Challenges in the Management of Malignant Adenomioepithelioma of the Breast: a Case Report and Review of the Literatures

Abstract Background: Malignant adenomyeloblastoma is a rare tumor, with no guidelines available for its treatment. Case presentation: We discuss a case of malignant adenomyeloblastoma of the right breast in a 59-year-old woman. The patient underwent a lumpectomy, and pathological diagnosis of malignant adenomyeloblastoma was distinct from the preoperative needle-biopsy diagnosis of intraductal papilloma. The patient underwent mastectomy and chemotherapy immediately. However, local recurrence was occurred 11 months after the initial operation. We summarize the characteristics of patients with malignant adenomyeloblastoma in PubMed and Web of Science databases, and share our experience and discoveries. Statistics included the univariate and multivariable Cox proportional hazards models. Overall, 34 patients were analyzed. Conclusions: At diagnosis, the median age was 59.3 years and the median tumor size was 30.0 mm. The most common initial surgery was breast-conserving surgery (50%), and a proportion of patients underwent axillary lymph node dissection (32%). Age, max diameter, and axillary status were associated with overall survival. Complete surgical excision with adequate margins is the most important treatment. Patients may not benefit from remedial adjuvant therapy, but may benefit from prompt postoperative adjuvant therapy. Multidisciplinary cooperation and follow-up are essential for the management of these patients. Our data would provide novel insights into a rare tumor that might avoid over- or under-treatment of the patients.