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KMID : 0356820130290020054
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Korean Journal of Head & Neck Oncology
2013 Volume.29 No. 2 p.54 ~ p.57
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A Case of Malignant Triton Tumor on Supraclavicular Area
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Lim Sang-Ho
Park Hee-Tack
Hong Ki-Hwan
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Abstract
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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.
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KEYWORD
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Malignant peripheral nerve sheath tumor(MPNST), Malignant triton tumor, Rhabdomyoblast
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