Endo/Neuroendo/Urol Flashcards

1
Q

How should patients with suspected pheochromocytoma be initially investigated?

A

Plasma free metanephrines (from supine position) and/or urinary fractionated metanephrines

Note this is not vanillymandelic acid (VMA) - which has a high false positive rate

(Endocrine Society Clinical Practice Guidelines 2014)

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2
Q

When is a MIBG scintigraphy indicated in management of phaeo/paraganglioma?

A

With metastatic disease when radiotherapy using I-MIBG is planned, otherwise use PET-CT

(Meta-iodobenzylguandidine)
(Endocrine Society Clinical Practice Guidelines 2014)

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3
Q

What germline mutation should be tested for in patients with Paragangliomas?

A

Succinate dehydrogenase (SDH) - about 50% prevalence if present

(Endocrine Society Clinical Practice Guidelines 2014)

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4
Q

How should functional phaeochromocytomas/paragangliomas be managed in the preoperative setting?

A

A-adrenergic blockers first choice, allowing 7-14 days prep.
Advice re high sodium/fluid diet (to combat intravascular depletion post op)

(Endocrine Society Clinical Practice Guidelines 2014)

A blockers - phenoxybenzamine (irreversible), phentolamine, doxazosin

Often give beta blockade after establishing alpha blockade (labetolol)

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5
Q

How should patients with resected PPGL be monitored?

A

Annual plasma/urine metanephrines

(Endocrine Society Clinical Practice Guidelines 2014)

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6
Q

When should an open approach be taken to resection of pheochromocytoma?

A

Large >6cm, invasive tumours - minimise rupture, complete margins

Most paraganlgiomas

Partial can be considered rarely

(Endocrine Society Clinical Practice Guidelines 2014)

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7
Q

How does a phaeochromocytoma differ from a Paraganglioma?

A

Phaeo is tumour arising adrenomedullary chromatin cells (producing one or more of epinephrine, norepinephrine and dopamine) - about 80-85%.

Paraganglioma 15-20% - extra-adrenal chromatin cells of sympathetic paraveterbral ganglia of thorax, abdomen and pelvis. Can also arise from parasympathetic ganglia along IX and X nerves and base of skull - do not produce catecholamines

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8
Q

What is the prevalence of PPGL among adult hypertensive outpatients?

A

0.2-0.6% — 1.7% children

5% of incidental adrenal masses

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9
Q

Which syndromes are associated with PPGL?

A

MEN2a - MTC, HPTH, lichen amyloidosis
MEN2b - MTC, neuromas, intestinal gangliomas (Hirschprungs)
vHL - HAemangiomblasoma, retinal angioma, clear cell RCC, pancreatic NETs and cyst adenomas, papillary cyst adenomas of epididymis
NF1 - neurofibromas, cafe-au-lait spots, freckling, iris harmartomas (Lisch nodules)

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10
Q

What proportion of phaeochromocytomas are bilateral?

A

10%

10% children, 10% malignant, 10% not hypertensive

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11
Q

How are appendiceal neuroendicine neoplasms most frequently detected?

A

Incidentally during appendicectomy

(ENETS consensus guidelines 2016)

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12
Q

Which patients with incidentally identified neuroendocrine tumours at appendicectomy do not require further treatment?

A

<1cm size
Invasion up to subserosa or up to 3mm in mesoappendix
clear surgical margins

Those at base or larger also more likely to recur

(ENETS consensus guidelines 2016)

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13
Q

Where do appendiceal NENs most often appear?

A

70% at tip

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14
Q

What tumour markers are useful in appendiceal NEN?

A

Chromogranin A (CgA) - useful to differentiate from goblet cell carcinoma, follow up with metastatic

urinary 5-HIAA in rare patients with carcinoid

(ENETS consensus guidelines 2016)

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15
Q

How are Neuroendocrine tumours graded?

A

WHO 2010 classification

Ki67 Index (%) and Mitotic Rate (HPF)

G1 NET - Ki ≤2%, MR <2/10
G2 NET - KI 3-20%, MR 2-20/10
G3 NET - KI >20, MR >20

G3s - either poorly differentiated or carcinoma

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16
Q

What is the metastatic rate for appendiceal NETS >2cm?

A

about 40%

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17
Q

When should a right hemicolectomy be considered with appendiceal NETs 1-2cm in size?

