Malignant triton tumor of the anterior mediastinum: a rare tumor in a rare location

Malignant triton tumors are an extremely aggressive form of malignant peripheral nerve sheath tumor that display rhabdomyosarcomatous features. While these tumors are extremely rare, they have a much higher incidence in patients with neurofibromatosis-1. We present a case of a 64-year-old male with neurofibromatosis-1 who presented to the hospital with sudden worsening of shortness of breath and dysphagia to solids. Radiological examination revealed a large mass in the anterior mediastinum causing significant narrowing and displacement of the upper trachea and esophagus. Biopsy of the mass, done by interventional radiology, demonstrated features of an MTT. The mass was subsequently resected but without confirmation of tumor-free margins and the patient underwent adjuvant radiation therapy. Repeat radiological examination approximately four months later revealed growing malignancy and new metastases, which eventually contributed to the patient's death seven months after his presentation to the hospital.