Objective:

To report the case of a patient diagnosed with ectopic mammary tissue in the vulva, and to conduct a literature review of the diagnosis, treatment and prognosis of this condition in that location.

Material and methods:

A 49-year-old patient who presented with a painful vulvar mass to a private intermediate complexity center in Bogotá, Colombia. The lesion was assessed on ultrasound and then surgically excised; histopathology showed ectopic mammary tissue with absence of malignancy. A search was conducted in the PubMed, Embase, Cochrane, LILACS and Scielo databases using the keywords “Vulva,” “Breast” and “Ectopic.” Case reports and case series of women with histopathology-confirmed mammary tissue in the vulva were included.

Results:

Overall, 184 titles were identified and, of these, 94 were ultimately included, for a total of 126 cases, with 57.9% being benign tumors, 95% in women under 50 years of age, and 42.06% being malignant tumors, 92% in women over 50 years of age. Diagnosis was made on the basis of the clinical findings, with ancillary diagnostic imaging, tumor markers and immunohistochemistry in some cases. Local excision was performed in 91% of cases with benign pathology and in 43% of cases with malignant pathology, with the diagnostic method being therapeutic.

Conclusion:

Ectopic mammary tissue in the vulva must be considered as part of the differential diagnosis of vulvar masses, prognosis being different in pre and postmenopausal women. Further studies are needed to enhance the characterization of this condition and define the ideal course of treatment in terms of relapse and survival.

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