1. Term includes malignant schwannoma and neurogenic sarcoma
2. In NF-1 MPNST's can occur (2-16 % of patients with NF-1 develop one over their lifetime), or they can occur sporadically, or post-radiation.
3. Occassionally a previously existing benign neurofibroma can transform and become malignant.
4. They are located in the same areas as solitary and plexiform neurofibromas.
5. Pain and nerve dysfunction are greater than in benign tumors.
6. The tumors can become large before being detected.
7. Therapy involves amputation, radiation, chemotherapy.
8. The prognosis is poor with local recurrences and metastases to the lungs.
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3. Occassionally a previously existing benign neurofibroma can transform and become malignant.
It should be noted it's benign plexiform neurofibromas that can undergo malignant degeneration. Solitary neurofibromas rarely turns. Some of the literature state that MPNST in individual with NF1 almost always arises from previously existing plexiform neurofibromas. Sporadic MPNST is de novo.
5. Pain and nerve dysfunction are greater than in benign tumors.
Pain and dysfunction can be greater in MPNST but benign tumors can wreak havoc just as much and sometimes more than MPNSTs.
7. Therapy involves amputation, radiation, chemotherapy.
Amputation is only an option if the tumor is located in the limbs. In the cases where it's in the trunk/retroperitoneal/ect just trying to get wide-margins could be difficult.
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