Malignant triton tumour of the nerve roots: Challenging the neuropreservation paradigm. Presentation of two cases and review.

Malignant triton tumour constitutes a rare and particularly aggressive subgroup within the so-called malignant peripheral nerve sheath tumours. It belongs to the group of sarcomas and is diagnosed more frequently and aggressively in patients with neurofibromatosis type I, with a peak age of around 30 years. They develop preferentially in medium and large nerves such as cranial nerves, plexuses and nerve roots. The 5-year survival rate is approximately 5-15% according to published series, compared to 34-52% in other malignant tumours of the nerve sheaths. Radical surgery with clear margins followed by radiotherapy for local control appears to offer the best results in terms of survival. We describe two cases treated at our centre and review the scientific literature, highlighting the role of the pathologist and neurosurgeon in their management.