Malignant triton tumor in the thoracic spine

We present a 15-year-old patient diagnosed with peripheral neurofibromatosi s (NF-1), who was admitted with paraparesis caused by a large intrathoracic tumor with an intracanalicular component that affected the spinal cord. Af ter surgery his condition improved, but a year later he suffered a relapse and died. Histologically the tumor was diagnosed as malignant with neurogen ic and myogenic differentiation ("malignant triton tumor"). Malignant trito n tumors (MTT) are infrequent; those found in the head and neck and the upp er or lower extremities have better prognosis than those in the retroperito neum, buttock, or trunk. It is not clear whether this variation is due to a difference in tumor grade, stage, or resectability, or whether it is a con sequence of therapy.