Lymphangioma circumscriptum

Published on 18/03/2015 by admin

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Lymphangioma circumscriptum

Patrick O.M. Emanuel and Ben Tallon

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Lymphangioma circumscriptum is an uncommon lymphatic malformation. It presents on the skin surface as grapelike groups of thin-walled translucent lymph-filled vesicles, often compared to frog spawn. Hemorrhage within the lesions can create a deep red or brown appearance.

More commonly congenital, they are typically noted at birth or appear during childhood. They are most commonly found around the shoulder girdle and proximal limbs. There is a morphologically identical acquired variant related to lymphatic obstruction as a consequence of surgery, radiation, or malignancy.

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 owing to the persistence of deep lymphatic cisterns which may delve deep into the subcuticular adipose tissue, skeletal muscle, and nerves. Every treatment option has associated recurrence rates/complication profiles. 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 provides the most effective treatment option.

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 CO2 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.

Specific investigations

In the majority of 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, when used preoperatively, can help prevent unnecessarily extensive or incomplete surgical resection. CT, ultrasound, and lymphoscintigraphy have also been useful in determining the extent of a lesion.

Secondary lesions may be investigated for an underlying cause if the cause is not clinically obvious. Lymphangiomas may be associated with rare disorders such as Proteus, Cobb and Klippel–Trenaunay syndromes, and so appropriate investigations and consultations should be sought if these conditions are suspected.

First-line therapies

image Conservative/observation A
image Antibiotics D
image Sclerotherapy A
image OK-432 A
image Hyperosmolar saline D
image Sodium tetradecyl sulfate E
image Surgery A

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

Surgery

Second-line therapies

image CO2 laser D
image Pulsed dye laser D
image Radiotherapy E
image Cryotherapy D
image Argon laser D
image Suction-assisted lipectomy D

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Lymphangioma circumscriptum

Patrick O.M. Emanuel and Ben Tallon

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Lymphangioma circumscriptum is an uncommon lymphatic malformation. It presents on the skin surface as grapelike groups of thin-walled translucent lymph-filled vesicles, often compared to frog spawn. Hemorrhage within the lesions can create a deep red or brown appearance.

More commonly congenital, they are typically noted at birth or appear during childhood. They are most commonly found around the shoulder girdle and proximal limbs. There is a morphologically identical acquired variant related to lymphatic obstruction as a consequence of surgery, radiation, or malignancy.

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 owing to the persistence of deep lymphatic cisterns which may delve deep into the subcuticular adipose tissue, skeletal muscle, and nerves. Every treatment option has associated recurrence rates/complication profiles. 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 provides the most effective treatment option.

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 CO2 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.

Specific investigations

In the majority of 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, when used preoperatively, can help prevent unnecessarily extensive or incomplete surgical resection. CT, ultrasound, and lymphoscintigraphy have also been useful in determining the extent of a lesion.

Secondary lesions may be investigated for an underlying cause if the cause is not clinically obvious. Lymphangiomas may be associated with rare disorders such as Proteus, Cobb and Klippel–Trenaunay syndromes, and so appropriate investigations and consultations should be sought if these conditions are suspected.

First-line therapies

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image Conservative/observation A
image Antibiotics D