Surgical Management of Primary Vulvar Lymphangioma Circumscriptum and Postradiation: Case Series and Review of Literature

Abstract 

Vulvar lymphangioma circumscriptum is a defect of lymphatics in the reticular dermis. It can occur as either acquired—induced by radical hysterectomy and pelvic radiation for cervical cancer–or congenital. Lymphangiomas of the vulva are rare. Thirteen cases of congenital and 24 cases of the acquired form have been reported in the literature. To describe major labiaectomy as surgical management of primary vulval lymphangioma and postradiation, experiences about surgical treatment of 3 cases of this rare disease are reported. Three female patients (aged 17, 18, and 63 years) had persistent edema, papules, and vesicles of the labia major. Two patients had congenital lymphangioma circumscriptum. Another patient developed it 9 years after radical hysterectomy, lymph node dissection, and adjuvant pelvic radiation of cervical cancer. Qualitative analysis of signs and symptoms that occurred before and after surgery was performed. Clear improvement in symptoms and signs associated with these conditions occurred in all patients postoperatively. In particular, all patients reported reduction in the amount of edema after surgery. Our data suggest major labiaectomy is more successful than methods such as lymphovenous anastomoses, lymph angioplasties, and laser therapy. A single operation may provide benefit for a long period.

Keywords: Vulvar lymphangioma, Congenital, Acquired, Radiation, Major labiaectomy, Outcome