Congenital lymphangioma circumscriptum of vulva presenting as multiple giant mass lesions: a case report and literature review

A G2L2 33-year-old woman presented to our clinic with large verrucous warty masses in labia major, perinea and the end portion of her spine measuring about 15 x 7, 9 x 7 and 8 x 8 cm, which had been enlarged following puberty and pregnancy. Her right upper and left lower limbs had gross congenital lymphedema. The masses were removed by superficial partial vulvectomy with a qualified margin and repaired without skin graft. Pathology report showed lymphangioma circumscriptum. Several months of follow up revealed normal healing and no recurrence. In conclusion, congenital vulvar lymphangioma, which is a rare disorder, can be highly triggered by hormonal-stimulating situations like puberty and pregnancy. Thus, it is better to visit the affected cases in a timely manner in order to excise these lesions before massive enlargement. In our experience, a superficial partial vulvectomy without skin graft can be a sufficient procedure. Obviously, an appropriate approach during and after surgery would be very important for obtaining a desirable healing.