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Angiomatosis is defined as diffuse infiltration of bone or soft tissue by hemangiomatous or lymphangiomatous lesions (1,5,52,53,54,55,56,57). Generally, patients are affected in the first 3 decades of life. Predominant involvement of osseous structures (30% 40% of angiomatosis cases) often follows a relatively indolent course (5,52,53,54). On the other hand, extensive soft-tissue and visceral involvement by angiomatosis (60% 70% of cases) carries a poorer prognosis (5,55,56,57). Generally, the lesions in angiomatosis have no malignant potential except in Maffucci syndrome (58).
Osseous lesions occur, in order of decreasing frequency, in the femur, ribs, spine, pelvis, humerus, scapula, other long bones, and clavicle (5,52,53). At radiography, angiomatosis involving bone appears as lytic areas, often with a honeycomb or latticework (hole-within-hole) appearance (Figure 14), similar to that of a solitary osseous hemangioma. When bone is involved extensively, the term cystic angiomatosis has been applied. Multifocal involvement is usually apparent and may occur on both sides of a joint (Figure 14). Osseous lesions may have sclerotic margins.
The appearance of angiomatosis at CT and MR imaging is identical to that of solitary angiomatous lesions (54). These modalities allow assessment of the extent of associated soft-tissue involvement.
At lymphangiography, the lymphangiomatous components of angiomatosis can show uptake of contrast material. Soft-tissue involvement by lymphangiomatous elements can be associated with chylothorax, chyloperitoneum, lymphedema, hepatosplenomegaly, and cystic hygroma.
Angiomatosis