A Case of Lung Cancer associated with von Recklinghausen's Disease

Von Recklinghausen 병에 동반된 폐암 1예

Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with characteristic cafe-au-lait spots of skin and multiple neurofibromatosis. It is complicated by malignancies, which in most cases is neurofibrosarcoma. The development of lung cancer in von Recklinghausen's disease is rare. A 61-year-old male was admitted for cough and sputum for 20 days. He had multiple cafe-au-lait spots and subcutaneous neurofibromas in whole body area and Lisch nodules in both iris and he had been diagnosed von Recklinghausen's disease 35 years ago. Chest radiography showed emphysematous bullae in both upper lung field and mass in right upper lung field. Chest CT scan revealed subcarinal lymph node enlargement. Bronchoscopic biopsy was done in mass in superior segment of right lower lobe and the results showed squamous cell carcinoma. The presence of von Recklinghausen's disease and lung cancer are noteworthy.

Von Recklinghausen병에 동반되는 악성종양은 주로 신경섬유육종이지만 드물게는 폐암이 동반되는 경우가 보고되고 있고 ras 유전자가 양 질환의 발생에 영향이 있음이 알려져 있어 앞으로 이에 관한 연구가 필요하리라 생각된다.