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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Malignant peripheral nerve sheath tumour is a sarcoma that grows from the covering of a nerve, most often in people with neurofibromatosis type 1 when a benign plexiform neurofibroma turns malignant. Surgery with radiotherapy is the only cure; chemotherapy with doxorubicin and ifosfamide shrinks some tumours, and drugs targeting the tumour's lost NF1 and PRC2 brakes are in trials.
Malignant peripheral nerve sheath tumour arises from Schwann cell lineage in a peripheral nerve or a pre-existing neurofibroma, sporadically, after radiotherapy or, in half of cases, in neurofibromatosis type 1, where atypical neurofibromatous neoplasms of uncertain biological potential are the recognised precursor. Its genome shows loss of NF1, then CDKN2A, then the polycomb repressive complex 2 components SUZ12 or EED, producing global loss of H3K27 trimethylation that pathologists now use as a diagnostic marker, alongside TP53 loss in high-grade tumours.
Treatment of localised disease is wide resection with radiotherapy, which controls local disease but does not prevent metastasis; the tumours are large, deep and often involve major nerves and the spine, so margins are frequently compromised. Chemotherapy activity is modest: the SARC006 phase 2 trial of neoadjuvant doxorubicin-ifosfamide followed by ifosfamide-etoposide produced responses in a minority, more often in sporadic than NF1-associated tumours, and adjuvant chemotherapy is offered to fit patients with high-grade disease on the general sarcoma evidence.
| Setting | Approach | Guideline |
|---|---|---|
| Localised | Wide resection with preoperative or postoperative radiotherapy; nerve sacrifice and reconstruction as needed; consider neoadjuvant or adjuvant anthracycline-ifosfamide for large high-grade tumours. | not mapped |
| Advanced | Doxorubicin plus ifosfamide (EORTC 62012), then ifosfamide-etoposide or trials; response rates lower in NF1-associated tumours. | not mapped |
| Neurofibromatosis type 1 surveillance | Whole-body MRI and FDG-PET for growing or painful plexiform neurofibromas; biopsy of atypical lesions; MEK inhibitors for symptomatic plexiform neurofibromas. | not mapped |