A

if Base or R1,

or if risk factors - V1,L1, G2/3

(ENETS consensus guidelines 2016)

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18
Q

How often do small intestinal NETs present with metastasis?

A

50%

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19
Q

How do metastatic small intestinal NETs usually present?

A

With vague nonspecific symptoms - the carcinoid syndrome is still rare

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20
Q

What symptoms are associated with Carcinoid syndrome?

A

Secretory diarrhoea (60-80%)
Flushing (60-85%)
Intermittent bronchial wheezing (<10%)
Right heart valve fibrosis - Carcinoid Heart disease, Hedinger syndrome (20%)

Seen with liver metastatic disease bypassing hepatic clearance of serotonin

5% of patients can have retroperitoneal/ovarian tumours/metastases

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21
Q

How should patients be optimally investigated for small intestinal NET?

A

CT/MRI followed by Ga-DOTATOC PET or SRS Spect

For G3 a FDG PET may be useful

(ENETS consensus guidelines 2016)

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22
Q

What biochemical tests are useful in SI NETs?

A

Serum Chromogranin A
Urinary 5-HIAA (product of serotonin metabolism) highly sensitive

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23
Q

What are the types of well differentiated gastroduodenal NETs? (3)

A

Type 1 - ECLoma (Enterochromaffin like cell) - chronic atrophic gastritits
Type 2 - ECLoma -Zollinger Ellison
Type 3 - rare, sporadic, often high grade

If <1cm - endoscopic resection

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24
Q

How should rectal NETs be managed?

A

If <1cm - Endoscopic resection (unless G3)
>2cm - Anterior resection
1-2cm variable

(ENETS consensus guidelines 2016)

