Tuberc Respir Dis.  1998 Jun;45(3):604-608. 10.4046/trd.1998.45.3.604.

A Case of Lung Cancer associated with von Recklinghausens Disease

Affiliations
  • 1Department of Internal Medicine, School of Medicine, Kyung-Hee Univeristy, Seoul, Korea.

Abstract

Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with characteristic cafeau-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 buliae 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.

Keyword

Von Recklinghausens disease; Lung cancer

MeSH Terms

Biopsy
Cafe-au-Lait Spots
Carcinoma, Squamous Cell
Cough
Genetic Diseases, Inborn
Humans
Iris
Lung Neoplasms*
Lung*
Lymph Nodes
Male
Middle Aged
Neurofibroma
Neurofibromatoses
Neurofibromatosis 1*
Neurofibrosarcoma
Radiography
Skin
Sputum
Thorax
Tomography, X-Ray Computed
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