Tumours: Inherited Cancer Syndromes

Question 1

what are the 2 common inherited cancer syndromes?

Answer 1

• Hereditary Non Polyposis Coli (HNPCC) (100 polyps)

Question 2

which hereditary cancer syndrome presents with > 100 polyps

Answer 2

FAP

Question 3

what are the less important inherited cancer syndromes?

Answer 3

MYH - associated polyposis
Turcot’s syndrome
Gardner’s syndrome

Question 4

which are the autosomal dominant?

Answer 4

HNPCC and FAP

Question 5

The defect is in the DNA mismatch repair (tumour suppressor) resulting from an inherited mutation in MLH-1, MLH-2, PMS - 1 or MSH - 6 genes, which is this ?

Answer 5

HNPCC

Question 6

The defect is in the ___ ___ ___ (tumour suppressor) resulting from an inherited mutation in MLH-1, MLH-2, PMS - 1 or MSH - 6 genes

Answer 6

The defect is in the DNA mismatch repair (tumour suppressor) resulting from an inherited mutation in MLH-1, MLH-2, PMS - 1 or MSH - 6 genes

Question 7

HNPCC - which genes cause the defect in DNA mismatch repair? 4

Answer 7

MLH-1, MLH-2, PMS - 1 or MSH - 6 genes

Question 8

what are 4 identifying factors of HNPCC?

Answer 8

Right sided tumours

Question 9

which inherited cancer syndrome is associated with gastric and endometrial carcinoma?

Answer 9

HNPCC

Question 10

FAP - There is a defect in___ -___ resulting from an inherited mutation in the ___ gene

Answer 10

There is a defect in tumour suppression resulting from an inherited mutation in the FAP gene

Question 11

what are 4 identifying factors of FAP?

Answer 11

Throughout the colon
>100 polyps
Adenocarcinoma NOS
There is no specific inflammatory response

Question 12

which inherited cancer syndrome is associated with desmoid tumours and thyroid carcinoma?

Answer 12

FAP