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25
How is Zollinger Ellison syndrome diagnosed?
Elevated fasting serum gastrin (x10 normal) in the presence of hypergastrinaemia when gastric pH<2 60-80% in duodenum (90-100% in those with MEN1) (ENETS consensus guidelines 2016)
26
How is an insulinoma diagnosed? (4)
Plasma glucose <3mmol Plasma insulin ≥3uU C-peptide ≥0.6ng Proinsulin ≥5pmol For non-functioning p_NETs Chromogranin A can be useful
27
How should pNETs be localised?
68GA labelled somatostatin analog PET-CT is better than SR SPECT, except for insulinoma - GLP1-receptor analogue scintigraphy or intra-arterial injection and measurement of some gradient
28
What surgical approach can frequently be used for insulinoma?
Enucleation
29
When should non-functioning P-nets be excised?
≥2cm when major pancreatic resection required
30
What are the types of appendiceal neoplasm?
1) Mucinous epithelial neoplasm -Serrated polyp -Low grade Mucinous neoplasm (LAMN) -High grade mucinous neoplasm (HAMN) -Mucinous adenocarcinoma 2) Non-mucinous epithelial neoplasm (adenoma/adenocarcinoma) 3) Epithelial with neuroendocrine features (NET or Goblet cell) 4) Mesenchymal neoplasms (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
31
What are the types of appendiceal neoplasm?
1) Mucinous epithelial neoplasm -Serrated polyp -Low grade Mucinous neoplasm (LAMN) -High grade mucinous neoplasm (HAMN) -Mucinous adenocarcinoma 2) Non-mucinous epithelial neoplasm (adenoma/adenocarcinoma) 3) Epithelial with neuroendocrine features (NET or Goblet cell) 4) Mesenchymal neoplasms (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
32
What tumour markers should be measured in appendiceal PMP?
CEA + CA19-9 +/- CA125 (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
33
What is the risk of synchronous tumours when colonic cancer is diagnosed?
3-5%
34
When should a right hemicolectomy be performed for goblet cell carcinoma of the appendix?
Any of: Not Tang A Tumour ≥20mm Involved margins Mesoappendix invasion ≥3mm V1/L1 Ki62>2% If perforated consider adjuvant CRS/HIPEC (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
35
If a pseudomyxoma is suspected as an incidental finding at surgery, how should it be managed?
Appendicectomy (?caecetomy?lymph node resection) + sampling of mucin Avoid right hemicolectomy (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
36
In patients where pseudomyxoma is suspected who are undergoing a laparoscopy, where should trocars be placed?
In the midline, so incisions can be excised (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
37
When is CRS/HIPEC indicated with a completely excised LAMN?
pM1a/pM1b - acellular/cellular mucin PCI≤3 A right hemicolectomy is not indicated (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
38
When should a right hemicolectomy be performed with a HAMN?
Probably in all cases, definitely if perforated. Similar for CRS and HIPEC Also for mucinous adenocarcinoma and GCC (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
39
What defines complete cytoreduction?
CC0 - no visible disease or CC1 - remaining nodules <2.5mm achieved in 73.8% --> 87.4% 5 year survival (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
40
What are the absolute (2) and relative (6) contraindications to cytoreductive surgery?
Absolute: - Extensive small bowel serosal involvement - Mesenteric involvement causing retraction Relative: - Age>75 - Aggressive histologies - high grade with signet ring, mucinous, GCC, PCI>20 - Involvement of liver hilum - Infiltration of anterior pancreatic surface - Ureteric obstruction - Need for total gastrectomy (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
41
How should ovaries be managed in patients with appendiceal PMP?
In postmenopausal women bilateral salpingoophrectomy, probably in premenopausal too (Peritoneal Surface Oncology Group International (PSOGI) Guidelines 2021)
42
What imaging is indicated for patients with suspected primary hyperparathyroidism?
Usually Ultrasound and Sestamibi scan. SPECT may be useful, particularly if reoperation is being considered If not localised or scans discordant, consider 4 gland exploration anyway, no need for further scans first. NICE (2019)
43
How can primary hyperparathyroidism be differentiated from familial hypocalciuric hypercalcaemia?
24 hour urinary calcium excretion or random renal calcium:creatinine excretion ratio Random serum calcium:creatinine clearance ratio (>0.01) NICE (2019)
44
When should surgery be considered in patients with primary hyperparathyroidism?
adjusted Ca>2.85 or symptoms of hypercalcaemia or end organ disease (renal stones, fragility fractures or osteoporosis - t score -2.5 or less
45
How should patients be followed up after parathyroidectomy?
adjusted Ca and PTH before discharge adjusted Ca at 3-6 months and then annually if normal
46
When is cinacalcet indicated for primary hyperparathyoidism?
If surgery failed or inappropriate and: aCa>2.85 + symptomatic aCa>3 regardless
47
Why does a thyroglossal cyst move on extension of tongue?
Arises from descent from foramen cecum of tongue
