Clinical assessment of the thoracic surgical diseases associated with von Recklinghausen's disease

摘要

Type I neurofibromatosis (NF-I), also referred to as von Recklinghausen's disease, is an autosomal dominant disease characterized by neurofibromas and abnormal cutaneous pigmentation (cafe-au-lait spot). We studied retrospectively the 8 cases operated in our hospital between January 1979 to December 2002, which were complicated with von Recklinghausen's disease and a thoracic surgical disease. The patients were 6 males and 2 females and the age from 16 to 70 (the averaged age was 36 +/- 22). The thoracic diseases were consist of mediastinal tumors (n = 7) and esophageal cancer (n = 1). The operative procedures were tumorectomy (n = 6), subtotal esophagectomy (n = 1), and pericardial cystectomy (n = 1). The mediastinal tumors were neurofibroma (n = 3), malignant schawannoma (n = 1), ganglioneurinoma (n = 2), and pericardial cyst (n = 1). Malignant neoplasms were recognized in 2 cases (25%). The postoperative survival was 10 months for malignant schwannoma, and 8 months for esophageal cancer, and the others were alive. For 1 case of neurofibromas, there was observed to be the reoperated one after the postoperative recurrence. von Recklinghausen's disease are apt to be complicated with thoracic surgical neoplasms, it should be required a careful and systemic exploration especially for malignant neoplasms.