Malik Reeni, Malik Rajesh, Tandon Suneet, Tandon Puneet
Department of Pathology, Gandhi Medical College, Bhopal, India.
Indian J Pathol Microbiol. 2008 Oct-Dec;51(4):515-8. doi: 10.4103/0377-4929.43745.
Classification of skeletal angiomatosis into aggressive and nonaggressive types is on the basis of their clinical behavior and pattern of skeletal involvement (regional and disseminated). Gorham's disease (massive osteolysis) is an aggressive form of skeletal angiomatosis that shows regional involvement, frequently involving the shoulder and hip areas. Cystic angiomatosis is a nonaggressive form of skeletal angiomatosis with multifocal involvement, predominantly affecting the trunk bones. The imaging modalities gave the diagnosis of cystic angiomatosis of humerus showing multicystic lytic areas. The histopathological differential diagnosis was cystic angiomatosis and Gorham's disease, as microscopically both are indistinguishable from each other. Both represent a complex network of dilated thin-walled capillaries growing in the marrow space associated with the destruction of bone and infiltration into the adjacent soft tissues. The case is presented because of its extreme rarity and due to the diagnostic dilemma, whether to label it as Gorham's disease or as cystic angiomatosis. Considering the site involved and its aggressiveness, the diagnosis is in favor of Gorham's vanishing bone disease as cystic angiomatosis is multicentric and nonaggressive, involving mostly vertebrae and skull with multicystic lytic lesions.
骨血管瘤病分为侵袭性和非侵袭性类型是基于其临床行为和骨骼受累模式（局部和播散性）。戈勒姆病（大块骨质溶解症）是骨血管瘤病的一种侵袭性形式，表现为局部受累，常累及肩部和髋部区域。囊性血管瘤病是骨血管瘤病的一种非侵袭性形式，有多灶性受累，主要影响躯干骨。影像学检查诊断为肱骨囊性血管瘤病，表现为多囊性溶骨区。组织病理学鉴别诊断为囊性血管瘤病和戈勒姆病，因为在显微镜下两者无法区分。两者均表现为在骨髓腔内生长的扩张薄壁毛细血管构成的复杂网络，伴有骨破坏和向邻近软组织浸润。之所以呈现该病例，是因为其极为罕见，且存在诊断困境，即应将其诊断为戈勒姆病还是囊性血管瘤病。考虑到受累部位及其侵袭性，诊断倾向于戈勒姆骨消失病，因为囊性血管瘤病是多中心且非侵袭性的，主要累及脊椎和颅骨，伴有多囊性溶骨病变。
