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Outline. Neurofibromatosis type 1 (NF1)MPNST Epidemiology/symptoms/risk factorsGrowth control pathwaysPreclinical ModelsCurrent treatments for MPNSTOther molecular targetsFuture Directions. NF1: Neurofibromatosis type 1. Familial tumor predisposition syndromeGermline mutations in the NF1 tum
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1. Treatment Strategies for MPNSTs Angela C. Hirbe
Hematology Oncology Grand Rounds
5/4/2012
No Financial Disclosures
2. Outline Neurofibromatosis type 1 (NF1)
MPNST
Epidemiology/symptoms/risk factors
Growth control pathways
Preclinical Models
Current treatments for MPNST
Other molecular targets
Future Directions
3. NF1: Neurofibromatosis type 1 Familial tumor predisposition syndrome
Germline mutations in the NF1 tumor suppressor gene: 17q11.2
>85% cutaneous neurofibromas
33% plexiform neurofibromas
3-5% develop MPNSTs -benign and malignant tumors of the peripheral and central nervous system-benign and malignant tumors of the peripheral and central nervous system
4. NF1: Neurofibromatosis type 1