48
What are the likely positions of the superior parathyroid gland?
1) In fat pad on thyroid surface caudal to inferior thyroid artery 2) Inferiorly behind inferior thyroid artery and oesophagus 3) behind upper pole
49
What are the likely positions of the inferior parathyroid gland?
1) Along thyrothymic axis (30%) 2) Under capsule of lower thyroid pole (50%) 3) down to accessible mediastinal thymus 4) within carotid sheath 5) intrathymic 6) within thyroid lobe
50
What is the recommended first line treatment for Graves disease?
If mild and uncomplicated --> Carbimazole (12-18months) or radioiodine If severe radio iodine Surgery, only if concerns re compression, malignancy or others unsuitable NICE (2019)
51
What is the recommended first line treatment for toxic nodular goitre?
Radioactive iodine. If unsuitable, total thyroidectomy or antithyroid drugs NICE (2019)
52
When should propylthiouracil be considered over carbimazole?
- patients with allergy to carbimazole - pregnant or within 6 months - history of pancreatitis Do not use either if history of agranulocytosis NICE (2019)
53
What is Conns syndrome?
Primary aldosteronism - 33% adenoma, 66% bilateral adrenal hyperplasia For adenoma --> surgery, bilateral --> spironolactone Present with hypertension, depressed renin-angiotensin axis, hypokalaemia (increased urinarly losses) Plasma aldosterone:renin ratio is diagnostic about 5-10% of hypertensive patients
54
What is the embryological origin of the inferior parathyroids?
Third pharyngeal pouch, along with the thymus Supplied by glossopharyngeal nerve (IX)
55
What is the embryological origin of the superior parathyroids?
Fourth pharyngeal pouch, along with parafollicular thyroid C-cells + larynx supplied by superior laryngeal nerve (vagus derivative)
56
What is the staging peculiarity for patients with differentiated thyroid cancer?
Patients under 45 are all stage 1 if M0 and stage 2 if M1. Stages 1-3 have a 10 year survival of >98%
57
What is the incidence of permanent recurrent laryngeal nerve injury after total thyroidectomy?
0.2% 2% risk of permanent hypocalcaemia
58
What proportion of patients with sporadic hyperparathyroidism have a single adenoma to explain their symptoms?
85%
59
What is the most useful biomarker of medullary thyroid disease?
Calcitonin - derived from the parafollicular C-cells Can be used to screen in familial cases and monitor for metastatic disease
60
What proportion of patients with an adrenal nodule <4cm will have a carcinoma?
2% 25% >6cm Age, sex, FH and functional studies are not a reliable indicator of malignancy
61
What is the incidence of pheochromocytoma in adrenal incidentalomas?
about 5% 30% of phaeos are diagnosed in this fashion
62
What is the genetic abnormality in Li-Fraumeni syndrome?
Autosomal dominant p53 mutation - Sarcoma - Breast cancer - Leukaemia - Adrenocortical adenocarcinoma SBLA syndrome
63
What are the common manifestations of MEN-1?
Parathyroid, pituitary and pancreatic tumours, foregut carcinoids
64
What are the common manifestations of MEN2a?
Parathyroid adenomas, Medullary thyroid cancer, pheochromocytoma RET oncogene mutation
65
What are the common manifestations of MEN2b?
Mucosal neuromas, marfinoid habitus, Medullary thyroid cancer, pheochromocytoma RET oncogene mutation
66
How is malignant phaeochromocytoma diagnosed?
Requires presence of distant metastases Likelihood is increased by 3+ of Weiss criteria.
67
What is the other name for Chronic Lymphocytic thyroiditis?
Hashimotos disease (hypothyroidism, elevated thyroid autoantibodies)
68
What is deQuervain's thyroiditis?
Post viral thyroiditis - treat with NSAIDs +/- beta blockers if necessary
69
What is Reidel's thyroiditis?
Woody hard swelling of thyroid as an isolated abnormality F>M Usually treat with high dose steroids
70
Which type of thyroid cancer characteristically metastasises by the haematogenous route?
Medullary
71
When does tertiary hyperparathyroidism require surgery?
If persists >12 months beyond resolution of CRF - about 40%
72
How are thyroglossal cysts best treated?
Due to recurrent infection, formal excision with part of hyoid bone (Sistrunk procedure)
73
What is the gender disparity of thyroid nodules?
5:1 F:M
74
What are risk factors for a thyroid nodule being malignant?
- Age <20 or >60 - 'Firmness' - Rapid growth - Fixation to adjacent structures - Vocal cord paralysis - Lympadenopathy - History of neck irradiation - History of Hashimotos (lymphoma)
75
When should a core biopsy of a thyroid lump be performed?
If lymphoma is suspected, otherwise FNAC
76
How are USS findings for thyroid nodules graded?
U1-U5 FNAC for 3-5, discharge 1-2 unless other risk factors
77
How are thyroid nodule FNACs categorised?
Thy 1 - non diagnostic --> repeat Thy 2 - benign --> no follow up Thy 3F - follicular lesion --> hemithyroidectomy (with isthmus) or total thyroidectomy Thy 3A - atypia -> repeat, if still present --> hemithyroidectomy Thy 4 - suspicious --> diagnostic hemithyroidectomy Thy 5 - malignant --> total thyroidectomy
78
In PTC/FTC, when is hemithyroidectomy sufficient?
