Abstract:

Vulvar lymphangioma is a rare entity which may present in variety of clinical settings and often resembling a cyst or mass. Its etiology has been attributed to infections, malignancies and radiotherapy. We performed a retrospective search using keywords ‘vulvar lymphangioma’ that was ever entered in our database and identified five cases, but only included three due to lack of clinical information. One patient had history of squamous cell carcinoma of the vagina and was treated by excision and primary radiation. She subsequently developed chronic lymphedema and presented with painful blisters in her labia a decade later that led to a partial vulvectomy. Sections revealed florid lymphangiectasia with lymphangioma circumscriptum-like pattern in the superficial papillary dermis which stained positive for ERG, CD31, D2-40 and negative for CD34. All the other cases had similar histology with varying degree of lymphatic channels within the superficial papillary dermis, abutting the epidermis and slightly below with flat endothelial lining, many containing eosinophilic proteinaceous lymph fluid. Vulvar lymphangiomas are benign proliferations generally present at birth or developed later in childhood. While some have categorized these lesions as ‘acquired’, others have classified it as a reactive proliferation which have been reported to arise in the setting of radiotherapy secondary to chronic lymphedema. These entities are probably underreported in gynecologic pathology although it is well recognized in the breast. Acquired cutaneous lymphangiectasia are prone to recurrence if the deeper lymphatic abnormalities are not completely excised. Malignant transformation arising in such lesions is reportedly rare in the literature.

Financial Disclosure:
No current or relevant financial relationships exist.

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Annie Abraham, MD