Secretory breast carcinoma in a seven-year-old girl: case report and review of the literature

Secretory breast carcinoma is an extremely rare entity initially described in children and in adolescents, but a relatively frequent number of cases has been reported in adults. It is often associated with good prognosis. The present case refers to a seven-year-old girl with a progressively growing tumor in the left breast. Clinical examination identified a hard, mobile, non-painful, subareolar nodule with regular margins; further macroscopy of surgical specimen showed a smooth surface and 1.3 cm in diameter. The final diagnosis of malignancy led to mastectomy and intra-operatively negative sentinel lymph node assessment. Immunohistochemical profile included a strong positive stain for cytokeratins (AE1/AE3) and TP53, and estrogen-receptor and progesterone-receptor and Her-2/neu protein expression negative; and MIB-1 (Ki-67) labeling index was 10%. Further follow-up examinations have shown no evidence of distant metastases, with normal development of secondary sexual characteristics (pubarche, right thelarche, and menarche occurring in July, 2009). Seven years later, the teenager still does not have the disease and waits reconstructive plastic surgery of the left breast.