Generalized Lymphangiomatosis: Radiologic Findings in Three Pediatric Patients

Generalized lymphangiomatosis is a rare disease that is characterized by wide-spread bony and soft tissue involvement of lymphangioma. Radiological evalua-tion is crucial because the site and extent of the lymphangioma are important prognostic factors. We reported here on three cases of generalized lymphan-giomatosis and all three cases showed similar radiologic findings, but a different clinical course. The CT, US and MR images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung and lower neck. The whole body MR imaging with the short tau inversion recovery (STIR) sequence showed good capability for evaluating the extent of disease. eneralized lymphangiomatosis is a rare disease that is characterized by a diffuse proliferation of lymphatic vessels (1). The lymphangioma is composed of lymphatic endothelium-lined cystic spaces, and this can be histologically differentiated from other vascular disorders such as cavernous or capillary hemangioma (1, 2). However, some cases of lymphangioma may be confused with other vascular disorders due to the potential overlapping histologic findings (2). Generalized lymphangiomatosis can involve any organ except the central nervous system, and the prognosis depends on the involving organ and the extent of the disease (1 3). Because radiologic examination, including CT and MR imaging, is useful for evaluating the morphologic feature as well as the extent of disease, it is important to know the radiologic findings of generalized lymphangiomatosis. In this report, we described the radiologic findings of generalized lymphangiomatosis in three patients. CASE REPOR