A CASE OF BREAST CANCER IN VON RECKLINGHAUSEN NEUROFIBROMATOSIS

Abstract

von Recklinghausen's disease is often associated with neural malignant tumor, but the association of epithelial malignant tumor is rarely reported. This paper describes a case of breast cancer associated with von Recklinghausen's disease. A 54-year-old woman was seen at the hospital becuse of a left breasttumor. Cafe au lait spots were noticed on the whole body skin. Fine needle aspiration cytology revealed breast cancer. Modified radical mastectomy (Br+Ic+Ax) was performed. Histopathologically, the breast tumor was papillo-tubular adenocarcinoma, and tnm clasification was t2n2m0 in stage III. Both estrogen and progesterone receptor were negative by EIA method. But immunohistochemically, positive reactions against anti-bodies of cerbB-2 and p53 protein were noted. We could not find any reports association of breast cancer and p53 protein in case of von Recklinghausen's disease, and thought that these findings were interesting. Adjuvant chemo-endocrine therapy was added, but 5 months after the operation recurrence was noticed. The patient died 3 years after the operation. Fifty-four cases of breast cancer with von Recklinghausen's disease have been reported in the Japanese literature. Most cases are in stage II or more advanced stage that might be derived from delayed diagnosis due to skin lesion. Careful observation is necessary for early detection of breast tumor in case of von Recklinghausen's disease.