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FRCS Part 1 > Ch43 Pituitary tumours > Flashcards

Ch43 Pituitary tumours Flashcards

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1
Q

What is the most common pituitary tumour?

A

Pituitary adenoma

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2
Q

How do pituitary tumours present?

A

Endocrinopathy, mass effect, headache, incidental finding and pituitary apoplexy

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3
Q

Which pituitary tumour is managed medically?

A

Prolactinoma with DA agonists (Cabergoline / bromocriptine / Quinagolide)

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4
Q

Pituitary carcinomas are invasive. Which hormones are they likely to secrete?

A

Prolactin or ACTH

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5
Q

What are the most common type of pituitary adenoma?

A

Non-functioning

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6
Q

Which familial syndrome is most commonly related to pituitary adenomas?

A

MEN1 - Autosomal dominant and also involves pancreatic islet cell tumours and parathyroid tumours. The pituitary adenomas are usually non-secretary.

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7
Q

Which hormones are secreted by adenomas?

A

PRL (48%), GH (10%), ACTH (6%) and TSH (1%)

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8
Q

How do prolactinomas present?

A

In Females - amenorrhoea-galactorrhea syndrome In Males - impotence

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9
Q

What is the stalk effect?

A

Compression of the pituitary stalk results in loss of DA inhibition and a modest rise in prolactin

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10
Q

What is the difference between Acromegaly and Gigantism?

A

Gigantism occurs before the closure of the epiphysis

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11
Q

What is thyrotropin?

A

TSH

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12
Q

How do FSH / LH secreting tumours present?

A

Usually clinically silent. FSH may cause amenorrhea-galactorrhea syndrome due to ovarian hyperstimulation

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13
Q

Which hormones are likely to be deficient with pituitary adenomas?

A

Go Look For The Adenoma GH > LH > FSH > TSH > ACTH

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14
Q

What does chronic panhypopituitarism cause?

A

Pituitary cachexia AKA Simmond’s cachexia - characterised by anorexia, amenorrhea, premature aging and low metabolic rate

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15
Q

Selective single pituitary deficiency is rare with adenomas. What other diagnosis should be considered?

A

Autoimmune hypophysitis which commonly causes deficiency of ACTH or ADH - resulting in DI

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16
Q

How does GH deficiency present?

A

In children with short stature

In adults with muscle loss, centripetal obesity and reduced exercise tolerance

Hypogonadism

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17
Q

How does hypothyroidism present?

A

Weight gain

Hair loss / dry skin

Cold intolerance

Myexdema

Entrapment neuropathy (carpal tunnel)

Tiredness

Constipation

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18
Q

What is Woltman’s sign o hypothyroidism?

A

Delayed relaxation of the ankle jerk

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19
Q

What conditions are associated with DI at presentation?

A

Autoimmune hypophysitis

Hypothalamic glioma

Suprasellar GCT

Craniopharyngioma

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20
Q

What is Kallmann syndrome?

A

Combination of anosmia and central hypogonadatrophic hypogonadism. MRI reveals a lack of olfactory bulb in 60%,

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21
Q

What are the consequences of mass effect by a pituitary lesion?

A

Optic chiasm > bitemporal hemianopsia starting from the upper fields

Thrid ventricle > obstructive hydrocephalus

Cavernous sinus > Cranial neuropathy, proptosis, chemosis from venous congestion, encasement of carotids

CSF leak

Headache

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22
Q

What is the definition of apoplexy?

A

Sudden expansion of a sellar mass causing neurological or endocrinological deterioration

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23
Q

What causes expansion with apoplexy?

A

Haemorrhage, necrosis or infarction

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24
Q

How does apoplexy present?

