Korean Journal of Head & Neck Oncology (대한두경부종양학회지)

The Korean Society for Head and Neck Oncology (대한두경부종양학회)
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A Case of Malignant Triton Tumor on Supraclavicular Area

쇄골 상부에 발생한 악성 Triton 종양 1예

  • Lim, Sang Ho (Department of Otolaryngology Head & Neck Surgery, School of Medicine, Chonbuk National University) ;
  • Park, Hee Tack (Department of Otolaryngology Head & Neck Surgery, School of Medicine, Chonbuk National University) ;
  • Hong, Ki Hwan (Department of Otolaryngology Head & Neck Surgery, School of Medicine, Chonbuk National University)
  • 임상호 (전북대학교 의학전문대학원 이비인후-두경부외과학교실) ;
  • 박희택 (전북대학교 의학전문대학원 이비인후-두경부외과학교실) ;
  • 홍기환 (전북대학교 의학전문대학원 이비인후-두경부외과학교실)
  • Received : 2013.06.10
  • Accepted : 2013.08.06
  • Published : 2013.11.30
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Abstract

Malignant triton tumor(MTT) is a rare type of malignant peripheral nerve sheath tumor(MPNST) with focal rhabdomyoblastic differentiation. MTT constitutes about 5% of all MPNSTs and described the first case of a MTT in a patient with Von Recklinghausen disease by Masson in 1932. MTT is commonly seen in the head, neck, extremities and trunk. It can occur in sporadic form or over a setting of neurofibromatosis-1(NF-1). The diagnosis can be confirmed based on morphologic grounds supported by an immunostain such as S-100 protein. Desmin, myo-D1 and myogenin are immunostains positive for rhabdomyoblasts. MTT has an aggressive biological behavior so prognosis of this rare and highly malignant tumor is poor and optimal treatment remains unclear. But modern treatment consisted of radical excision and postoperative radiotherapy has improved the prognosis of such cases.

Keywords

References

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