endocrine tumours Flashcards
neuroblastoma
tumour arises from neural crest tissue of the adrenal medulla + sympathetic nervous system
- top 5 causes of cancer in kids
- median age onset = 20months
neuroblastoma presentation
- abdominal mass
- pallor weight loss
- bone pain, limp
- hepatomegaly
- paraplegia
- proptosis
neuroblastoma investigation
raised vanillymandelic acid (VMA) + homobanillic acid (HVA) levels
calcification on abdo xray
biopsy !
phaemochromocytoma
catacholamine secreting tumour
- 10% familial
assoc with MEN II, neurofibromatosis, von Hippel-Lindau syndrome
- bilateral in 10%
- malignant in 10%
- extra-adrenal in 10%
commonest site of extra adrenal phaeochromocytoma
organ of zuckerandl, adjacent to bifurcation of aorta
phaeochromocytoma presentation
features typically episodic
- headaches
- palpitations
- sweating, anxiety
suuuper hypertensive
phaeochromocytoma investigations
24hr urinary collection of metanephrines
phaeochromocytoma management
definitive = surgery
stabilised with medical management first;
- alpha blocker (phenoxybenzamine) given before a
- beta blocker - propranolol
Multiple endocrine neoplasia (MEN) type 1
3 P’s
- parathyroid (hyperparathy)
- pituitary
- pancreas (insulinoma)
—> leads to recurrent peptic ulceration
MEN1 gene
hypercalcaemia = commonest presentation
Multiple endocrine neoplasia (MEN) type 2a
medullary thyroid cancer
2 P’s
- parathyroid
- phaeochromocytoma
RET oncogene
Multiple endocrine neoplasia (MEN) type 2b
medullary thyroid cancer
1 P = phaeochromocytoma
- marfanoid body habitus
- neuromas
RET oncogene
insulinoma
a neuroendocrine tumour deriving mainly from pancreatic Islets of Langerhans cells
- 10% malignant, 10% multiple (50% of these have MEN1)
insulinoma presentation
- hypoglycaemia - typically early morning or before meal - diplopia, weakness
- rapid weight gain
- high insulin
- raised proinsulin:insulin ration
- high C-peptide
diagnosis + management of insulinoma
dx
- supervised prolonged fasting - up to 72hrs
- CT pancreas
mx
- surgery
- diazoxide + somatostatin if unsuitable for surgery
prolactinomas
type of pituitary adenoma, benign tumour of pit gland
- most common pituitary adenoma
pituitary adenoma classification
size
- microadenoma <1cm
- macroadenoma >1cm
hormonal status
- secretory/functioning adenoma -> produces excess of a hormone
- non-secretory/functioning adenoma
prolactinoma presentation
women
- amenorrhoea
- infertility
- galactorrhoea
- osteoporosis
men
- impotence
- loss of libido
- galactorrhoea
sx of macroadenomas
- headache
- visual disturb - bitemporal hemianopia
diagnosis + management of prolactinomas
dx = MRI
mx
- dopamine agonists -> cabergoline, bromocriptine (inhibit release of prolactin)
- surgery if don’t respond to medical
–> transsphenoidal approach
management of GH secreting pituitary adenomas
somatostatin analogues
- octretide
- lanreotide
GH receptor antagonists - pegvisomant
management of ACTH-secreting pituitary adenomas
cortisol synthesis inhibitors
- ketoconazole
- metyrapone
