Solitary Intracerebral Juvenile Xanthogranuloma

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.

Spectroscopic increase in choline signal is a nonspecific marker for differentiation of infective/inflammatory from neoplastic lesions of the brain

“…However, all these lesions occur in association with extracutaneous juvenile xanthogranulomas or systemic or predominantly cerebral forms of histiocytosis X (24). There is only case of solitary intracerebral xanthogranuloma reported in the literature so far (24). The present case is the second case of solitary intracerebral xanthogranuloma and the first case to be reported with MR spectroscopic findings.…”

“…Xanthogranulomas of the CNS are commonly seen in a wide variety of degenerative, inflammatory, metabolic, and neoplastic conditions (24). These granulomas are composed of xanthoma cells, which are characterized by intracellular accumulation of lipid droplets, predominantly occurring within histiocytes or macrophages, but can also been seen within astrocytes and choroid plexus epithelium (24).…”

“…These granulomas are composed of xanthoma cells, which are characterized by intracellular accumulation of lipid droplets, predominantly occurring within histiocytes or macrophages, but can also been seen within astrocytes and choroid plexus epithelium (24). Intraparenchymal xanthogranulomas may occur as single or multiple and circumscribed or diffuse lesions.…”

“…The hypothalamus is particularly affected (25,26). However, all these lesions occur in association with extracutaneous juvenile xanthogranulomas or systemic or predominantly cerebral forms of histiocytosis X (24). There is only case of solitary intracerebral xanthogranuloma reported in the literature so far (24).…”

Primary Cerebral Angiitis Containing Marked Xanthoma Cells With Massive Intraparenchymal Involvement-Case Report-

“…5,7) Some xanthogranulomas originate from the intraventricle choroid plexus or the subdural space, but intraparenchymal xanthogranulomas are extremely rare. 3,6,12,14) In addition, most xanthogranulomas are space-occupying lesions with fibrous capsules. Only one case of a small intraparenchymal mass lesion was not sharply demarcated from the surrounding parenchyma.…”