A

Headache

Visual disturbance (opthalmoplegia > VFD)

Loss of conciousness (raised ICP or hypothalamic involvement)

Cavernous sinus compression (Cranial neuropathy / venous congestion)

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25
What is the difference between Cushing's syndrome and disease?
Cushing's syndrome is a constellation of symptoms secondary to hypercortisolism. Cushing's disease is hypercortisolism secondary to an ACTH-secreting adenoma.
26
What are the causes of endogenous hypercortisolism?
Pituitary adenoma (80%) - mildly elevated ACTH Ectopic ACTH from lung ca (10%) - very elevated ACTH Adrenal adenoma / carcinoma (10%) - low ACTH as directly secretes cortisol suppressing ACTH Hypothalamic CRH release (very rare) - elevated ACTH
27
Which carcinomas can release ectopic ACTH?
Small cell lung ca Thymoma Carcinoid tumours Phaeochromocytomas Medullary thyroid ca
28
What is the gender predominance of Cushing's disease?
10x more common in women Note: Ectopic ACTH is 10x more common in men!
29
What is more common, ACTH releaseing adenoma or Acromegaly?
Acromegaly is 4x more common than Cushing's disease
30
What is the difference between the types of pituitary adenomas in adults and children?
Cushing's disease is more frequent in children and non-functioning is less frequent.
31
What size are ACTH-releasing adenomas typically?
\<5 mm in 50% of cases
32
What are the clinical features of Cushing's syndrome?
Hands - easy bruising, hyperpigmentation (if ACTH high), carpal tunnel, thin skin Arms - prox. myopathy, hypertension, osteoporosis Face - plethoric facies, hirsuitism, acne, depression, dementia Body - centripetal obesity, buffalo hump, purple striae
33
What are the laboratory findings with Cushing's disease?
Hyperglycaema, hypokalemic alkalosis, loss of diurnal cortisol variation, inappropriately high or normal ACTH levels, high 24 hours urine free cortisol, failure to suppress with low dose dexamethasone test
34
What is Nelson's syndrome?
Occurs in 30% of patients that undergo bilateral adrenalectomies performed for Cushing's disease which is performed when adenoma is non-resectable or failure of medical therapy after TSH. Presents with hyperpigmentation (due to ACTH cross reactivity with MSH), high ACTH and progression of pituitary tumour
35
What is the treatment for Nelson's syndrome?
Resection of adenoma, Radiotherapy or Medications e.g. Metyrapone
36
Why does Nelson's syndrome occur after bilateral adrenalectomy?
Cortisol levels normalise \> CRH levels rise \> ACTH-releasing adenoma grows \> high ACTH causes hyperpigmentation due to MSH corss reactivty.
37
Why do you get testicular enlargement with Nelson's syndrome?
Due to high ACTH stimulating hypertrophy of adrenal rest cells in the testes, which then secrete cortisol and in some cases return of Cushing's disease despite adrenalectomy
38
What is the management of pituitary apoplexy?
Hydrocortisone administration Visual field assessment Endocrine evaluation Rapid surgical decompression is required for sudden constriction of the visual fields or deterioration in acuity. Bills et al 1993 showed in a retrospective study of 37 patients that surgery within 7 days was better then after 7 days.
39
What is the goal of transsphenoidal surgery in apoplexy?
1. Decompress the optic apparatus, pituitary gland, cavernous sinus and third ventricle if hydrocephalus 2. Histological diagnosis Complete removal fo the tumour is not necessary
40
What is the Mod. Hardy's (Wilson) system for classification of pituitary adenoma extension / spread?
**_Extension_** Suprasellar: A) In suprasellar cistern, B) Recess of 3rd ventricle obliterated, C) Floor of 3rd ventricle grossly displaced Parasellar: D) Intrdural, E) Into cavernous sinus **_Spread_** I - sella \<10mm, II sella \>10mm III - localised or IV - diffuse destruction of sella floor V - spread via CSF
41
What is Forbe's Albright syndrome?
