Congenital circumscribed lymphangioma of the vulva.
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Lymphangioma circumscriptum is not a tumor as the term might suggest. Lymphangiomas are actually lymphangiectases, whether they are acquired and secondary to tumors, radiotherapy or in any case to obstructions of the normal lymphatic circulation, whether they are congenital and thus malformations separated from the normal lymphatic circulation. It is not strange that congenital circumscribed lymphangiomas undergo transitory color changes from white to bluish-red due to spontaneous or traumatic intralesional hemorrhages, because lymphatic and venous malformations often coexist. With ultrasound it is really possible to demonstrate the presence of malformed blood vessels in the interstitium between the lymphatic cavities of macrocystic lymphangiomas. Congenital vulvar lymphangioma is rare and its therapy must be decided after magnetic resonance based on the biological needs of the patient (1, 2).