Hallmarks of Inherited Cancer syndromes

Question 1

Cancer Risk Factors

Answer 1

Age, lifestyle, other conditions, occupational hazards, ethnicity, geographic, family history

Question 2

Most cancer predisposition genes have _ penetrance

Answer 2

reduced (<100)

Question 3

Autosomal Dominant Cancer Syndromes

Answer 3

Hereditary breast and ovarian cancer syndrome (BRCA1/2)
FAP (APC)
NF1 (NF1)
Von Hippel Lindau (VHL)

Question 4

Autosomal Recessive Cancer Syndromes

Answer 4

Bloom syndrome (BLM)
MYH- associated polyposis (MYH)

Question 5

X-linked Cancer Syndromes

Answer 5

Fanconi Anemia (FANCB)

Question 6

Features of Inherited Cancer syndromes

Answer 6

several relative with same or related cancer
younger age of onset than typical
AD pattern
rare cancers
multifocal or bilateral cancers
multiple primary cancers
other nonmalignant features
absence of external factors

Question 7

Rare cancers

Answer 7

male breast cancer
ovarian cancer
pheochromocytomas
paraglangliomas
pancreatic cancer

Question 8

Approximately _% of patients with pathogenic variant in a cancer predisposition did not meet criteria for testing

Answer 8

40%

Question 9

Reasons someone may not meet criteria

Answer 9

no significant/ limited family history
no personal history/ characteristics of disease