Endo Neoplasms II

Question 1

pituitary adenomas are categorised by

Answer 1

**size **(micro <10mm macro >10mm)
functional (secrete hormone) / non-functional (non-secreting)
Cell origin affected - somatotrph, thyrotroph, lactotroph, gonadotroph, corticotroph

Question 2

pituitary adenomas are benign or malignant?

Answer 2

benign

Question 3

mutation of lactinotroph adenoma

Answer 3

derived - PIT-1 lineage
sporadic - MEN-1

Question 4

hypogonadism
lack of libido
amennorhoea
galactorrhoea
gyecomastica

Answer 4

lactotrophic adenoma (prolacin)

Question 5

treatment of lactotrophic adenoma

Answer 5

bromocriptine
cabergoline

Question 6

mutation of somatotroph adenoma

Answer 6

GNAS 1

Question 7

Growth hormone has a negative feedback on

Answer 7

glucose
IGF-1

Question 8

what is growth hormone effect on sodium

Answer 8

antinaturetic
sodium retention

Question 9

hyperglycaemia, hypertension
headache, visual defects
organomegaly, myopathy

Answer 9

somatotroph adenoma

Question 10

somatotroph adenoma increases the risk of

Answer 10

GIT cancers
Heart failure

Question 11

mutation of corticotroph adenoma

Answer 11

transcription factor TPIT

Question 12

30-40yo female
high cortisol
cushing disease
microadenoma <10mm

Answer 12

corticotroph adenoma

Question 13

headache, visual disturbances
high ACTH, low cortisol
increased MSH - pigmentation

Answer 13

nelson syndrome

Question 14

craniopharyngiomas develop from

Answer 14

Rathke’s pouch
ectoderm buds

Question 15

supratentorial
children
cystic
dystrophic calcification
mass effects

Answer 15

craniopharyngioma

Question 16

gene mutation of craniopharyngioma

Answer 16

CTNNB1
B catenin gene mutation

Question 17

cystic tumour with haemorrhage
dystrophic calcification
motor oil - cholesterol rich

Answer 17

craniopharyngioma

Question 18

islands of epithelium
peripheral nuclar palasaiding
loose areas stellate reticulum

Answer 18

adamantinomatous craniopharyngioma

Question 19

fibrovascular core
peripheral palasaiding squamous cells
spongy reticulum

Answer 19

papillary craniopharyngioma

Question 20

thyroid nodules - malignant features on RIU

Answer 20

solitary
younger pt
male
cold nodule

Question 21

female
non-functional
hot nodule
can be a toxic adenoma

Answer 21

follicular thyroid adenoma

Question 22

solitary spherical
encapsulated
uniform follicles
contain colloid

Answer 22

follicular thyroid adenoma (benign)

Question 23

mutations of thyroid carcinomas:
papillary, follcular, medullary, anaplastic

Answer 23

Papillary = RET/PTC + radiation, BRAF
Follicular = RAS, PAX8-PPAR-y rearrangement, PIK3CA & PTEN
Medullary = MEN2, RET
Anaplastic = P53

Question 24

20-30yo female
radiation / hashimotos
RET translocation
lymphatic invasion
orphan annie eye nuclei

Answer 24

papillary thyroid carcinoma

Question 25

40-50yo female
iodine deficiency
N-RAS mutation
painless, cold nodules
capsular / vascular invasion

Answer 25

follicular thyroid carcinoma

Question 26

medullary carcinomas derived from which cells

Answer 26

parafollicular C cells

Question 27

MEN2
mass in neck
secretes calcitonin
c cells
amyloid accumulation
congo red stain

Answer 27

medullary thyroid carcinoma

Question 28

65yo
inactivation P53
rapidly enlarging neck mass
inavdes neighbouring tissues
often fatal

Answer 28

anaplastic thyroid carcinoma

Question 29

diarrhoea
flushing
itching
are a result of which thyroid tumour & secretion of what…

