Contributed by Kent J. Donelan, MD, Brad B. Randall, MD and Paul E. Newby, PA-ASCP
DIAGNOSIS
Acute subarachnoid and intraventricular hemorrhage secondary to acute hemorrhage within a choroid plexus xanthogranuloma.
DISCUSSION
We report an unusual case of a 51-year-old man who died suddenly and was found to have an intraventricular and subarachnoid hemorrhage secondary to acute hemorrhage within a choroid plexus xanthogranuloma. The pattern of acute hemorrhage found within the left lateral ventricle, third and fourth ventricles, and subarachnoid space, along with the finding of both acute and chronic hemorrhage within the left lateral ventricle choroid plexus suggests that the hemorrhage originated within the xanthogranulomatous lesion at that site. This is a highly unusual source of bleeding and to our knowledge has not been previously described in the literature. Nevertheless, other common sources, including ruptured aneurysm, arterial venous malformation (AVM), and hypertension-associated intracerebral hemorrhage were all excluded.
Common causes of spontaneous intracranial hemorrhage include hypertension, aneurysm, vascular malformation, neoplasm, coagulopathy, drugs or alcohol, hemorrhagic infarct and others. Each year, approximately 37,000 to 52,400 people in the United States have an intracranial hemorrhage (4). Spontaneous intracranial hemorrhage has not been previously reported due to acute hemorrhage within a choroid plexus xanthogranuloma. Choroid plexus xanthogranulomas are usually considered benign, incidental findings. Reports show that they are found in 1.6%-7% of postmortem examinations (3). Most arise in the lateral ventricular choroid plexus and are asymptomatic with only a few symptomatic cases described in the literature (6). The first case of xanthogranuloma of the choroid plexus in man was reported by Blumer in 1900 under the diagnosis of "cholestomatous endothelioma" (3, 5). The stroma, and to a lesser extent the epithelium of the choroid plexus is subject to xanthomatous change, typically in the glomus within the trigone, or atrium of the lateral ventricle (1). Shuangshoti et al, suggested that the epithelium of the choroid plexus continuously proliferates (6). Desquamated epithelial cells enter the interstitium of the plexus at sites of disruption of the basal laminae and tubular walls (6). Lipid accumulates in these detached epithelial cells and as they degenerate these cells release lipids into the stroma of the plexus which provokes a response of macrophages and multinucleated foreign-body giant cells (6). These choroid plexus xanthogranulomas are then composed of xanthoma cells, cholesterol clefts, giant cells, hemosiderin, fibrosis, and occasional calcium deposits (1, 6).
In summary, we report an unusual case of a 51-year-old man who died suddenly and was found to have an intraventricular and subarachnoid hemorrhage secondary to acute hemorrhage within a choroid plexus xanthogranuloma.
ACKNOWLEDGMENTS The authors thank Dr. Arie Perry (Division of Neuropathology, Washington University School of Medicine, St. Louis, Missouri) for his reference diagnosis on the present case.
REFERENCES
Contributed by Kent J. Donelan, MD, Brad B. Randall, MD and Paul E. Newby, PA-ASCP
International Society of Neuropathology