A review of the world literature regarding carcinoma of ectopic breast tissue along with the addition of one case is reported. A total of 90 cases of carcinoma of ectopic breast tissue were found, 64 of which occurred in the axilla. The combined survival beyond the 4-year post-treatment period was 9.4%. No survival advantage was found for radical or modified radical mastectomy over that of local excision combined with axillary dissection or radiation. The addition of radiation therapy to either type of mastectomy provided no additional benefit. The correct preoperative diagnosis was rarely made. Outcome was reported in 42 cases; 28 survived longer than 1 year, with 12 recurrences at the time of reporting, and 6 were alive with no evidence of disease at 4 years or longer. Improved prognosis requires diagnostic suspicion and early biopsy of unidentified lesions of the axilla or embryonic milk line.

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