Amenorrhea-Galactorrhea syndrome secondary to prolactin-secreting pituitary tumour
42
What is McCune Albright syndrome?
Polyostotic fibrous dysplasia Cafe au lait spots Precocious puberty
43
What % of prolactinomas are microadenomas at diagnosis?
90% in women and 60% in men
44
What % of GH are macroadenomas at diagnosis?
75%
45
What work up is needed for a patient with acromegaly?
Endocrine Opthalmology Cardiac (cardiomyopathy) Colonoscopy
46
What are the criteria for biochemical cure in acromegaly?
Normal IGF-1, GH \<5 ng/ml and GH nadir \< 1 ng/ml after OGTT
47
What are the causes of ectopic GH secretion?
Carcinoid tumours of the lung, pancreas and GI tract
48
What % of GH-adenomas also secrete prolactin?
25%
49
Which genetic syndromes are associated with acromegaly?
MEN-1, McCune Albright, familial acromegaly and Carney complex (PRKAR1A = protein kinase A regulatory subunit 1A)
50
What are the clinical findings associated with acromegaly?
MNEMONIC: STDS ROC A PIMPS BENT GO BAC Spade-like hands Tremor Diabetic testing marks Sweating Rings not fitting Osteoarthritis - Heberden's nodes Carpal tunnel syndrome Acanthosis nigricans Prox. myopathy Incisor spacing Macroglossia Prognathism Supra-orbital riding Bossing of frontal bone Ears enlarged Nose enlarged Temporal hemianopia Goitre Organomegaly Bowel cancer Apnea sleep Cardiomyopathy
51
Why is life-expectancy reduced with untreated acromegaly?
Diabetes Hypertension Cardiovascular risk Colon cancer Sleep apnoea
52
What cardiovascular complications occur in acromegaly?
Cardiomyopathy - reduced LV diastolic function, increased LV size, arrhythmias and fibrous hyperplasia of the connective tissue. Hypertension exacerbates the cardiomyopathic changes
53
What % of adenomas release TSH?
\<1%
54
What is the difference biochemically between primary and secondary hyperthyroidism?
Primary has high T4 and low TSH Secondary has high T4 and high TSH
55
What are the clinical symptoms of hyperthyroidism?
Anxiety Palpitations Atrial fibrillation / SVT Heat intolerance Hyperhidrosis Weight loss despite increased food intake
56
What are the clinical signs with hyperthyroidism?
Hyperactivity, lid lag, tachy, irreg heart rate, hyperreflexia and tremor. Exopthalmos and pretibial myxedema are only with Grave's disease.
57
What are the most common histologies with non-functional adenomas?
Null cell adenomas Oncocytomas Silent gonadotrophin / corticotrophin adenomas
58
What is the most common tumours in the posterior pituitary?
METASTASIS due to the rich blood supply
59
What is the acute management of apoplexy?
Pituitary profile Empirical IV hydrocortisone if signs of addisonian crisis (haemodynamic instability) VFD when stable. Urgent TSH within 7 days should be performed if low conscious level or progressive visual field deterioration (not cranial nerve palsy from cavernous sinus compression).
60
What is a predictor of hypopituitarism following apoplexy?
Low prolactin levels
61
When do you check morning cortisol after transsphenoidal surgery?
Check 9 am cortisol on day 2 or 3 (omit the evening dose of hydrocortisone the night before). If \>550 nmol/l then does not need steroids If 400-550 nmol/l then needs steroids when ill If \<400 nmol/l then commence steroid replacement All hormones should be rechecked at 4-8 weeks
62
Can DI occur with primary adrenal insufficiency?
NO, you cannot diagnose DI unless the adrenal glands are functioning as the mineralocorticoid (aldosterone) is needed to concentrate the urine. Steroids can mask DI as they have mineralocorticoid effect.
63
When are LH & FSH highest?
Mid-cycle (FSH follicular 0.5-5, mid-cycle 8-33 and luteal 2-8) (LH follicular 3-12, mid-cycle 20-80 and luteal 3-16)
64
How does the water deprivation test distinguish cranial and nephrogenic DI?
Serum osmo \>290 i.e. becomes dry with water deprivation If urine osmo fails to concentrate i.e. \<300, but the administration of DDAVP does lead to urine concentration then this is a cranial cause i.e. lack of ADH release. If the DDAVP does not lead to urine concentration then this is a nephrogenic cause of DI as the kidney is unable to contrate the urine.
