Endo Neoplasms II Flashcards by megan king

pituitary adenomas are categorised by

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

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pituitary adenomas are benign or malignant?

benign

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mutation of lactinotroph adenoma

derived - PIT-1 lineage
sporadic - MEN-1

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hypogonadism
lack of libido
amennorhoea
galactorrhoea
gyecomastica

lactotrophic adenoma (prolacin)

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treatment of lactotrophic adenoma

bromocriptine
cabergoline

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mutation of somatotroph adenoma

GNAS 1

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Growth hormone has a negative feedback on

glucose
IGF-1

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what is growth hormone effect on sodium

antinaturetic
sodium retention

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hyperglycaemia, hypertension
headache, visual defects
organomegaly, myopathy

somatotroph adenoma

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somatotroph adenoma increases the risk of

GIT cancers
Heart failure

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mutation of corticotroph adenoma

transcription factor TPIT

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

corticotroph adenoma

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

nelson syndrome

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craniopharyngiomas develop from

Rathke’s pouch
ectoderm buds

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supratentorial
children
cystic
dystrophic calcification
mass effects

craniopharyngioma

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gene mutation of craniopharyngioma

CTNNB1
B catenin gene mutation

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cystic tumour with haemorrhage
dystrophic calcification
motor oil - cholesterol rich

craniopharyngioma

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islands of epithelium
peripheral nuclar palasaiding
loose areas stellate reticulum

adamantinomatous craniopharyngioma

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fibrovascular core
peripheral palasaiding squamous cells
spongy reticulum

papillary craniopharyngioma

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thyroid nodules - malignant features on RIU

solitary
younger pt
male
cold nodule

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female
non-functional
hot nodule
can be a toxic adenoma

follicular thyroid adenoma

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solitary spherical
encapsulated
uniform follicles
contain colloid

follicular thyroid adenoma (benign)

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

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

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

papillary thyroid carcinoma

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

follicular thyroid carcinoma

medullary carcinomas derived from which cells

parafollicular C cells

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

medullary thyroid carcinoma

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

anaplastic thyroid carcinoma

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

medullary carcinoma calcitonin secretion MEN2A MEN2B

physical findings of thyroid tumours

fixed nodules hoarseness cervical lymphadenopathy

tests for thyroid cancer diagnosis

radioactive iodine uptake fine needle aspiration core tissue biopsy

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

parathyroid adenoma

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

parathyroid hyperplasia

4 glands enlarged nodular / diffuse clear cell hyperplasia MEN syndrome

parathyroid carcinoma

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

pheochromocytoma of adrenal medulla

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

pheochromocytoma of adrenal medulla

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

pheochromocytoma of adrenal medulla

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

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

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

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

what mutations have predisposition to pheochromoctyoma

von Hippel-Lindau syndrome MEN -1 NF-1

what are the metabolic products of EPI and NOR

metanephrine vanillylmandelic acid (VMA)

list some hyperadrenergic (adrenal) symptoms

pain - headache pressure - raised BP perspiration palpitation pallor

name the 4 cell types in islet of langerhans

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

what is the pathway of insulin metabolism

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

what pancreatic tumour is non-malignant

insulinoma

that 3 major genes contribute to pancreatic tumours

MEN-1 PTEN ATRX, DAXX

3 clinical syndromes associated with functional pancreatic endocrine neoplasms

hyperinsulinism hypergastrinaemia - Zollinger-Ellison syndrome MEN syndrome

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

insulinoma

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

insulinoma Whipples triad

giant islets preserved cords of monotonous cells abundant amyloid deposition

insulinoma

malignant post-menopausal high plasma glucose nerolytic migratory erythema

glucagonoma

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

necrolytic migratory erythema feature of glucagonoma

malignant secretes gastrin MEN1 abdominal pain, diarrhoea

gastrinoma

zollinger ellison syndrome is associated with what tumour

gastrinoma

abdominal pain heartburn Diarrhea Nausea and vomiting weight loss tarry stool

Zollinger Ellison Syndrome - assoc. with gastrinoma

**secretion** injection test - what is seen in ZES

dramatic rise in gastrin levels (G cells normally inhibited)

watery diarrhoea, hypokalaemia, anchlorydria tea coloured odourless urine

VIPoma vasoactive intestinal peptide

diabetes mellitus cholelithiasis steatorrhoea hypochlorhydria

somatostatinoma