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Lymphangiomas are composed of sequestered, noncommunicating lymphoid tissue lined by lymphatic endothelium and are thought to be caused by congenital obstruction of lymphatic drainage (41). Lymphangiomas are subclassified by vessel size as capillary (rare and located in subcutaneous tissue), cavernous (located about the mouth and tongue), and cystic. However, lymphangiomas are often an admixture of histologic subtypes. Cystic and cavernous lymphangiomas contain large, unilocular or multilocular spaces filled with proteinaceous material, and histologic distinction from pure cavernous hemangiomas may be difficult (41).
The vast majority of lymphangiomas of the musculoskeletal system are soft-tissue lesions (Figure 11, Figure 12), with an osseous location being rare (42,43). These lesions are usually found at birth (50% 65% of cases) or at least within the first 2 years of life (90%) (44). Cystic lymphangioma (cystic hygroma) is the most common type of lymphangioma. It contains serous or chylous fluid and extends into the mediastinum in 3% 10% of cases (45,46). Clinically, these lesions are soft, fluctuant masses that involve the head, neck (75% of cases), and axilla (20%). Cystic hygroma is also associated with hydrops fetalis and Turner syndrome (47).
At radiography, a lymphangioma appears as a nonspecific soft-tissue mass. At CT, MR imaging, or sonography, lymphangiomas show cystic characteristics, often multilocular, with fibrous septations (Figure 11, Figure 12) (44,45,46,47,48). Uncommonly, these lesions may have a more complex appearance, with serpentine vascular channels similar to those seen in hemangiomas. Siegel and coworkers (46) reported that in four of 17 cases the lymphangiomas had high signal intensity, similar to that of fat, on T1-weighted MR images. Lymphangiomas can be opacified during lymphangiography or direct puncture with injection of contrast material (Figure 11). Aspiration of these lesions may reveal clear or milky fluid, as opposed to the hemorrhage seen in hemangiomas.
Lymphangioma