von Recklinghausen's disease and malignant changes in three patients.
Three Japanese patients with von Recklinghausen's disease and malignant changes were studied. The primary lesions of malignant changes were the chest wall in Case 1, the buttock in Case 2, and the neck in Case 3. After excision of the tumors, there were local recurrences in Cases 1 and 2. Distant metastases developed during treatment in these patients. One of the patients (Case 1) died 21 months after the initial diagnosis of malignancy. Multidisciplinary treatment was of some value in suppressing local recurrences and pulmonary metastases in Case 2. Pulmonary metastases were evident at the time of admission to our hospital in Case 3. Although the primary and metastatic tumors were removed, the patient died 8 months after the initial diagnosis of malignancy. We conclude that careful follow-up should be required in patients with von Recklinghausen's disease because of the possibility of malignant changes. When there is a suspicion of malignancy, an extended en-block excision must be performed.
D000293:Adolescent; D000328:Adult; D002081:Buttocks; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008175:Lung Neoplasms / Q000473:pathology* / Q000601:surgery; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery