LYMPHANGIOMA CIRCUMSCRIPTUM is a benign disorder of the lymphatics. Primary lymphangioma circumscriptum is usually present at birth or develops in early childhood, whereas secondary lymphangioma circumscriptum is induced by surgical intervention and radiation therapy for malignancy. 1–3 Vulvar involvement is very rare. Radical hysterectomy, with subsequent pelvic radiation therapy, appears to be an important but only recently recognized risk factor. 3–5
We report two cases of vulvar lymphangioma circumscriptum and present a brief review of the literature.
A 75-year-old woman presented to our outpatient clinic with edema of both labia majora, papules in this area, and oozing of clear fluid. The symptoms had started 10 years previously with a few papules, which had multiplied during the course of the disease. Fifteen years after radical abdominal hysterectomy, lymph node dissection, and subsequent irradiation of a stage III squamous cell carcinoma of the cervix (in 1975), the cutaneous symptoms had started. Genital warts were suspected and podophyllotoxin was applied topically, but this treatment was unsuccessful.
On examination, edema of the vulva and multiple papules and vesicles on both labia majora, with a partly uneven and partly verrucous surface, were noted (Figure 1). General examination findings were unremarkable.
Histologic examination revealed multiple dilated vascular channels in the papillary dermis containing few erythrocytes and mostly fibrin, lined by a thin wall consisting only of endothelial cells. There was an inflammatory infiltrate in the papillary dermis. The overlying epidermis was partly hyperkeratotic, with thinning of the stratum malpighii (Figure 2). There was no evidence of malignancy.
The patient was first treated with CO2 laser vaporization of the whole area (350 J; pulse frequency, 20/sec). After a relapse occurred 3 months later, the second treatment consisted of two phases: the first with vaporization at 0.350 J and 20 pulses/sec and the second (24 hours later) with vaporization at 0.400 J and 30 pulses/sec. Because the lymphangioma circumscriptum was associated with chronic lymphedema, complex physical decongestive therapy was used as a preventive measure; this was initiated as daily treatment for 4 weeks and continued once a week in combination with the use of compression stockings, worn up to the groins. This time, no relapse had occurred by the time of a follow-up at 8 months after laser treatment.
In spring 2000, a 46-year-old woman presented with gross edema of the vulva, some vesicles (Figure 3), oozing, and pain, 9 years after hysterectomy that had been followed by lymph node dissection and irradiation of cervical cancer. Histology confirmed the diagnosis of lymphangioma circumscriptum.
Treatment with CO2 laser vaporization proved unsuccessful. To the contrary, solid nodules developed in the areas of laser coagulation at the vulva only a few months after treatment. Histologic examination revealed multiple bundles of eosinophil collagen. The collagen bundles were markedly thickened. Between them, dilated lymphatic vessels were seen, as well as small granulomas that had developed around destroyed hair follicles. The diagnosis was vulvar keloid. The patient refused to undergo surgical intervention.
The pathogenesis of secondary lymphangioma circumscriptum (synonyms: acquired lymphangiectasis, acquired lymphangioma) occurring after lymphatic damage due to cancer treatment, up to 43 years after therapy, is not yet fully understood. 2 It is presumably related to increased hydrostatic pressure within vessels proximal to lymphatic obstruction. Alternatively, lymphangioma circumscriptum may in some instances represent the lymphatic counterpart of papillary dermal teleangiectasia and hemolymphangioma, which seldom follow radiation therapy. There is evidence that they occur in the area of a previously existing lymphedema, although the latter is not essential. 2,4,6 Vulvar involvement is especially rare: only 28 cases have previously been reported. 5
Lymphangioma circumscriptum is characterized by persistent groups of thin-walled pseudovesicles, but rarely the morphologic characteristics range from papular, nodular lesions to wartlike lesions of the vulva, as in our first patient, and are accompanied by itching, pain, lymphorrhea, and recurrent cellulitis. 4 The lesions can be indistinguishable from genital warts, a circumstance which makes differential diagnosis difficult. 7 For this reason, differential diagnosis of vulva lesions occurring after surgical and radiologic cancer therapy should include lymphangioma circumscriptum.
In contrast to lymphangiosarcoma of Stewart–Treves, lymphangioma circumscriptum occurring after cancer treatment is not known to have a potential for malignant transformation.
The traditional treatment of lymphangioma circumscriptum consisted of surgical removal, which has proved to be frequently unsuccessful, and rapid relapses have been observed. 1,8,9 Recently, vaporization with a CO2 laser has been recommended as treatment for lymphangioma circumscriptum of the vulva. 1,5,8,9 It is also effective in vaporizing some of the communicating vessels to deeper cisterns, with an acceptable cosmetic result. 1 Surgical excision should be reserved for treatment failures. 8 Therapeutic failures after laser treatment of lymphangioma circumscriptum, as observed in our first patient after the initial course of CO2 treatment, have already been reported. Whereas this patient responded well to modified CO2 laser therapy, our second patient presented with greater edema and pain and more vesicles, and keloids developed after the first CO2 laser surgery. To our knowledge this is the first report of keloid development after laser therapy for lymphangioma circumscriptum of the vulva, and the possibility of such an occurrence should be considered before application of CO2 laser surgery for this particular entity.
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