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Cystic angiomatosis of the bone appearing as intrathoracic lung masses (1)

Cystic angiomatosis of the bone (CAB) is a rare, multi-centric disease that involves both the vascular and lymphatic systems, producing diffuse cystic lesions in the skeleton that are often accompanied by visceral involvement, especially the spleen. As of 1999, 56 cases have been reported in the medical literature.  There is a wide range of clinical manifestations, from a mild, asymptomatic form to a very severe form that can lead to early death . Both the appendicular and the axial skeleton can be affected, and the ribs are frequently involved. The typical appearance is one of multiple, small round or oval intraosseous cysts. We present the case of a 47-year-old woman with rib lesions appearing as intrathoracic masses; to our knowledge, this is the first such case in the literature. Her cough was usually dry with occasional white sputum, and was triggered by cold air and strong odors. Right knee and hip (Figure 1). She was told at that time that her chest radiograph was normal. ventolin inhaler

Figure 1. Left Bone biopsy showing replacement
Figure 1) Left Bone biopsy showing replacement of the intramedullary marrow space by a population of blood-filled vessels of variable size, lined by a single layer of well differentiated endothelium. The vessels are supported by a fibrous stroma. Right There is prominent osteoclastic resorption oftrabecular bone adjacent to the vasoformative lesions. In places, collections ofosteoclasts and stroma resemble the lesions seen in osteitis fibrosa cystica. (Hematoxylin and eosin stain, original magnification X400)

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