Background: The vulva is an extremely rare site for lymphangioma, a benign tumor of the lymphatic vessels. A small number of case reports have described cavernous lymphangioma involving the unilateral labium majus of young women. We report a case of cavernous lymphangiomas involving the bilateral labia minora. Case: A 14-year-old girl presented with bilateral tumors of the labia minora, which were removed surgically and histologically diagnosed as cavernous lymphangioma. Positive nuclear staining for estrogen and progesterone receptors was demonstrated in the stromal fibroblasts immunohistochemically. Conclusion: It is suggested that an increase in estrogen and progesterone levels may have led to, or at least affected, the development of these tumors in this patient. Excision followed by cosmetic repair may be the optimal treatment.
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