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7 August 2013 - Case #282

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Thanks to Dr. Eman Abdelzaher, Alexandria University (Egypt), for contributing this case and the discussion.

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Case #282

Clinical history:
A 17 year old girl presented with headache. MRI showed a small enhancing choroid plexus lesion involving the occipital horn of the right lateral ventricle. On CT scan, the lesion was densely calcified, but no mass effect was noticed. At surgery, the lesion was attached to the choroid plexus.

Soft tissue specimen (2 x 1.5 cm) is received. Cut section showed multiple granular whitish foci. The entire specimen was processed.

Radiology images:


Microscopic images:




What is your diagnosis?

Click here for diagnosis and discussion:


Diagnosis: Xanthogranuloma of the choroid plexus

Discussion:
Microscopic examination revealed a xanthogranulomatous reaction. Cholesterol clefts, multinucleated foreign body giant cells, and scattered calcospherites were seen. The background is fibrous and shows a lymphoplasmacytic inflammatory infiltrate and hemosiderin deposits. Focal degeneration and hemorrhage were seen. Focally, aggregates of xanthoma cells were noted. Choroid plexus was noted in the lesional vicinity. Psammoma bodies were also seen.

Intracranial xanthogranulomas are somewhat rare, benign lesions and occur most commonly as choroid plexus xanthogranulomas. The exact etiology is uncertain. The first reported case of xanthogranuloma of the choroid plexus was reported by Blummer in 1900, under the diagnosis of cholestomatous endothelioma. Most xanthogranulomas of the choroid plexus arise in the lateral ventricular choroid plexus. They are usually asymptomatic incidental findings, found in 2 - 7% of postmortem examinations. The xanthomatous reaction usually involves the stroma of the choroid plexus, and to a lesser extent, the epithelium. Like other xanthogranulomas, these choroid plexus xanthogranulomas are composed of xanthoma cells, cholesterol clefts, giant cells, hemosiderin, fibrosis and occasional calcium deposits.

References: Burger: Tumors of the Central Nervous System, 1st Edition, 2007, AJR Am J Roentgenol 1984;143:401, J Neurosci Rural Pract 2010;1:97, Neurology 1975;25:547


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