[A case of lung cancer in a patient with von Recklinghausen's disease]

A 57-year-old man was admitted to our hospital for further investigation of an abnormal shadow in his chest X-ray. He had cafe-au-lait spots and multiple subcutaneous neurofibromas and was diagnosed as having von Recklinghausen's disease. Bronchofiberscopy was performed, but an adequate specimen was not obtained. Therefore, percutaneous needle biopsy was performed, and the specimen showed poorly-differentiated adenocarcinoma. The left subclavian artery was deviated on aortography, therefore, neoadjuvant chemotherapy with CDDP & VDS was performed. He subsequently underwent left upper lobectomy. Pathologically, the tumor cell showed necrosis and scarring. Including this case, there have been 11 reports of von Recklinghausen's disease associated with lung cancer in the Japanese literature. Adenocarcinoma was observed in 72.9% of cases, and poorly-differentiated tumor was observed in 7 out of 8 patients with distinct tumor differentiation.