaque of Demyelination Mimicking Cerebral Tumor in a Pediatric Patient

Abstract

Focal, demyelinating lesions of the cerebrum mimicking brain tumors are a rare phenomenon, and even rarer in a pediatric population. We document the case of a 14-year-old female whose clinical, radiological and operative findings were strongly suggestive of glioma. However, histopathology revealed a demyelinating process. This case is significant as the lesion could not be distinguished from a glioma at any time in the presentation. At 41 months follow-up, the patient remains stable without further evidence of demyelination in other areas of the brain. Such a case suggests a cautious note for the pediatrician when presented with a similar patient and illustrates the importance of consideration of a demyelinating lesion in the differential diagnosis of a mass lesion in a pediatric population.

Recurrent tumefactive demyelination in a child

“…1,2 Several reports in the literature describe the infrequent presentation of primary demyelinating disorders with large lesions mimicking intracranial neoplasms or abscesses, which have been termed tumefactive demyelination. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] There are reports of tumefactive demyelinating lesions in the pediatric age group. 5,[12][13][14][15][16][17][18][19][20][21] We present a child with two episodes of demyelinating disease each time with a tumefactive lesion.…”

Acquired Demyelinating Disorders of the CNS in Children

“…There are further reported cases of solitary, large plaque like lesions, which were histologically confirmed to be foci of demyelination (Kumar et al, 1998;Gutling and Landis, 1998). The aetiology is unclear; however an association with tuberculosis was described in 3 South African children (Pretorius et al, 1998).…”

“…These include deficits in short term memory, verbal processing skills and complex attention. Patient with early onset ADEM (<5year of age) were also more likely to have cognitive deficits and behaviour problems (Kumar et al, 1998). Follow-up MRI's showed complete or partial resolution of abnormalities in the majority of cases (Kesslering et al, 1990;Dale et al, 2000;Tenembaum et al 2007;Govender et al, 2010).…”

Concurrence of glioma and multiple sclerosis

“…An MS patient with an unusual appearing lesion poses a diagnostic dilemma because it could be a plaque or, a primary intracranial glioma. [1][2][3] The demyelinating MS plaques are usually found in the periventricular white matter, optic tracts, cerebellum, brainstem and spinal cord. On MRI or CT, these plaques may show contrast enhancement and usually lack mass effect.…”

“…4 Exceptionally, they enhance and show mass effect, raising the possibility of an intracranial neoplasm. 3,5 The size of MS plaques usually varies from 3 to 16 mm. Larger plaques are unusual, the largest reported thus far being 7.2 cm.…”