A 51‐YEAR‐OLD MAN WITH SUDDEN UNEXPECTED DEATH

CLINICAL HISTORY

A 51‐year‐old man was discovered deceased on the bathroom floor of his home. He had no history of hypertension or other chronic disease. Due to the lack of significant medical history and the unexpected nature of the death, an autopsy was ordered by the county coroner. At autopsy, no apparent thoracic or abdominal cause of death was identified. The decedent did have mild cardiomegaly (525 grams) with left ventricular hypertrophy (2.4 cm). No acute ischemic changes were seen grossly. Sectioning of the coronary arteries showed minimal luminal narrowing and no evidence of acute thrombotic occlusion.

GROSS AND MICROSCOPIC PATHOLOGY

The brain weighed 1300 grams, post fixation. The meninges were clear over the convexities, however, there was diffuse subarachnoid blood accumulation over the base of the brain in a symmetric distribution (Figure 1). A large amount of subarachnoid blood was especially noted near the brainstem at the level of the fourth ventricle foramina. The vasculature was dissected away from the base of the brain. Atherosclerosis was absent and no aneurysms were found on close inspection. There was no evidence of herniation. Coronal sections of the brain showed no gross intra‐parenchymal hemorrhage. However, located in the left lateral ventricle and the pineal region/recess of the third ventricle, was an area of accumulated acute blood clot and prominent vessels, suggesting the possibility of a vascular malformation. The hemorrhage extended into the rostral aqueduct of Sylvius, as well as the basal cisterns and adjacent leptomeninges. On cut surface, a central nodule of firm tan‐grey tissue measuring 1 cm in greatest diameter was found within the blood cast of the left lateral ventricle.

Figure 1.

Microscopically, tissue sections revealed a thick layer of acute subarachnoid hemorrhage surrounding the pineal gland. Embedded within the area were thick‐walled muscular blood vessels and small thin‐walled venous channels, but they were anatomically unremarkable and thus, there was no evidence of a true vascular malformation. A small cystic structure within the third ventricle showed a papillary epithelial lining with features of non‐neoplastic choroid plexus. The surrounding gray and white matter appeared unremarkable. The pineal gland was histologically unremarkable and was partially calcified. Sections of the lateral left ventricle showed acute non‐organizing hemorrhage within the ventricle and adjacent choroid plexus. Microscopically, the nodular focus seen grossly in the left lateral ventricle revealed marked chronic xanthogranulomatous inflammation with extensive cholesterol clefts, foreign body reaction, and focal calcifications. A periphery of normal choroid plexus was identified around the nodule (Figure 2). There was evidence of both recent and remote hemorrhage, the latter evidenced by collections of hemosiderin‐laden macrophages. Additional representative sections demonstrated evidence of acute hemorrhage within the third ventricle, aqueduct, and subarachnoid space, particularly heavy within the pineal recess.

Figure 2.

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 (2). 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.

ABSTRACT

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. This is a highly unusual source of bleeding and to our knowledge has not been previously described in the literature. The man was discovered deceased on the bathroom floor of his home and an autopsy was ordered by the county coroner. Examination of the brain showed diffuse subarachnoid blood accumulation over the base of the brain in a symmetric distribution. A large amount of subarachnoid blood was especially noted near the brainstem at the level of the fourth ventricle foramina. Sections of the lateral left ventricle showed acute non‐organizing hemorrhage within the ventricle and adjacent choroid plexus. Microscopically, a nodular focus seen grossly in the left lateral ventricle revealed marked chronic xanthogranulomatous inflammation with extensive cholesterol clefts, foreign body reaction, and focal calcifications. A periphery of normal choroid plexus was identified around the nodule. There was evidence of both recent and remote hemorrhage.