Lymphangiomas are benign lesions of vascular origin that show lymphatic differentiation. It is considered the lymphatic equivalent of a hemangioma of blood vessels.
This article focuses on the general features of lymphangiomas. For a specific discussion in other locations, please refer to the articles:
- head and neck: cystic hygroma (cystic or nuchal lymphangioma)
- hepatic lymphangioma
- splenic lymphangioma
- pancreatic lymphangioma
- renal lymphangioma
- retroperitoneal lymphangioma
They can present at any age but most often occur in the pediatric population (~90% in those less than 2 years old 3). The worldwide incidence of lymphangiomas is 1:6000-16000 live births. Males and females are equally affected.
Generally, the presentation may be with symptoms related to local mass effect and/or hemorrhage.
For example, a lymphangioma within the orbit may present with progressive proptosis with acute deterioration in symptoms, the mass effect resulting in compressive optic neuropathy, diplopia/ocular muscle weakness and orbital bruising.
The clinical examination may reveal soft, non-tender masses on palpation with a doughy consistency.
Typically comprised of thin-walled cystic masses and may contain:
- large macroscopic interconnecting cysts: cystic hygroma or cystic lymphangioma
- microscopic cysts: cavernous lymphangioma
Their wall consists of connective tissue, smooth muscle, fat, blood vessels, nerve, or lymphatic tissue.
They can occur at almost any location:
- marked predilection in the head and neck: 95% in the neck and axillary regions
- mesentery, retroperitoneum, abdominal viscera, lung, and mediastinum: ~5%
There are several recognized subtypes, classified according to the size of the lymphatic cavities. In order of increasing size:
- simple microcystic or capillary lymphangioma
- macrocystic or cavernous lymphangioma
- cystic hygroma: cystic lymphangioma
- other: lymphovascular malformation (e.g. venolymphatic malformation)
Lymphangiomas may cross more than one compartment. For instance, in the head and neck region, larger lesions tend to occupy more than one deep space, sandwiching between normal structures.
- multilocular cystic masses
- internal septa of varying thickness
- cystic contents: usually anechoic, hyperechoic if debris, high lipid concentration, infection or hemorrhage
- wide variations exist: solid areas, or mostly solid with cystic foci
- color Doppler: +/- arterial or venous flow in the septa
Most lymphangiomas appear homogeneous and cystic on CT, but some appear inhomogeneous because of the presence of proteinaceous, fluid, blood, or fat components within the lesion. It is rare for CT to demonstrate intrinsic septations. There is only minimal or no displacement/compression of adjacent structures.
Fluid-fluid levels may be seen if complicated by hemorrhage. Signal characteristics include:
- T1: can be variable especially dependent on protein content
- T2: usually high signal
Treatment and prognosis
Surgical excision or interventional sclerotherapy (with interferon, OK-432, or bleomycin) is often necessary 3. Other possible treatment methods include steroid therapy, laser treatment, aspiration, radiofrequency ablation, or cautery.
Differentials will depend on which compartment or viscera is considered. Possible imaging differential considerations include:
- 1. Levy AD, Cantisani V, Miettinen M. Abdominal lymphangiomas: imaging features with pathologic correlation. AJR Am J Roentgenol. 2004;182 (6): 1485-91. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Shaffer K, Rosado-de-christenson ML, Patz EF et-al. Thoracic lymphangioma in adults: CT and MR imaging features. AJR Am J Roentgenol. 1994;162 (2): 283-9. AJR Am J Roentgenol (abstract) - Pubmed citation
- 3. Ha J, Yu YC, Lannigan F. A review of the management of lymphangiomas. (2014) Current pediatric reviews. 10 (3): 238-48. Pubmed
- 4. Bansal AG, Oudsema R, Masseaux JA, Rosenberg HK. US of Pediatric Superficial Masses of the Head and Neck. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (4): 1239-1263. doi:10.1148/rg.2018170165 - Pubmed