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  [in Japanese] RETROPERITOINEAL MALIGNANT SCHWANNOMA IN A PATIENT WITH VON RECKLINGHAUSEN'S DISEASE : REPORT OF A CASE  [in Japanese]

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後腹膜に原発する神経鞘腫は非常に稀な疾患である.われわれは, von Recklinghausen病に合併した後腹膜悪性神経鞘腫の1切除例を経験したので本邦報告例の文献的考察を加えて報告する.<br> 症例は17歳の男性で,主訴は腹部腫瘤・下肢痛であった. 1992年より左下腹部腫瘤・左下肢痛が出現し,増悪傾向を認めたため精査加療目的で1994年10月21日当科に入院した. CT検査および超音波検査にて左後腹膜腔に径約13cmの腫瘍を認め,神経線維腫の悪性化を疑い1994年11月14日摘出術を施行した.摘出した腫瘍は被膜に包まれた15×11×10cm,重量790gの黄色充実性の腫瘍であった.病理組織学的には,悪性神経鞘腫(悪性末梢性神経性腫瘍)であった. von Recklinghausen病に合併する後腹膜悪性神経鞘腫は早期発見が難しく予後不良である.よって, von Recklinghausen病の患者には経過観察のための定期的な画像診断検査が必要であると考えられる.

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.


  • The journal of the Japanese Practical Surgeon Society

    The journal of the Japanese Practical Surgeon Society 57(1), 177-183, 1996-01-25

    Japan Surgical Association

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Cited by:  3


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