1997 Volume 58 Issue 6 Pages 1396-1400
It has been reported that von Recklinghausen's disease is often associated with nonepithelial malignant tumors such as neurofibrosarcoma, but an association of epithelial malignant tumor is relatively uncommon. In this paper a very rare case of synchronous double cancer of the breast and colon with von Recklinghausen's disease is reported. A 60-year-old woman was admitted to the hospital because of a right large breast mass with massive bleeding. She also had cafe au lait spots and neurofibromas all over the body. Simple mastectomy was carried out under a suspicion of neurofibrosarcoma arising in the breast region. But postoperative diagnosis was made as breast cancer, which was mucinous carcinoma histologically. After the mastectomy she underwent radiologic and endoscopic examination of the colon, which demonstrated a type 2 cancer in the sigmoid colon. Sigmoidectomy with lymph node dissection was performed. Pathological examination showed a moderately differentiated adenocarcinoma with regional lymph node metastasis. Postoperative course was uneventful and the patient is now being followed up. Von Recklinghausen's disease itself is a benign disease, and hence, an associated malignant disease turns out a determinant for the prognosis. Systemic and careful exploration is essential for the patients with von Recklinghausen's disease in order to detect a possibly associated cancer or sarcoma in an early stage.
