Juvenile secretory carcinoma of the breast

Juvenile secretory carcinoma of the breast, a rare tumor in infants and children, has an unusual histological appearance and clinical behavior. Isolated case reports and small series have appeared since this lesion was first described by McDivitt and Stewart in 1966. Our case of a 3-year-old boy with axillary metastasis and 17 cases in children, from the literature, provide the basis of this review. Of the 18 patients, there were three boys and 15 girls. Their ages ranged from 3 to 17 years (mean 9.8 years). All patients presented with an asymptomatic mass in the breast. None had nodes which were clinically involved. Eight patients had excisional biopsy only. Two patients had quadrantectomy. Four had simple mastectomy; one of whom received postoperative axillary irradiation, and one of whom had axillary nodal sampling (our patient). One patient had a modified radical mastectomy and three had radical mastectomy. Two of eight children who had excisional biopsy alone developed local recurrences. In the first patient, the recurrences occurred at 2 and 8 years following initial therapy. In the second, they occurred at 4 and 21 years. Axillary nodal metastases were found in three of the six patients in whom nodal biopsies were performed. In only one patient were estrogen receptors measured and they were negative. No deaths have been reported in children during a follow-up period ranging from 0 to 22 years (mean 6.5 years). Secretory carcinoma of the breast in this group of patients appears to be a slow growing, locally recurring malignancy. Adults with histologically similar tumors also have a good prognosis. Excisional biopsy is probably inadequate therapy. We recommend simple mastectomy with adequate margins and sampling or excision of the axillary contents. There is no evidence that adjunctive radiation or chemotherapy is necessary.