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Endocrinology > Extra endocrine > Flashcards

Extra endocrine Flashcards

1
Q

What is a phaeochromocytoma

A

Catecholamine producing tumour

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2
Q

Where do you phaeochromocytomas

A

Usually arises from chromaffin cells of adrenal medulla (10% are bilateral and 10% are malignant)
Or can be non-adrenal in origin - in which case called paragangliomas

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3
Q

Cause of phaeochromocytoma

A

Sporadic cases, cause unknown
Familial in up to 30% - including MEN2a, neurofibromatosis type 1, von-hippel-lindau, and mutations in subunits of mitrochondrial enzyme succinate dehydrogenase (SDHB, SDHC, SDHD)

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4
Q

Features of phaeochromocytoma

A

Paroxysmal episodes of sweating, headache
Palpitations, chest pain, dyspnoea
Epigastric pain, nausea and constipation
Weakness, tremor and anxiety

Hypertension - 2/3 sustained 1/3 paroxysmal
Pallor tachycardia, fever and weight loss

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5
Q

Investigations for phaeochromocytoma

A

24hr urine to look for urinary catecholamines (adr, NA, dopamine)

CT or MRI for tumour identification

123I-MIBG scintigraphy to look for malignancy

Screen for associated conditions

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6
Q

Bloods in phaeochromocytoma

A

calcium may be elevated, hypokalaemia

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7
Q

What drugs can increase measured catecholamines

A

TCA’s

Levodopa

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8
Q

Medical treatment of phaeochromocytoma

A

rehydrate first
alpha blockade with phenoxybenzamine
b-blockade with propanolol (2 days after alpha blockade)

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9
Q

Surgical management of phaeochromocytoma

A

Adrenalectomy

Chemotherapy and surgery if mets

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10
Q

Follow up for phaeochromocytoma

A

Lifelong follow up because of recurrence

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11
Q

What is carcinoid syndrome

A

Constellation of symptoms caused by systemic release of humoral factors (including biogenic amines, polypeptides, prostaglandins eg. serotonin, histamine, tachykinins, kallikrein ) from carcinoid tumours

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12
Q

What are carcinoid tumours?

A

Slow-growing neuroendocrine tumours mostly derived from serotonin-producing enterochromaffin cells

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13
Q

Where are majority of carcinoid syndrome producing tumours

A

In small bowel

Common sites include appendix and rectum but these are often benign and non-secretory

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14
Q

When do symptoms of carcinoid syndrome present?

A

Hormones released into portal circulation are metabolised in liver therefore usually don’t get symptoms until mets have spread to liver and then get products in hepatic vein

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15
Q

Signs + symptoms of carcinoid tumour

A

Paroxysmal flushing, diarrhoea, crampy abdo pain, wheeze, sweating and palpitations
Facial flushing, telangiectasia
Tricuspid stenosis, regurge or pulmonary stenosis

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16
Q

What are symptoms of carcinoid crisis

A

Profound flushing, bronchospasm, tachycardia and fluctuating blood pressure

17
Q

Investigations in carcinoid syndrome

A

24h urine collection for 5-HIAA levels (metabolite of serotonin)

Blood for plasma chromogranin A and B - fasting gut hormones

Radioisotope scan with radiolabelled somatostatin analogue helps localise tumour

18
Q

Management of carcinoid crisis

A

Octreotide infusion, IV antihistamine and hydrocortisone

19
Q

Treatment of carcinoid syndrome

A

Octreotide (somatostatin analogue inhibits hormone release and tumour growth) radiolabelled may be beneficial
Interferon-alpha if octreotide is not enough

20
Q

When consider surgery for carcinoid syndrome

A

For resectable nodal or hepatic mets or tumours

Valve surgery for symptomatic carcinoid heart disease

21
Q

What is men 1?

A 
Also called Wermer syndrome
Pancreatic tumours
Pituitary adenomas 
Parathyroid hyperplasia 
Angiofibroma (vascular lesion at back of nasal cavity)
Lipoma
22
Q

What is Men 2a

A

Medullary thyroid carcinoma
Phaeochromocytoma
Parathyroid hyperplasia

23
Q

What is Men 2b

A

Medullary thyroid carcinoma
Phaeochromocytoma
Marfanoid body habitus
Mucosal neuroma (lumps on mucosal surfaces eg. lips and tongue)

24
Q

Management of acute severe hypercalcaemia

A

Fluid resus

Bisphosphonates - IV pamidronate - takes a few days to work

25
Q

Serum level required for hypercalcaemia

A

2.65 or higher

26
Q

Common causes of hypercalcaemia

A

HyperPTH

Cancer

27
Q

What is MODY

A

Maturity onset diabetes of the young - onset of diabetes t2 in under 25yr old

28
Q

Features of MODY

A

Autosomal dominant with strong family history
Doesn’t usually present with ketosis
Normally very sensitive to sulphonylureas

29
Q

Test to tell between T1DM and T2DM

A

C-peptide - decreased in T1DM

Raised or normal in T2dm

30
Q

Type of goitre in hashimotos

A

non-tender goitre

31
Q

Goitre in de quevains thyroidistis

A

painful

32
Q

Thyroid bruit + hyperthyroid?

A

Graves

Endocrinology (8 decks)

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