Fibrous xanthomas and xanthosarcomas of the meninges and the brain

Kepes, John J.; Kepes, Magda; Slowik, F.

doi:10.1007/bf00687875

Cited by 98 publications

(27 citation statements)

References 9 publications

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“…Pleomorphic xanthoastrocytoma (PXA) was originally reported as fibrous xanthoma of the meninges and regarded as a mesenchymal tumour (Kepes et al, 1973;McLendon et al, 2006). However, the expression of GFAP in PXA subsequently modified this opinion and in 1979, Kepes (1993) established PXA as originating from glial cells.…”

Section: Pleomorphic Xanthoastrocytomamentioning

confidence: 98%

Tumours of the Central Nervous System

Liberski

2013

The Ultrastructure of Human Tumours

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Section: Pleomorphic Xanthoastrocytomamentioning

confidence: 98%

Tumours of the Central Nervous System

Liberski

2013

The Ultrastructure of Human Tumours

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“…10,16) PXA predominantly occurs in patients in the first decade of life as a superficially located, well-circumscribed, cystic or solid enhanced mass, and tends to affect the adjacent meninges with cerebrospinal fluid (CSF) seeding. 6,8,9,13,14,19,21,22) Inner table remodeling of the skull was identified on neuroimaging in 12.5% of patients with PXAs, 6) and meningeal invasion was identified microscopically in 92% of patients. 9) PXA may rarely mimic pituitary adenoma and meningioma.…”

Section: Introductionmentioning

confidence: 99%

Anaplastic Pleomorphic Xanthoastrocytoma With a Component of Anaplastic Astrocytoma Presenting as Skull Base Tumor Followed by Downward Extracranial Extension -Case Report-

Tsutsumi

Abe

Yasumoto

et al. 2010

Neurol. Med. Chir.(Tokyo)

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A 16-year-old female, with unremarkable medical and family history, presented with a huge duralbased mass in the right frontotemporal fossae, manifesting as headache. The patient underwent subtotal tumor resection. Intraoperative findings revealed focal erosion in the temporal fossa dura mater and skull adjacent to the lesion. Most of the tumor was located extraaxially, but a part of the tumor had invaded the temporal lobe, and had tightly adhered to the middle cerebral artery and its perforating vessels. Histological examination revealed cellular pleomorphism with mitotic activity, focal necrosis, but lacking endothelial proliferation, consistent with anaplastic pleomorphic xanthoastrocytoma (PXA) with component of anaplastic astrocytoma. Postoperatively, the patient underwent local irradiation and temozolomide administration, but the tumor relapsed 13 months later. Second tumor resection was performed followed by gamma knife radiosurgery, but the residual tumor progressively grew, extending into the contralateral hemisphere, and formed an enormous mass in the left frontal lobe at 17 months. Magnetic resonance imaging performed at 18 months revealed extracranial infiltration of the frontal tumor, through the cribriform plate, with enormous extension into the paranasal sinuses, nasal cavity, and orbit during the next month. The patient died at 20 months after the initial surgery. PXA with anaplastic appearance may have a component of anaplastic astrocytoma with more aggressive behavior.

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“…Only two cases of intracranial xantho matous tumors have been reported (1), and no familial occurrence is known. In common xanthomatous disease such as familial hypercholesterolemia (2), generalized xanthomas of tendons as well as other tissues with lipid deposits in the choroid plexus and Virchow-Robin spaces have been described (3).…”

Section: Introductionmentioning

confidence: 99%

Familial Spinal Xanthomatosis with Sitosterolemia.

et al. 1992

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A family with multiple spinal xanthomas and sitosterolemia is described. A 48-year-old woman presented with paraplegia due to multiple intradural extramedullary tumors. The patient also showed marked tendon xanthomas and analysis of sterol composition in both plasma and the xanthoma established the diagnosis of the rare inherited metabolic disease, sitosterolemia and xanthomatosis. Two other siblings in the family presented with marked tendon xanthomas and coronary atherosclerosis, but did not show any neurological signs or symptoms. Magnetic resonance imaging (MRI) study revealed multiple intradural extramedullary tumors in spinal canals of the proband and her sister, but not in the other affected sibling (brother). This is the first report of familial occurrence of multiple extramedullary spinal tumors due to the inherited metabolic abnormality.

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Fibrous xanthomas and xanthosarcomas of the meninges and the brain

Cited by 98 publications

References 9 publications

Tumours of the Central Nervous System

Tumours of the Central Nervous System

Anaplastic Pleomorphic Xanthoastrocytoma With a Component of Anaplastic Astrocytoma Presenting as Skull Base Tumor Followed by Downward Extracranial Extension -Case Report-

Familial Spinal Xanthomatosis with Sitosterolemia.

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