65
What test should you perform in a patient with synchronous germinoma?
Serum and CSF B-HCG and AFP (as there may be non-germinomatous elements)
66
What is a normal response to a short synacthen test?
Levels should be \>600 nmol/l after 30 mins otherwise the patient has primary adrenal insufficiency!
67
Which hormone is most likely to be deficient in non-functioning adenomas?
FSH/LH
68
What is a contraindication to GH therapy?
Active malignancy
69
What is the gold-standard test for GH deficiency?
If **IGF-1 level** is low then dynamic testing with **insulin tolerance test** is required. This is contraindicated in those with IHD, epilepsy and obesity. In which case stimulation testing with **GHRH-arginine test** is needed.
70
What prolactin levels are associated with a stalk effect vs a prolactinoma?
Normal levels are \<100 ng/l or \<2000 mIU/l Stalk effect levels are \<200 ng/l or \<4000 mIU/l Prolactinoma level are \>200 ng/l or \>4000 mIU/l
71
How do you treat a non-functional pituitary adenoma with significant cavernous sinus invasion?
TSH to debulk the lesion and decompress the optic nerves Ensure enough distance between the tumour and the optic nerves so that SRS can be given to the cavernous sinus residual tumour.
72
Why does hypothyroidism result in raised prolactin levels?
As prolactin release is stimulated by high levels of TRH (which is elevated in hypothyroidism)
73
Why should females starting bromocriptine/cabergoline also commence oral contraceptives?
As they will normalise the prolactin levels and restore fertility
74
Why is the management of prolactinomas challenging during pregnancy?
As prolactin levels are unreliable. Growth during pregnancy is 5% for micros and 15-40% for macros
75
What is the significance of a hypointense area in the pituitary gland in a patient with Cushing's syndrome?
This is a nonspecific finding that is also seen in 10% of normal patients. so IPSS should be performed to rule out ectopic ACTH secretion from a carcinoid tumour in the lung, adrenal or GI systems.
76
What are the risks of untreated Cushing's disease?
Cardiovascular events Obesity Infections etc 5x higher mortality with Cushing's disease which is reversed with treatment.
77
What are the options after failed TSH in Cushing's disease?
Hemihypophysectomy or completion hypophysectomy Medical treatments (metyrapone, ketoconazole, mitotane and etomidate) Glucorticoid-R blocker (mifepristone) Bilateral adrenalectomy (risk of Nelson's syndrome)
78
How do you diagnose Acromegaly?
Raised IGF-1 If unequivocal then needs an OGTT. If it fails to suppress GH to \<1ng/ml then confirms GH-releasing adenoma.
79
What is the initial management of a patient with a CSF leak after TSH?
CT head to r/o hydrocephalus and subdurals Abx if signs of meningitis Flat bed rest (spontaneous resolution in 70%) If fails to resolve spontaneously then lumbar drain +/- re-exploration. If defect \<1cm - fat plug If larger needs cartilage graft / nasoseptal flap
80
What are the MRI features of a Rathke's cleft cyst?
T1 hyperintense cyst due to the proteinaceous content of the cyst fluid. Thin uniform wall which does not enhance.
81
What are the features of cystic VS?
More rapid growth Frequent CN7 involvement Unpredictable biological behaviour Heamorrhage into the cyst esp after SRS may be associated with brainstem compression and obstructive hydrocephalus
82
What is a marginal sinus?
A sinus that runs on the inner aspect of the foramen magnum
83
What are the boundaries of Trautmann's triangle?
Superior petrosal sinus above Sigmoid sinus behind Jugular bulb below Semicircular canal anterior
84
What SRS dose is given to vestibular schwannomas?
12Gy. Not 16 Gy is associated with facial nerve injury in 1/3!

FRCS Part 1 (85 decks)

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