RETROPERITONEAL MALIGNANT SCHWANNOMA IN A PATIENT WITH VON RECKLINGHAUSEN'S DISEASE -REPORT OF A CASE-

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  • UEDA Junji
    Department of Surgery I, Faculty of Medicine, Kyushu University
  • OOHATA Yoshihiro
    Department of Surgery I, Faculty of Medicine, Kyushu University
  • MIBU Ryuichi
    Department of Surgery I, Faculty of Medicine, Kyushu University
  • TANAKA Masao
    Department of Surgery I, Faculty of Medicine, Kyushu University
  • HAMADA Manabu
    Department of Pathology II, Faculty of Medicine, Kyushu University

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  • von Recklinghausen病に合併した後腹膜悪性神経鞘腫(末梢性神経性腫瘍)の1例

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Abstract

Schwannomas arising in the retroperitoneum are very rare. A case of malignant retroperitoneal schwannoma in a patient with von Recklinghausen's disease is reported. A 17-year-old man with von Recklinghausen's disease was admitted to the hospital in 1994 because of a left lower abdominal mass and pain of he left thigh which started in 1992 and were gradually getting worse. On physical examination, an elastic hard, tender mass with smooth suface, about 13 cm in diameter, was palpated in the left lower abdomen. Computed tomography, ultrasonography and angiography showed a solid and mild hypervascular tumor in the retroperitoneal space. The tumor was extirpated completely. The extirpated speciemen was a 15×11×10cm yellowish solid tumor and 790 g in weight. Histopathological diagnosis was malignant schwannoma. Retroperitoneal malignant schwannoma is hardly diagnosed in an early stage and it's prognosis is relatively poor especially with von Recklinghausen's disease. The patients with von Recklinghausen's disease should be followed by a periodic check-up including computed tomography and ultrasonography for early detection of such a neoplasm.

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