We report herein a rare case of syringomatous adenoma (SA) of the nipp
le, Only 22 cases of SA of the nipple have been documented in the worl
d literature, and to the best of our knowledge this is the first case
to be reported in Japan. The patient was a 68-year-old woman who prese
nted with a painful mass in the right subareolar region of over 15 yea
rs' duration, Clinical examination including needle biopsy indicated a
high possibility of carcinoma; however, the final histopathological d
iagnosis after mastectomy proved to be SA. SA is a benign locally infi
ltrating neoplasm of the nipple which shares many clinicopathological
features with adenoma of the nipple. Both lesions sometimes show decep
tive clinicopathological findings, causing then to be easily confused,
vith carcinomas. However, SA can be distinguished by its unique histol
ogical appearance resembling sweat duct, or syringomatous, tumors and
locally infiltrating growth, which is probably responsible for its mis
leading clinical findings and higher rate of recurrence. Surgeons shou
ld be aware of the possibility of diagnosing this extremely rare tumor
, and the appropriate treatment.