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7 August 2013 - Case of the Week #282

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Case of the Week #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.

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

Xray image:



Micro images:

               

What is your diagnosis?































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.(1). The first reported case of xanthogranuloma of the choroid plexus was reported by Blummer in 1900, under the diagnosis of “Cholestomatous endothelioma”(2). Most xanthogranulomas of the choroid plexus arise in the lateral ventricular choroid plexus. (3) They are usually asymptomatic incidental findings, found in 2-7% of postmortem examinations (2). The xanthomatous reaction usually involves the stroma of the choroid plexus, and to a lesser extent, the epithelium (4). Like other xanthogranulomas, these choroid plexus xanthogranulomas are composed of xanthoma cells, cholesterol clefts, giant cells, hemosiderin, fibrosis, and occasional calcium deposits (3,4).

References:
1. Burger PC, Scheithauer BW. AFIP Atlas of Tumor Pathology, Series 4, Tumors of the Central Nervous System. Armed Forces Institute of Pathology, Washington DC, 2007. pp 537-538

2. Pear BL. Xanthogranuloma of the choroid plexus, AJR Am J Roentgenol 1984;143:401

3. Shetty J, Devadiga KV, Chandrika, Pai M. Unusual presentation of xanthogranuloma of the choroid plexus, J Neurosci Rural Pract 2010;1:97

4. Shuangshoti S, Phonprasert C, Suwanwela N, and Netsky MG. Combined neuroepithelial (colloid) cyst and xanthogranuloma (xanthoma) in the third ventricle, Neurology 1975;25:547

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