小腸間膜原発多形型悪性線維性組織球腫の1例  [in Japanese] A CASE OF PLEOMORPHIC MALIGNANT FIBROUS HISTIOCYTOMA ORIGINATING FROM THE MESENTERY OF A SMALL INTESTINE  [in Japanese]

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Abstract

症例は49歳,女性.前医で子宮筋腫の診断で開腹.回腸間膜原発10cm径の充実性腫瘍で回腸間膜を含めて80cmの回腸切除術を施行.病理診断はc-kit(+~-),CD34(-),SMA(+),S-100(-),MIBI 10%で未分化多形肉腫(多形型MFH)と診断された.3度目の腹膜再発後に紹介され回盲部切除+S状結腸切除+腹膜移転巣9個切除し,残存空腸70cmであった.7カ月後腹膜再発したが,短腸症候群のため切除術ではなく化学療法の適応とした.IF0+VP16,CDDP+DXR 4クール後CRとなったがCR 14カ月継続後に腹膜再発した.腎機能低下のためプロトコールを変更し化学療法を継続したが,17カ月後に腸閉塞症状を発症し,在宅中心静脈栄養で全身管理後に全身状態悪化し永眠された.初回手術から6年後であった.腹部軟部悪性腫瘍は稀な疾患であり,手術に加えて化学療法を含めた集学的治療が必要であるが,整形外科,腫瘍内科医などと連携しつつ有効な治療法の検討が必要である.

A 49-year-old woman had a third recurrence of peritoneal dissemination related to an abdominal malignant soft tissue tumor. She had an ileocecal resection, partial sigmoidectomy, and extirpation of 9 disseminated lesions ; the remnant ileum was 70 cm long. Histological finding included undifferentiated pleomorphic sarcoma (pleomorphic malignant fibrous histiocytoma). Immunohistochemistry showed non-specific differentiation with negative CD34, S-100, desmin, and positive SMA. Seven months later, the patient had recurrent tumor related to dissemination. Since she already had short-bowel syndrome, surgery was not an option ; thus, chemotherapy was given She achieved complete remission (CR) after 4 courses of combined chemotherapy with IFO/VP16 and CDDP/DXR. After 14 months of CR, she had a recurrent disseminated tumor. She was given chemotherapies in reduced dosages due to renal dysfunction, but this was not effective, and she died 6 years after the initial operation. Abdominal malignant soft tissue tumors are rare, and complete resection combined with chemotherapy is necessary. Surgeons must not hesitate to consult a chemotherapy specialist.

Journal

  • Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)

    Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 72(5), 1287-1292, 2011-05-25

    Japan Surgical Association

References:  20

Codes

  • NII Article ID (NAID)
    10029099597
  • NII NACSIS-CAT ID (NCID)
    AA11189709
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    13452843
  • Data Source
    CJP  J-STAGE 
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