Moyamoya disease is a specific chronic cerebrovascular occlusive disease first reported by Japanese surgeons in 1957. The disease is characterized by stenosis or occlusion of the terminal portions of the bilateral internal carotid arteries and abnormal vascular network in the vicinity of the arterial occlusion. It may cause ischemic attacks or cerebral infarction, which is more frequent in children than in adults. In adults, cerebral hemorrhage may occur. The disease is distributed in all age groups, but the highest peak is in childhood at less than 10 years of age. The characteristic histopathologic features of the steno-occlusive arteries are fibrocellular thickening of the intima containing proliferated smooth muscle cells and prominently tortuous and often duplicated internal elastic lamina. There is usually no atheromatous plaque in the arterial wall. Etiology of the disease is still unknown; however, multifactorial inheritance is considered possible because of a higher incidence of the disease in Japanese and Koreans and approximately 10% of familial occurrence among the Japanese. Recent genetic studies suggest some responsible genetic foci in chromosomes 3, 6 and 17.
We here report on a rare case of Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) detected in both brain parenchyma and in an organized chronic subdural hematoma (OCSH). A 96-year-old man diagnosed with asymptomatic OCSH in the left frontal convexity was referred to our hospital because of a de novo mass lesion just beneath the OCSH on contrast-enhanced magnetic resonance imaging. The size of the OCSH remained stable. We diagnosed the lesion as a malignant tumor. At surgery, the organized hematoma and the soft fragile tumor were removed. Histological examinations revealed pleomorphic lymphoid cells not only in the brain tissue but also in the OCSH component with tumor necrosis, and these were immunopositive for B-cell markers. In situ hybridization revealed positive signals for EBV-encoded small RNAs, consistent with EBV-positive DLBCL. Since the membranes of the subdural hematoma were fibrous and the tumor progression resulted in necrosis of the tumor, the DLBCL may have originally developed in the OCSH and infiltrated into the brain parenchyma. We believe that this rare case provides crucial information for the understanding of DLBCLs associated with OCSH.
To clarify the morphogenesis of vascular anastomoses in the leptomeninx, we examined the leptomeningeal vessels of six autopsied patients with moyamoya disease who died of cerebral hemorrhage. Sites examined in the brain (the frontal pole, frontoparietal convexity, and lateral occipital region) were selected by the presence of angiographic abnormalities on the convexity of the hemisphere. At all examined sites, neither the vascular density (number of blood vessels per unit length of underlying cortical surface) nor the arterial/venous ratio differed significantly between the patients and 14 controls. All patients showed dilatative changes of both arteries and veins; however, attenuation or disruption of the internal elastic lamina and fibrous intimal thickening were more prominent in those patients who had had moyamoya disease longer. Our results indicate that the leptomeningeal vessels of moyamoya disease are not newly formed but are merely dilated preexisting vessels and that the structural alteration of the vascular walls suggests their adaptability for participation in the collateral circulation at the cerebral surface.
MRI before a biopsy has a high detectability of prostate cancer, particularly with tumor size of more than 5 mm in short-axis diameter or 10 mm in long-axis diameter.
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