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Radiologic-Pathologic Conferences of The University of Texas M. D. Anderson Cancer Center |
1 Division of Diagnostic Imaging, Box 57, The University of Texas M. D. Anderson
Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030.
2 Department of Pathology, The University of Texas M. D. Anderson Cancer Center,
Houston, TX 77030.
Received April 14, 2003;
accepted after revision May 14, 2003.
Address correspondence to E. M. Marom
(emarom{at}di.mdacc.tmc.edu).
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Lymphangiomas are rare, benign lesions that are believed to result from abnormal development of the lymphatic system. These lesions consist of endothelium-lined spaces that are supported by connective tissue stromata of variable thickness that contain lymphoid tissue, round cells, and smooth muscles. Lymphangiomas are locally infiltrative and tend to grow along tissue planes. They are classified into three types according to the size of the cystic spaces: simple lymphangiomas, cavernous lymphangiomas, and cystic lymphangiomas. The cystic spaces contain chyle. If blood cells contaminate the tissue specimens, the lymphangiomas may be mistaken for hemangiomas [1, 2]. Occasionally, lymphangiomas occur diffusely, and this process is referred to as "generalized lymphangiomatosis."
As many as 65% of lymphangiomas present during infancy or childhood. These masses have been reported to occur in all organs except the brain, which is devoid of lymphatic channels [1]. Lymphangiomas are often slow-growing and progressive lesions. Patients may present with a variety of symptoms depending on the organ involved; these symptoms include cosmetic abnormalities, organomegaly, and chest tightness and shortness of breath. Chest symptoms may be caused by pleural effusions, diffuse pulmonary involvement, ascites, or a pathologic fracture of a rib.
Radiographic findings are nonspecific and may show pleural effusions, mediastinal widening, or soft-tissue masses, sometimes with coarse calcifications. Historically, imaging evaluation was performed using lymphangiography [3]. CT and MRI are the current techniques used to evaluate lymphangiomas, which appear as large multicystic fluid-filled masses. Imaging is often helpful in distinguishing lymphangiomas from various vascular disorders [3] and is commonly used for preoperative planning before surgical resection. MRI is the imaging technique of choice for delineating the infiltrative components of the lesion [1].
CT and MRI of lymphangiomas are particularly important because, when revealed, multiorgan involvement suggests the diagnosis of generalized lymphangiomatosis. Common findings in generalized lymphangiomatosis are cystic lesions in parenchymal organs, mesenteric thickening, lytic bone lesions, diffuse thickening of the pulmonary interstitium, pulmonary nodules, mediastinal masses, and pleural and pericardial effusions [1–3]. Although tissue diagnosis is recommended in all suspected cases of lymphangioma [1], the imaging appearance of multiorgan involvement is typical for generalized lymphangiomatosis and precludes multiple biopsies.
Treatment of lymphangiomas depends on the extent of disease and can be problematic because of the slow, infiltrative, and progressive nature of the disease. Surgical excision is recommended for localized lesions. However, because lymphangiomas are commonly infiltrative, surgical resection is often incomplete and the local recurrence rate is high. With extensive disease or generalized lymphangiomatosis, the role of surgery is limited to biopsy for diagnosis or for palliation. Palliative procedures often include draining pericardial effusions or pleurodesis for recurrent pleural effusions. Treatment with interferon-alfa or radiation therapy with a fractionated dose of 18–20 Gy has been shown to be effective and safe [1].
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  H. Liu, D.R. Shatzkes, and R.A. Holliday Bilateral Orbital Lymphangiomas in Two Patients with Generalized Lymphangiomatosis AJNR Am. J. Neuroradiol., March 1, 2007; 28(3): 491 - 492. [Abstract] [Full Text] [PDF]
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