Infected Giant Lymphangioma Circumscriptum Treated With a Combination Therapy
Article Outline
Lymphangioma circumscriptum is an uncommon benign disorder of skin and subcutaneous tissues characterized by dilated lymphatic channels. It is an uncommon vascular tumor and it rarely becomes infected. We report a 20-year-old man who had an infected giant tumor in his left thigh. After an extensive resection and radiofrequency energy therapy, he recovered well with an acceptable cosmetic result. We followed up the patient for 2 years without any recurrence.
Lymphangioma circumscriptum (LC) is an uncommon benign disorder of skin and subcutaneous tissues characterized by dilated lymphatic channels. It constitutes about 4% of all vascular tumors.1 The etiology is unclear, however, lymphatic obstruction has been suggested as a possible cause. It is classified into lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, and benign lymphangioendothelioma. It is a hamartomatous malformation of the lymphatic system of the skin and subcutaneous tissue. There are clusters of thin-walled vesicles on the surface of skin resembling frog spawn. This lesion rarely becomes infected.
Case Reports
A 20-year-old man was admitted to our hospital with complaints of swelling and pain in the left thigh. He had discomfort, difficulty in walking, and particularly in sitting. By examination, we detected a giant infected mass in the medial part of the left thigh (Fig. 1). It was around 12 × 16-cm in size. The skin overlying the swelling appeared thickened, irregular, and was not fixed to the underlying deep fascia and muscles. There were multiple scattered clusters of vesicles over the involved skin which was reddish and hot. Ultrasound verified that it was an infected vascular tumor and was confirmed by a magnetic resonance imaging (MRI).
Fig. 1
A giant mass in the medial part of the left thigh.
After a parenteral Ampicillin/Sulbactam therapy for 15 days, we performed an extensive surgical resection (Fig. 2). Primary closure of skin was performed with placement of drain and pressure bandage. Stitches were removed on the 14th day. The histopathological diagnosis revealed a lymphangioma circumscriptum. Three weeks after the operation, we used radiofrequency energy to treat two small residual lesions. With a copper ball electrode, power of 60 Watts was delivered for 2 seconds intermittently.
Fig. 2
Extensive surgical resection of the mass.
Two sessions were sufficient to eliminate these lesions. We followed up the patient for 2 years without any recurrence. He responded well with an acceptable cosmetic result.
Discussion
LC is an uncommon condition of the skin and subcutaneous tissue characterized by the presence of clusters of thin-walled vesicles filled with the clear fluid. The etiology is not clear, but the majority of cases are thought to represent congenital abnormality, possibly resulting from sequestered island of lymphatic tissue that develops without any connection to the normal lymphatic channel.2
Trauma including surgery and radiation therapy has been implicated in some cases as the causative agent.3 Lymphoedema is absent in the diseased area, because of the presence of normal lymphatic drainage.
LC is divided into smaller lesions (<7-cm) and extensive lesions. When size is greater than 7-cm it is also termed as lymphangioma diffusum. The incidence of LC is higher in the first and second decade of life with female preponderance. It can occur at any location. The common sites of involvement are proximal part of limbs, axillary folds, adjacent chest wall, and flanks.
The diagnosis is clinical with histological confirmation. Imaging is valuable in detecting the depth of penetration and the border of the lesions and its relationship to vital structures. MRI is a good choice to see the extent of involvement.4
The natural course of these lesions is slow progression of the lesion both in size and area with leakage of fluid, infection, local discomfort from hypertrophy, and irritation from clothing and movement.
Surgical excision is still the mainstay of treatment despite chances of recurrence.5 Other modalities are intra-lesional sclerotherapy using doxycycline or Picibanil (OK-432), vaporization by carbon dioxide laser, radiotherapy and radiofrequency energy.6, 7 The principle of surgery is based on the wide excision of all sequestrated lymphatic cisterns in the subcutaneous plane. The diseased subcutaneous tissue is recognized by the presence of cysts, fibrous strands, and wetness up to the deep fascia. The prognosis is excellent as it is a non-neoplastic condition. However, squamous cell carcinoma has been reported in an existing LC.8
Cosmesis and symptoms are the indications. MRI is sufficient to see the extension of tumor. To minimize the chances of recurrence, wide excision is recommended. In our opinion, combination therapies are more suitable for complicated patients such as our patient.
In conclusion, our patient was a young man, suffering from a giant tumor in the left thigh. The diagnosis revealed LC. After a combination therapy of surgical excision and radiofrequency energy, he recovered well in a few days without any recurrence within a follow-up period of 2 years.
References
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- . Surgical management of lymphangioma circumscriptum. Br J Surg. 1986;73:585–588
- . Lymphangioma circumscriptum following radical mastectomy and radiation therapy. Cancer. 1978;42:1989–1991
- . Disorders of lymphatic vessels. In: Champion RH, Burton JL, Burne DA editor. Rook/Wilkinson/Ebling Text book of Dermatology. 6th ed. New York: Blackwell Science; 1998;p. 2292–2293
- Lymphangioma circumscriptum. Pediatr Surg Int. 1998;208–210
- Sclerosing of recurrent lymphangioma using OK-432. J Paed Surg. 1995;30:1159–1160
- Treatment of lymphangioma circumscriptum with combined radiofrequency current and 900 nm diode laser. Dermatol Surg. 2006;32:790–794
- Squamous cell carcinoma arising within congenital lymphangioma circumscriptum. Br J Dermatol. 1993;129:337–339
PII: S0890-5096(10)00146-9
doi:10.1016/j.avsg.2010.02.048
© 2010 Annals of Vascular Surgery Inc. Published by Elsevier Inc All rights reserved.
