Periclitoral endometriosis: the dilemma of a chronic disease invading a rare location

Endometriosis affects 6% to 10% of women of reproductive age; extrapelvic endometriosis is considered a rare event with perineal endometriosis being even rarer still (only a few cases of spontaneous episodes described, the majority being from episiotomy scars). We present a unique case of periclitoral endometriosis, which to the best of our knowledge is the first in the literature. It is a 29-year-old nulligravida female with a painful fluctuant right periclitoral mass that had been growing with no response to antibiotic therapy. At the initial removal, pathology reported the lesion as endometriosis. The patient was placed on oral contraceptives, and she was noted to have monthly swelling and shrinking of the site with her menstrual cycles. When she went off hormonal contraception, she represented with the growing lesion 3.5 weeks after her last menses; she underwent re-excision. Because of the extension of the lesion medially and its adherence to the clitoral body, the decision was made to evacuate only as much of the capsule that could be safely identified to minimize the risk of damaging the clitoris. Complete excision in this case was difficult without sacrificing a portion of the clitoris and potentially resulting in decreased sexual function and persistent clitoral pain. In a patient in whom complete excision is not possible, there is potential for mass recurrence in the setting of residual tissue. Reviewing the literature suggests that there are risks with both recurrence and clitoral excision. We found that in-depth patient counseling, hormonal suppression, and close follow-up are necessary when dealing with periclitoral endometriosis postexcision.