Lymphangioma Clinical Presentation

Updated: Apr 23, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Lymphangioma circumscriptum

The typical history involves a small number of vesicles on the skin at birth or shortly after. In subsequent years, they tend to increase in number, and the area of skin involved continues to expand. Vesicles or other skin abnormalities may not be noticed until several years after birth. Usually, lesions are asymptomatic, but, occasionally, patients may have spontaneous episodes of minor bleeding and copious drainage of clear fluid from ruptured vesicles.

Cavernous lymphangioma

Typically, during infancy, a solitary rubbery nodule with no skin changes becomes evident in a single location, such as the face, trunk, or extremity. These lesions often have a fast growth phase similar to that of raised hemangiomas. No family history of prior lymphangiomas is described.

Cystic hygroma

Soon after birth, the infant is noted to have a deep subcutaneous cystic swelling, usually in the axilla, base of the neck, or groin. If these lesions are drained, they tend to fill up rapidly with lymph fluid. The lesions tend to grow and increase to a large size if they are not completely excised at surgery.

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Physical Examination

Lymphangiomas may affect a number of sites, including the scrotum. Multilocular lymphangiomatosis of the scrotum is unusual. [8] The site of a lymphangioma may determine its clinical signs and symptoms. For example, a lacrimal gland lymphangioma may result in acute proptosis and inferonasal displacement of an eye. [9]

Note the images below.

A 44-year-old woman with plaque on her forearm sin A 44-year-old woman with plaque on her forearm since birth that clinically appeared to be typical lymphangioma circumscriptum. Histologically, however, it had features of both hemangioma and lymphangioma.
Close-up demonstrating the clinical morphology to Close-up demonstrating the clinical morphology to better advantage.

Lymphangioma circumscriptum

Lymphangioma circumscriptum involves small clusters of vesicles measuring about 2-4 mm. These clear vesicles can vary from pink to red to black secondary to hemorrhage. [1] Although it may appear localized to the dermis, this neoplasm frequently extends deeply and laterally. The lesions can have a warty appearance on their surface; as a result, they are often confused with warts. Shah et al report a lymphangioma presenting on the penis. [10]

Cavernous lymphangioma

Typically, cavernous lymphangiomas appear as subcutaneous nodules with a rubbery consistency. They may have large dimensions. The overlying skin has no lesions or changes. The area of involvement varies, ranging from lesions smaller than 1 cm in diameter to larger lesions that involve an entire limb.

Cystic hygroma

Cystic hygromas are usually larger than cavernous lymphangiomas, and they more commonly occur in the neck and parotid area. Often, deep cavernous lymphangiomas are not evident on superficial examination, but cystic hygromas are detected with ease because of their size and location. These large cystic lesions are soft and translucent. Congenital and acquired lymphangiomas of the vulva are rare. [11, 7] Diffuse lymphangiomas may go unnoticed, evident as asymptomatic, erythematous flat, indurated, or atrophic plaques with or without swelling with or without any surface changes.

Fetal lymphangioma

Fetal lymphangioma may involve fetal skin and occasionally mucosa and subcutaneous tissue, typically on neck or axilla. [6] The fetal chest wall cystic lymphangioma is rare. [12]

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Complications

Lymphangioma circumscriptum may occur. Lymphangiomas circumscriptum is associated with minor bleeding, recurrent cellulitis, and lymph fluid leakage. Two cases of lymphangiosarcoma arising from lymphangioma circumscriptum have been reported. However, in both of the patients, the preexisting lesion was exposed to extensive x-ray therapy. Therefore, radiation therapy should be avoided in lymphangiomas.

In cystic hygroma, large cysts can cause dysphagia, respiratory problems, and serious infection if they involve the neck.

A cystic lymphangioma of the scrotum may present as an acute scrotum, owing to the rare complication of hemorrhage. [13]

A Dabska tumor may rarely arise within lymphangioma circumscriptum. [14]

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