Tumours

Question 1

Diseases associated with GI lymphoma

Usual location

Answer 1

HIV
Ataxia telangiectasia 
Wiscott-Aldrich
SCID
XLA
Coeliac

Distal ileum & caecum
Appendix

Question 2

Syndromes associated with polyps

Answer 2

Peutz jeghers
FAP
Gardner syndrome

Question 3

Hormone secreting tumours associated with diarrhoea

Answer 3

VIPoma (GIT/CNS)
Somatostatinoma (somatostatin)
Gastrinoma (gastrin)
Carcinoid (Serotonin)
Mastocytoma (histamine)
Medullary carcinoma (VIP, PGs, calcitonin)
Ganglioneuroma/ ganglioneuroblastoma ( catecholamines & VIP )
Phaeochromocytoma (catecholamines)

Question 4

Pancreatic tumours

Answer 4

Glucagonoma - a cell
Insulinoma - b cell
Frantz tumour

Question 5

Peutz Jegher’s
3 main characteristics
Genetics

Answer 5

Mucocutaneous pigmentation
Intestinal polyposis
Significant risk of GI malignancy

AD
50% no FMHx

Question 6

Peutz Jeghers

Polyps occur
Are they pre-malignant
What GI emergency can they lead to?

Malignancy risk %
GIT or extra GIT more common?

Answer 6

Hamartomas
Stomach, SB, Colon.
NOT premalignant
Intususseption, bleeding

50% malignancy by mid adulthood
Extra intestinal more common
Lung, thyroid, gall bladder, biliary, breast, pancreas, cervix, ovary, sertoli cell

GIT(15%)- adenomas and adenocarcinomas

Question 7

Retention polyps
Occur in: %
Presentations

Malignant change-

Answer 7

3-4%, 2-20y
Hamartomas
Painless rectal bleeding 
Iron deficiency 
Intussuseption (rare)
Prolapse 

Malignant change rare

Question 8

FAP
Gene
Inheritance 
Onset
Symptoms

Answer 8

80-90% truncation of APC gene
Ch5q21
AD

Late childhood or adolescence 
>5 polyps = FAP
200 by 2nd decade
100% malignant change 
Surveillance then complete resection

Question 9

Garderner
Gene
Inheritance 
Features 
Extra colonic

Answer 9

APC
AD
Multiple colonic and ampullary polyps 
Osteomas
Soft tissue masses
Desmoid
Dental anomalies 

Same Rx as FAPC

Question 10

Turcot syndrome
Gene
Features

Answer 10

APC mutation
Brain tumour/medulloblastoma
Multiple colorectal polyps

Question 11

Most common malignant liver tumour

What proportion liver tumours malignant

Answer 11

Hepatoblastoma (65%)

50%

Question 12

Hepatoblastoma associations

Answer 12

BWS/hemihypertrophy 
FAP (APC gene)
ELBW
Fetal alcohol
Maternal OCP

Question 13

Hepatoblastoma

Spreads to
Usually arises
Age presentation 
Secretes
MGMT

Answer 13

Lungs and lymph nodes
Unifocal- right lobe liver
First 18mo. 75%<1y
Afp, bhcg
Surgery 
Chemo: cisplatin, doxorubicin, vincristine