von Recklinghausen病に合併した巨大後腹膜神経線維腫の1例

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  • A CASE OF RETOROPERITONEAL NEUROFIBROMA IN VON RECKLINGHAUSEN'S DISEASE

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A 25-year-old man was seen at the hospital because of rectal bleeding. On physical examination the patient showed cáfe au lait spots scattered over the entire body. A large mass was palpated in the right lower abdomen. CT and MRI confirmed the presence of a retroperitoneal mass (about 15×15cm) between the right psoas muscle and corpus vertebrae. The tumor was diagnosed as neurogenic retroperitoneal tumor in von Recklinghausen's diasese, and an operation was performed. At surgery, a large tumor was found occupying the pelvic space, originating from the right L4, 5 nerve roots. The tumor was of child-head size, with elastically hard, almost smooth surface, and invasion to the surrounding organs was not recognized. On pathological examination this was shown to be a benign neurofibroma. Retroperitoneal neurofibroma in von Recklinghausen's disease is a rare entity, and only 21 such cases have been reported in Japan. Almost of these cases were malignant, and the recurrence was recognized soon after operation. The postoperative surveillance must be performed, because there are some reports that the tumor diagnosed benign at the postoperative pathological examination have eventually had a malignant potential.

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