A 37-year-old female was admitted with worsening neurologic function. On arrival from an outside hospital, the patient was obtunded and intubated. Magnetic resonance imaging of the brain revealed nodular enhancement of the leptomeninges, intracranial osteolytic lesions, and diffuse vasogenic edema causing mass effect. Imaging of the thoracic spine revealed pathologic compression fractures of 4 thoracic vertebrae. On review of the patient's electronic medical record, the patient had previously received treatment for secondary syphilis with intramuscular benzathine penicillin G. Surgical biopsies of the frontal bone and dura showed diffuse, chronic inflammation while a biopsy of the adjacent brain parenchyma revealed replicating spirochetes. The patient was subsequently prescribed dexamethasone and benzathine penicillin G. She regained neurologic function but later signed out against medical advice without completing her treatment regimen.
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.
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