Adenomyoepithelioma of the breast

Adenomyoepithelioma of the breast is a rare neoplasm that originates from the epithelial and myoepithelial cells of the mammary gland. It was first described by Hamperl in 1970 and is characterized by a dual population of cells.

Epidemiology

Adenomyoepithelioma of the breast is a rare tumor, accounting for less than 1% of all breast neoplasms. It can occur in both men and women, but is more common in women. The age of presentation varies widely, but most cases are diagnosed in the fifth to seventh decades of life.

Pathology

Adenomyoepithelioma of the breast is characterized by a dual population of epithelial cells and myoepithelial cells. The epithelial cells form glandular or ductal structures, while the myoepithelial cells are located in the periphery of these structures. The tumor can be benign, atypical, or malignant, depending on the presence of cellular atypia, mitotic activity, and invasive growth.

Clinical Presentation

Patients with adenomyoepithelioma of the breast typically present with a palpable mass. The tumor can be associated with pain, nipple discharge, or skin changes. However, many cases are asymptomatic and are discovered incidentally during routine mammography.

Diagnosis

The diagnosis of adenomyoepithelioma of the breast is based on histopathological examination of the tumor. Immunohistochemistry can be used to confirm the dual population of cells. The epithelial cells are typically positive for cytokeratin, while the myoepithelial cells are positive for smooth muscle actin and S-100 protein.

Treatment

The treatment of adenomyoepithelioma of the breast is primarily surgical. Depending on the size and location of the tumor, treatment options may include lumpectomy, wide local excision, or mastectomy. Adjuvant radiation therapy may be considered in cases with positive margins or high-risk features.

Prognosis

The prognosis of adenomyoepithelioma of the breast is generally good, especially for benign and atypical tumors. However, malignant tumors can metastasize to the lymph nodes and distant organs, and have a worse prognosis.

NIH genetic and rare disease info

• NIH info on Adenomyoepithelioma of the breast

Adenomyoepithelioma of the breast is a rare disease.