Lymphangioma circumscriptum

Management Strategies

Although observation is an appropriate option for many cases, cosmetic concern is the typical indication for treatment. Other indications may include persistent leakage of lymphatic fluid or blood and recurrent infection. The risk of developing angiosarcoma and squamous cell carcinoma is trivial and should not be used to rationalize full surgical excision.

Treatment is challenging and often thwarted by local recurrences due to the persistence of deep lymphatic cisterns. Consequently, there is disagreement in the literature as to which treatment option is the most effective. Although complete surgical excision has the lowest recurrence rates, it has the highest rate of complications; more extensive lesions may be deemed inoperable.

Sclerotherapy using a variety of sclerosants has been advocated as a less invasive and effective treatment modality, which is either a first-line alternative or an adjunct to surgery.

Other authorities suggest that after definitive diagnosis and radiologic mapping, surgical excision and postoperative histologic assessment of excision margins provide the most effective treatment options.

Resurfacing of the lesions can be attempted and achieved even if proper surgical excision is not possible and/or sclerotherapy fails, but recurrence rates are usually higher than with other therapies. The high-energy, short-pulse CO ₂ laser has been found to yield functionally and cosmetically acceptable results. This seals communicating channels to the deeper cisterns by vaporizing the superficial lymphatics and is said to have fewer complications than more aggressive treatment alternatives. Other laser methods, particularly the pulsed dye laser, have also been shown to be effective in selected superficial cases.

In most cases the clinical diagnosis is straightforward. In some, the differential diagnosis may be broad: genital lesions are often associated with verrucous changes, which give them a warty appearance and are often confused with viral warts or squamous cell carcinoma; discoloration of the vesicles can lead to confusion with hemangiomas and even malignant melanoma; herpetic infection and dermatitis herpetiformis are less frequent differential diagnoses. Biopsy is diagnostic for clinically unusual cases and exhibits numerous thin-walled, dilated lymphatic channels encroaching onto the epidermis (which may become hyperkeratotic), expanding the papillary dermis and extending deep into the dermis and subcutis. Immunostaining with VEGFR3 and D2-40 decorates the vessels and confirms their lymphatic origin.

MRI can define the entire anatomy of a lesion and can help prevent unnecessarily extensive or incomplete surgical resection. Computed tomography (CT), ultrasound, and lymphoscintigraphy have also been useful in determining the extent of a lesion.

Secondary lesions may be investigated for an underlying cause. Lymphangiomas may be associated with rare disorders such as Proteus, Cobb, and Klippel–Trénaunay syndromes, and so appropriate investigations and consultations should be sought.

Intralesional sclerotherapy with group A Streptococcus pyogenes of human origin (OK-432) has emerged as an effective sclerosing agent.