Answer 29

medullary carcinoma
calcitonin secretion
MEN2A MEN2B

Question 30

physical findings of thyroid tumours

Answer 30

fixed nodules
hoarseness
cervical lymphadenopathy

Question 31

tests for thyroid cancer diagnosis

Answer 31

radioactive iodine uptake
fine needle aspiration
core tissue biopsy

Question 32

solitary nodule
women 50-70yo
overexpression cyclin D1 gene
MEN syndrome

Answer 32

parathyroid adenoma

Question 33

4 glands enlarged
HRPT2 parafibromin gene
retinoblastoma locus
vascular invasion
bands dense fibrous tissue

Answer 33

parathyroid hyperplasia

Question 34

4 glands enlarged
nodular / diffuse
clear cell hyperplasia
MEN syndrome

Answer 34

parathyroid carcinoma

Question 35

unilateral, tumour of chromafin cells
produce catecholamines
headache, sweating, tachycardia

Answer 35

pheochromocytoma of adrenal medulla

Question 36

increased metanephrine
increased VMA
lack suppression of NOR with clonidine
hyperglycaemia
neutrophilic leucocytosis

Answer 36

pheochromocytoma of adrenal medulla

Question 37

LOF mutation in succinate dehydrogenase subunit
micro: zellballen, nuclear salt & pepper

Answer 37

pheochromocytoma of adrenal medulla

Question 38

adrenal medulla & organ or Zuckerkandl contain N-Methyltransferse, so produce

Answer 38

norepinephrine AND epinephrine
(N-M enzyme converts nor –> epi)

Question 39

Bladder wall, posterior mediasinum don’t produce N-Methyltransferase, so produce

Answer 39

norepinephrine NOT epinephrine
(N-M enzyme converts nor –> epi)

Question 40

what mutations have predisposition to pheochromoctyoma

Answer 40

von Hippel-Lindau syndrome
MEN -1
NF-1

Question 41

what are the metabolic products of EPI and NOR

Answer 41

metanephrine
vanillylmandelic acid (VMA)

Question 42

list some hyperadrenergic (adrenal) symptoms

Answer 42

pain - headache
pressure - raised BP
perspiration
palpitation
pallor

Question 43

name the 4 cell types in islet of langerhans

Answer 43

Alpha cell = glucagon
Beta cell = insulin
Delta cell = somatostatn
PP cell = pancreatic polypeptide

Question 44

what is the pathway of insulin metabolism

Answer 44

Beta cells –> preproinsulin –> proinsulin –> insulin –> c-peptide

Question 45

what pancreatic tumour is non-malignant

Answer 45

insulinoma

Question 46

that 3 major genes contribute to pancreatic tumours

Answer 46

MEN-1
PTEN
ATRX, DAXX

Question 47

3 clinical syndromes associated with functional pancreatic endocrine neoplasms

Answer 47

hyperinsulinism
hypergastrinaemia - Zollinger-Ellison syndrome
MEN syndrome

Question 48

benign
hyperinsulinaemia precipitated by exercise/fasting
decreased insulin
increased c peptide

Answer 48

insulinoma

Question 49

low fasting blood glucose
hypoglycaemia
neurological symptoms
relief of symptoms with glucose admin

Answer 49

insulinoma
Whipples triad

Question 50

giant islets
preserved cords of monotonous cells
abundant amyloid deposition

Answer 50

insulinoma

Question 51

malignant
post-menopausal
high plasma glucose
nerolytic migratory erythema

Answer 51

glucagonoma

Question 52

cutaneous paraneoplastic syndrome
painful f=pruritic crusty erythematous papules
brone coloured induration
palues & necrosis

Answer 52

necrolytic migratory erythema
feature of glucagonoma

Question 53

malignant
secretes gastrin
MEN1
abdominal pain, diarrhoea

Answer 53

gastrinoma

Question 54

zollinger ellison syndrome is associated with what tumour

Answer 54

gastrinoma

Question 55

abdominal pain
heartburn
Diarrhea
Nausea and vomiting
weight loss
tarry stool

Answer 55

Zollinger Ellison Syndrome - assoc. with gastrinoma

Question 56

secretion injection test - what is seen in ZES

Answer 56

dramatic rise in gastrin levels (G cells normally inhibited)

Question 57

watery diarrhoea, hypokalaemia, anchlorydria
tea coloured odourless urine

Answer 57

VIPoma
vasoactive intestinal peptide

Question 58

diabetes mellitus
cholelithiasis
steatorrhoea
hypochlorhydria

Answer 58

somatostatinoma