Recklinghausen disease causing malignant neurilemmoma with different malignancy after10 years interval.

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We report a case of a 26-year-old man suffering von Recklinghausen's disease associated with multiple malignant shcwannomas of various malignant potency. In 1986, two malignant tumors over 5 cm in diameter in the postperitoneal space were resected. In 1995, a rapidly growing tumor appeared on his left thigh and reached an adult head size. Besides, CT scan revealed two fistsized tumors in the abdominal cavity. The left thigh was amputated at the level of hip joint. Four months later, multiple metastatic tumors appeared in the lung and the patient died 10 months after the amputation due to rapid enlargement of these metastatic tumors. Four tumors, developed in the post-peritoneal space and the abdominal cavity in 1986 and 1995, grew slowly and showed histologically a low grade of malignancy with low mitotic rates, whereas the tumor on the thigh and metastatic lung tumors, both of which rapidly increased in size, showed frequent mitotic figures and high cellularity. Thus, histologic grades of malignancies well correlated with clinical behaviors of the tumors in this patient.
日本皮膚悪性腫瘍学会の論文