Oncology (WM) Flashcards
What’s the mechanism of action of Temozolamide?
Temozolamide is an oral alkylating agent
The cytotoxic effect is due to adding an alkyl group (methyl is the smallest alkyl group) to DNA primarily at the O6 and N7 positions on guanine.
With reference to Temozolamide, what’s the significance of MGMT?
Temozolamide adds an alkyl group to DNA, most cells repair this damage using the protein O6-methylguanine-DNA methyltransferase. Temozolamide is more effective against tumours with subnormal MGMT activity. MGMT promotor methylation reduces MGMT activty.
What’s the difference in the relationship to the nerve between neurofibromas and schwannomas?
Schwannomas displace axons (centrifugal)
Neurofibrommas are unencapsulated and engulf the nerve of origin (centripetal)
What’s the incidence of NF1 and NF2?
1: 3000 live births (NF1)
1: 40,000 live births (NF2)
What’s are the gene product in NF1 and NF2?
neurofibromin (NF1)
schwannomin (NF2)
Where’s the gene locus for NF1 and NF2?
NF1 ch17
NF2 ch22
What are the 7 diagnostic features of NF1? (2 of more needed for diagnosis)
3 skin: >6 cafe au lait spots >2 neurofibromas (or one plexiform neurofibroma) Freckling (axillary or intertriginous) 3 head: >2 lisch nodules (iris hamartomas) Osseous abnomality (sphenoid dysplasia or thinning of long bone cortex) Optic pathway glioma: 1 other: First degree relative
What’s the function of neurofibromin?
It’s a negative regulator of the RAS oncogene
What are the diagnostic criteria for NF2?
Bilateral VS
OR
A combination of unilateral VS +/ first degree relative +/- 2 of meningioma, schwannoma, glioma, posterior subcapsular lens opacity, cortical wedge cataract
OR
multiple meningioma and two of the following
What is the effect of pregnancy of 8th nerve tumours?
It can accelerate the rate of growth
What is the classic clinical triad of tuberous sclerosis?
Seizures
Mental retardation
Facial angiofibromas
What’s the incidence of tuberous sclerosis?
1:6000 live births
Give a differential for benign skull lesions
Sclerotic:
Fibrous dysplasia
Osteoma
Intraosseous meningioma
Lytic: Epidermoid cyst Dermoid cyst Calvarial sarcoid Langerhans cell histiocytosis
Not easily classified above:
Ossifying fibroma
Paget disease
Haemangioma (osseous venous vascular malformation)
Give a differential for malignant skull lesions
Primary
Osteosarcoma
Chordoma
Chondrosarcoma
Secondary
Multiple myeloma
Mets
(All lytic except sclerotic mets)
What’s survival in GBM?
In the stummer study 5-ALA resection group
Total resection ~16.5 months
Partial resection ~12