Abstract
Lymphangioma circumscriptum of the vulva occurs in patients who have undergone radical hysterectomy, lymph node dissection, or radiation therapy for management of advanced uterine cancer. Since vulvar lymphangioma circumscriptum typically presents as multiple, grossly verrucous vesicles of various sizes, it may be impossible to clinically distinguish vulvar lymphangioma circumscriptum from other vulvoperineal cutaneous diseases. In the present study, 16 (1.6%) out of the 1,024 vulvar biopsy or excision specimens were diagnosed as lymphangioma circumscriptum. In two (12.5%) out of the 16 cases, unusual histopathological findings were observed. Both patients had previously undergone radical hysterectomy with lymph node dissection and postoperative radiation therapy or concurrent chemoradiation therapy for advanced cervical cancer. Microscopic examination revealed high-grade squamous intraepithelial lesions, which were located immediately adjacent to the normal squamous epithelium covering the dilated subepithelial lymphatic vessels. Further, human papillomavirus genotyping confirmed that both patients were infected with high-risk human papillomavirus. High-grade squamous intraepithelial lesion cannot be grossly distinguished from vulvar lymphangioma circumscriptum because the multiple, verrucous vesicles that constitute the characteristic gross appearance of vulvar lymphangioma circumscriptum hinder its distinction. In this regard, our cases of high-grade squamous intraepithelial lesion, located adjacent to vulvar lymphangioma circumscriptum, support the notion that active surgical excision is necessary for the treatment of vulvar lymphangioma circumscriptum.
Highlights
Lymphangioma circumscriptum (LC) is a hamartomatous lymphatic malformation of the lymphatic channels of the skin, typically associated with a developmental anomaly of the lymphatics [1]
Vulvar LC can be acquired by uterine cancer patients who have undergone radical hysterectomy, pelvic lymph node dissection, and/ or pelvic radiation therapy that damages the lymphatic vasculature [4,5,6,7,8,9]
Contrary to the uncertainty of vulvar LC pathophysiology, almost all of the vulvar premalignant lesions are associated with human papillomavirus (HPV) infection [10,11,12]
Summary
Lymphangioma circumscriptum (LC) is a hamartomatous lymphatic malformation of the lymphatic channels of the skin, typically associated with a developmental anomaly of the lymphatics [1] It presents as a relatively well-circumscribed, localized lesion consisting of small, grouped vesicles that can ooze and drain lymphatic and sanguineous fluids. We recently experienced two HSIL cases, arising adjacent to vulvar LC, which occurred following surgery and postoperative concurrent chemoradiation therapy (CCRT) or radiation therapy (RT) for advanced cervical cancer. Our experience serves to remind the pathologists and gynecologists that HSILs can arise adjacent to vulvar LC and that checking for the presence of grossly unidentifiable HSIL by completely excising the vulvar LC lesion is important when LC develops in patients who have a history of surgery, radiation therapy, or concurrent chemoradiation therapy for advanced uterine cancer
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