Mandibular malignant peripheral nerve sheath tumour in a patient with a history of radiotherapy for cerebellopontine angle ganglioneuroma.
H W Zhang, R Xue, H X Miao et al.
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Case in brief
A patient previously underwent subtotal resection and radiotherapy for a ganglioneuroma in the cerebellopontine angle region and later developed a malignant peripheral nerve sheath tumour (MPNST) in the mandible. The report focuses on whether the mandibular tumour represented secondary disease or a de-novo MPNST. The abstract does not provide further clinical, imaging, pathological, or treatment details, or describe the outcome.
What made this case unusual
The mandibular MPNST occurred in a patient with prior radiotherapy for a cerebellopontine angle ganglioneuroma, raising the diagnostic question of secondary versus de-novo disease.
Diagnostic / clinical pearl
When an MPNST arises after treatment of another tumour, consider whether it is secondary or de novo; the prior radiotherapy and tumour history are relevant to that assessment.
Why it matters
The authors emphasize close clinical and radiologic follow-up for patients with high-risk ganglioneuroma, particularly after incomplete resection and radiotherapy.