쇄골 상부에 발생한 악성 Triton 종양 1예

A Case of Malignant Triton Tumor on Supraclavicular Area

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.