Akademik Veri Yönetim Sistemi
European Congress of Radiology 2025 , Vienna, Avusturya, 26 Şubat - 02 Mart 2025, (Yayınlanmadı)
Purpose or Learning Objective
The purpose of this presentation is to describe the imaging findings of Gorham-Stout disease and various involvements of skeletal system.
Methods or Background
Four cases are presented with imaging studies from our database between the years of 2012-2024. Three of the patients were male and one of them was female.
Gorham-Stout disease, also known as primary bone lymphangioma, hemangiomatosis with massive osteolysis, vanishing bone disease or phantom bone disease is a rare skeletal disorder that is characterized by massive osteolysis of a bone or a contiguous group of bones in the body.
Results or Findings
Initially described as “missing bone disease with intraosseous vascular alterations”, the monocentric form of osteolysis of a bone or contiguous area of bone, with a predilection for the axial skeleton, was subsequently named as Gorham-Stout syndrome. In decreasing order of frequency, it affects the scapula, proximal end of the humerus, femur, rib, iliac bone, ischium, and sacrum. In our patients, bones around shoulder girdle were also affected more. Gorham-Stout syndrome primarily affects children and young adults. The histopathological hallmark is the replacement of normal bone by fibrous tissue with aggressive, expansile, non-neoplastic proliferation of capillary or cavernous blood vessels, a process that can culminate in the replacement of the entire bone by fibrous tissue. The syndrome initially presents as foci of rarefaction in the bone marrow and subcortical bone, with slow, irregular progression that can result in effacement of the diaphysis of the bones, narrowing of the involved ends, and, in some cases, the complete disappearance of the bone. Pathological fractures that do not consolidate are common.
Conclusion
Although it is a rare disease, we must consider Gorham-Stout disesase in lytic lesions of the bones.
References
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