Solitary intracerebral juvenile xanthogranuloma. Case report and review of the literature.

Abstract:

:A case of an intracerebral juvenile xanthogranuloma (JXG) producing complex-partial seizures in a 13-year-old boy is presented. Although the child had no history of JXGs or a systemic histiocytic proliferative disorder, the histopathological and immunocytochemical studies of this lesion were identical to that of a cutaneous JXG. This represents the first description of an isolated intracerebral JXG. On extensive review of the literature, this case comprises only the second reported intracranial lesion and the fourth CNS lesion in a child without cutaneous manifestations of JXG. In contrast to cerebral xanthogranulomas of adulthood, all reported cases of isolated CNS JXG presented with focal symptoms referable to the neuroanatomic localization of the lesion. In this limited series, the magnetic resonance imaging appearance was predictable and consistent, although spinal and intracranial lesions differ with respect to their enhancement with paramagnetic contrast media. While complete surgical excision, if feasible, has been the most utilized form of therapy for symptomatic or progressive lesions, external beam irradiation and cytotoxic chemotherapy have been utilized for unresectable or recurrent disease. Given their rare occurrence and uncertain natural history, it remains unclear whether these solitary CNS lesions represent a distinct clinical entity with a potentially more aggressive clinical course or merely a variant of the systemic non-Langerhans cell histiocytoses.

journal_name

Pediatr Neurosurg

journal_title

Pediatric neurosurgery

authors

Schultz KD Jr,Petronio J,Narad C,Hunter SB

doi

10.1159/000121212

subject

Has Abstract

pub_date

1997-06-01 00:00:00

pages

315-21

issue

6

eissn

1016-2291

issn

1423-0305

journal_volume

26

pub_type

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