[Autopsy case of von Recklinghausen's disease associated with lung cancer, gastrointestinal stromal tumor of the stomach, and duodenal carcinoid tumor].
A 58-year-old man with von Recklinghausen's disease was admitted for further investigation of right chest pain. Chest X-ray revealed multiple emphysematous bullae in both lungs and a tumor shadow in the right upper lobe. Bronchofiberscopy was performed, but an adequate specimen was not obtained. The tumor was diagnosed as a non-small-cell lung cancer with direct invasion to the adjacent rib. Although chemotherapy and radiotherapy resulted in decrease in tumor size, the tumor subsequently increased in size and the patient died 14 months after the first admission. Autopsy revealed multiple emphysematous bullae, poorly differentiated adenosquamous cell carcinoma of the lung, gastrointestinal stromal tumor of the stomach, and duodenal carcinoid tumor. This case suggests the possibility that von Recklinghausen's disease associated with emphysematous bullae is a risk factor for lung cancer. It has also been suggested that the genetic abnormality responsible for von Recklinghausen's disease increases the risk for various types of malignancy. Although von Recklinghausen's disease is reportedly associated with various malignant tumors, it is quite rare for von Recklinghausen's disease to be associated with triple non-neurogenic tumors. Careful observation is mandatory for patients with von Recklinghausen's disease.
Satoh
Miki
M
Department of Respiratory Medicine, National Hospital Organization, Hakodate Hospital.
Wakabayashi
Osamu
O
Araya
Yoshikazu
Y
Jinushi
Eisei
E
Yoshida
Fumiaki
F
D001344:Autopsy; D002276:Carcinoid Tumor / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D018196:Carcinoma, Adenosquamous / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D004379:Duodenal Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D017809:Fatal Outcome; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D011656:Pulmonary Emphysema / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D012307:Risk Factors