Superficial Angiomyxoma of the Nipple in a Japanese Woman: A Case Report and Review of Literature

Int J Surg Pathol. 2020 Sep;28(6):683-687. doi: 10.1177/1066896920913116. Epub 2020 Mar 24.


Superficial angiomyxoma is a rare, benign, multilobulated cutaneous tumor composed of stellate and spindle cells, a prominent myxoid matrix, and numerous blood vessels. Superficial angiomyxoma may be indistinguishable from cutaneous lesions of the Carney complex, although superficial angiomyxoma can occur independently of the complex. In this article, we present the case of a 39-year-old Japanese woman with a 40 × 30 mm, focally ulcerated, polypoid superficial angiomyxoma on the left nipple without any evidence of Carney complex. The development of superficial angiomyxoma on the nipples in a patient without the Carney complex is extremely rare. Indeed, only 3 cases of superficial angiomyxoma arising on the nipple have been reported to date, and this is the first such report in Japan. In such cases, the majority of superficial angiomyxoma of the nipples develop in premenopausal women. The possibility of superficial angiomyxoma should be considered for all polypoid nipple lesion, particularly in premenopausal women, and complete excision with follow-up observation should be performed.

Keywords: Carney complex; angiomyxoma; immunohistochemistry; nipple; premenopausal women.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Asian People
  • Breast Neoplasms / pathology*
  • Female
  • Humans
  • Myxoma / pathology*
  • Nipples / pathology*