A Case of the Retroperitoneal Malignant Schwannoma Secondary to Von Recklinghausen's Disease

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We report a case of the retroperitoneal malignant schwannoma secondary to von Recklinghausen' s disease. An 11-year-old man consulted our hospital with the symptoms of gait pain in the right thigh and claudication. He had been diagnosed as von Recklinghausen's disease three years prior to the consultation. On admission, he showed arophy of the right quadriceps muscle with an absent knee jerk on the right. CT (computed tomography) and MRI (magnetic resonanse imaging) disclosed a fist-sized tumor in the right retroperitoneal space arising from the right femoral nerve. As the diagnosis by open biopsy was malignant schwannoma, extirpation of the tumor including the surrounding tissues was carried out.At eight months after the operation, there is no recurrence nor metastasis of the tumor. In spite of weakness of the right quadriceps muscles, he can run and take part in sports activities.
西日本整形・災害外科学会の論文