Giant malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1 : A case report

DOI 11 References
  • HIRAO Ayaka
    <i>Department of Dermatology, Kinki University School of Medicine</i>
  • OHISO Naoki
    <i>Department of Dermatology, Kinki University School of Medicine</i>
  • NARITA Tomohiko
    <i>Department of Dermatology, Kinki University School of Medicine</i>
  • YOSHIDA Masuki
    <i>Department of Dermatology, Kinki University School of Medicine</i>
  • YOSHINAGA Eiji
    <i>Department of Dermatology, Kinki University School of Medicine</i>
  • KAWARA Shigeru
    <i>Department of Dermatology, Kinki University School of Medicine</i>
  • KAWADA Akira
    <i>Department of Dermatology, Kinki University School of Medicine</i>

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Other Title
  • 神経線維腫症1型に生じた巨大悪性末梢神経鞘腫瘍の1例

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Abstract

A 40-year-old woman, who had NF1, visited us in May, 2008, with a giant skin tumor, 15cm in diameter, with a granulomatous, necrotic and ulcerative appearance on the left neck. The patient had noticed the development of the tumor 6 months before. Based on histopathological findings with positive S-100 and vimentin stainings, a diagnosis of malignant peripheral nerve sheath tumor was established. The patient was unable to undergo a complete excision, because the tumor had a rapid progression with multiple metastases on the lungs, pleura and liver. The patient succumbed to the disease about 5 months later. The poor prognosis seemed to be due to the giant size and the cervical location of the tumor. This case indicated that an early and precise diagnosis is required for malignant peripheral nerve sheath tumor to improve the prognosis.[Skin Cancer (Japan) 2010 ; 25 : 56-60]

Journal

  • Skin Cancer

    Skin Cancer 25 (1), 56-60, 2010

    The Japanese Skin Cancer Society

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