쇄골 상부에 발생한 악성 Triton 종양 1예

• 영문명: A Case of Malignant Triton Tumor on Supraclavicular Area
• 발행기관: 대한두경부종양학회
• 저자명: 임상호(Sang Ho Lim) 박희택(Hee Tack Park) 홍기환(Ki Hwan Hong)
• 간행물 정보: 『대한두경부종양학회지』제29권 제2호, 54~57쪽, 전체 3쪽
• 주제분류: 의약학 > 종양학
• 파일형태: PDF
• 발행일자: 2013.11.30

영문 초록

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, ex-tremities 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 behav-ior so prognosis of this rare and highly malignant tumor is poor and optimal treatment remains unclear. But mod-ern treatment consisted of radical excision and postoperative radiotherapy has improved the prognosis of such cases.

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키워드

Malignant peripheral nerve sheath tumor(MPNST) Malignant triton tumor Rhabdomyoblast 악성 신경초종 악성 triton 종양 횡문근아세포.