Abstract
Recklinghausen's disease is an inherited condition in which multiple neurofibromas are associated with spots of pigmentation of the skin, so-called Cafe au lait spots and often with malignant neoplasm. A case of advanced breast cancer with R's disease is reported.
A 46-year-old female was admitted to our hospital because of a left breast tumor with serous discharge. Metastasis to axillary lymph nodes, invasion to the chest wall and multiple metastasis of the bone were noted. The patient was diagnosed as having an advanced breast cancer (T4cN1bM1 Stage IV). After chemoendocrine therapy (per os), radical mastectomy and bilateral oophorectomy were performed.
In the literature, breast cancers with R's disease are commonly in an advanced stage more than stage II. The reason why this disease is found in an advanced stage, seems to lie in the existence of diffuse neurofibromas of the whole body skin. This case seems a good illustration suggesting the effectiveness of preoperative chemoendocrine therapy for advanced breast cancers.