[Incidence of the degeneration of cutaneous nerve tumors in Recklinghausen's disease].

A retrospective study was conducted in 85 patients with clinically certain von Recklinghausen's neurofibromatosis seen between 1972 and 1982. A similar study was conducted in 105 patients without neurofibromatosis observed during the same period who underwent excision of a cutaneous neurofibroma or schwannoma. Thirty-nine of the patients with neurofibromatosis never had surgical treatment for a nervous skin tumour; 46 had one or several tumours excised for diagnostic purposes or for cosmetic reasons. More than 300 tumours had been removed in these 46 patients. The post-operative follow-up period is now at least 2 years for all patients, 5 years for 85 p. 100 of them and more than 10 years for one-third. Only one case of malignant nervous skin tumour was observed in a patient who never had surgical treatment; it was a neurofibrosarcoma which resulted in death within two years. Two other deaths related to neurofibromatosis were recorded: one after surgical treatment of a duodenal schwannoma, the other due to septicaemia after infection of ulcers on a giant plexiform neurofibroma. Three other deaths unrelated to neurofibromatosis occurred. Local relapses without suspicion of malignancy were observed in 8 of the 46 surgical patients; 3 cases concerned large plexiform neurofibromas of the face; excision was histologically incomplete in most cases with or without relapse. No case of malignancy was recorded in the follow-up of the 105 patients without neurofibromatosis but with nervous skin tumours.(ABSTRACT TRUNCATED AT 250 WORDS)