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

lymphangiomatosis

Cystic angiomatosis of the bone is a rare but pathologically distinct entity characterized by multiple cysts scattered diffusely throughout the skeleton, which are often associated with similar changes in other tissues, notably the spleen. A wide variety of diagnostic terms such as ‘lymphangiomatosis’, ‘hemangiomatosis’ and ‘hamartous lymphangiomatosis’ of the bone have been used; however, the term ‘CAB’ is preferred because some cases have been described that contained either isolated hemangiomas or lymphangiomas, or a mixture of both vascular components. In addition, it is often impossible to differentiate hemangioma from lymphangioma, not only histopathologically but also clinically and radiologically. birth control pills
CAB usually presents during the first three decades of life, although this disorder has also been diagnosed in elderly patients (the oldest patient was 69 years of age). It had been reported to have a greater incidence in men ; however, a recent review showed no significant sex predominance. The clinical manifestations and course of the disease depend largely on the presence or absence of visceral involvement. When soft tissue is involved, the spleen is almost always affected. Other organs that may be involved include the liver, kidney, pleural and peritoneal membranes, mesentery, retro-peritoneum and lymph nodes . There is a wide range of clinical manifestations; the most common are soft tissue swelling, incidental findings on plain radiograph or pathological fracture. Other uncommon forms of presentation are dyspnea, hemoptysis, lymphedema, pleural effusion, splenomegaly, hepatomegaly, soft tissue masses and pneu-mocephalus.

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