Recurrent sporadic malignant triton tumor in the carotid sheath in the absence of neurofibromatosis

Abstract

Malignant Triton Tumors (MTTs) are a rare and aggressive subtype of malignant peripheral nerve sheath tumors (MPNSTs), often associated with neurofibromatosis type 1. This case report describes a unique instance of recurrent sporadic MTT within the carotid sheath in a 33-year-old male without any personal or familial history of neurofibromatosis. The patient initially presented with a biopsy-confirmed MTT in the right neck, involving the carotid body and brachial plexus, and underwent partial resection, radiation therapy, and chemotherapy. Six months later, the patient presented with recurrent MTT, and subsequently underwent radical tumor resection, segmental right carotid artery resection, and deep femoral vein interposition. Recovery was complicated by hematoma formation, and the patient developed vocal fold paralysis and a left vocal fold cyst, necessitating further surgeries. Yearly follow-ups for 8 years revealed no recurrence. This case emphasizes the importance of comprehensive patient evaluation, including clinical history, imaging, and biopsy findings, for accurate diagnosis and prompt surgical intervention in managing such rare and aggressive tumors. Further research is needed to identify novel therapies and improve survival rates for patients with MTTs.