<4cm, age <45, no other high risk factors (inc not pT3/T4a)
79
When should a level 6 lymph node dissection be conducted in PTC?
High risk cancers, or if lateral or other nodes confirmed to be involved Cervical lymph nodes should be managed by selective node dissection For FTC, only conduct level 6 nodal dissection if nodes are clinically involved
80
What treatment do patients require after total thyroidectomy for cancer?
Suppressive doses of levothyroxine 20ug/kg or liothyronine Calcium check within 24 hours Thyroglobulin >6weeks post surgery and then 6-12 monthly
81
When is I131 ablation performed after total thyroidectomy for cancer?
In all cases, unless tumour <1cm or minimally invasive with either no high risk papillary forms or medullary
82
What proportion of cases of medullary thyroid cancer are familial?
25% (MEN2a, MEN2b, Familial MTC) - therefore screening for RET mutation
83
Where MTC is suspected what preoperative investigations are required?
Calcitonin, CEA Urinary metanephrines (24hr) Calcium and PTH CT/MRI/USS neck RET (?after surgery)
84
What is the recommended surgical treatment of medullary thyroid cancer?
Total thyroidectomy with central compartment node clearance (level 6), even in the presence of disseminated metastasis If there are central lymph node metastasis, ipsilateral neck dissection should be performed (70% involved) All T2-T4 tumours also have bilateral selective neck dissections (IIa-Vb)
85
What USS features are suggestive of thyroid malignancy?
Hypoechoity Microcalcifications Lymphadenopathy Loss of halo Irregular margins
86
What are the histological features of papillary thyroid cancers?
Most common sub type Psammoma bodies (microcalfication) Orphan Annie Nuclei Metastasise via lymphatics
87
What are the skin manifestations of Glucagonoma?
Necrolytic migratory erythema (70%) (erythematous blisters of abdomen/buttocks with irregular border and intact/ruptured vesicles) Serum glucagon of >1000pg/ml produced by alpha cells 90% malignant
88
How should incidental adrenal lesions be managed?
75% non-functioning adenomas Risk of malignancy related to size - if >4cm, 25% malignant If suspected metastasis then biopsy as long as not phaeo Otherwise excise or monitor Also needs: Cortisol (morning/midnight) 1mg overnightDexamethasone suppression test - Cushing? 24 hour urinary cortisol and metnephrines - Phaeo Serum potassium (low) aldosterone (high) and renin (low) levels - Conns
89
What is the risk of hypocalcaemia following thyroidectomy?
5% requiring treatment, 80% resolve over 12 months (therefore 1-2% at 12 months)
90
Which tumour type is most likely to metastasise to the thyroid?
Renal cell carcinoma - clear cell morphology
91
How does PTH cause its effects?
Secreted by parathyroid chief cells Increases calcium level by: Bone - Osteoblast binding --> osteoclasts --> bone resorption Kidney - active reabsorption of ca and mg from distal convoluted tubule Intestine via kidney - increasing activated Vitamin D Increases activity of 1-a-hydroxylase enzyme (converts 25-hydroxycalciferol to 1,25-dihydroxycholecalciferol)
92
Which antibiodies are seen in patients with Hashimotos and Graves disease?
Hashimotos - Thyroid peroxidase (100%) Graves - Thyroid peroxidase (70%), TSH receptor (95%)
93
For patients with suspected parathyroid cancer on USS, what is the optimum treatment?
Straight to surgery, no tissue diagnosis. Hemithyroidectomy + gland - 89% 5 year survival Gland only 53% 5 year survival
94
How does Aldosterone exert its effects?
Produced by Zona glomerulosa of adrenal Controls sodium reabsorption in distal renal tubule via Na+/K+ and H+ pumps
95
Where are thyroglossal cysts most commonly found?
Immediately Inferior to hyoid bone
96
What are the types of Cushings syndrome?
ACTH dependent - pituitary (Cushing disease) or ectopic (mostly lung ca) ACTH independent - adenoma/carcinoma of adrenal
97
How is Cushings disease investigated?
24 hour urinary cortisol Dexamethasone suppression test (CT/MRI)
98
What medical treatment is effective for advanced/mestastatic adrenal cortical cancers?
Mitotane
99
What histological findings are often seen in Follicular carcinoma of the thyroid?
Hurthle cells (adverse prognosis)
100
What is the most common presentation of Cushing syndrome?
Obesity 95% Hirsuitism/hypertension 80% Myopathy 60% Buffalo hump 55% Easy bruising 40%
101
Which thyroid tumour type is most common after non- therapeutic irradiation?
Papillary
102
What morphological charactestics on CT suggest a benign adrenal lesion?
Homogenous and lipid rich HU<10 and less than 4cm, no further imaging
103
In patients with indeterminate adrenal lesions, what are indications for excision?
Increase in size by >20% + at least 5mm in 6-12 months
104
What additional tests should be performed for patients with bilateral adrenal nodules?
17-hydroxyprogesterone (exclude CAH)
105
What is the most common aetiology of an adrenal incidentaloma?
80% Adenoma (75% non functioning, 12% cortisol secreting, 2.5% aldosterone) 7% Phaeochromocytoma 8% Adrenocortical carcinoma 5% Metastasis
106
What are the chraracteristic findings of benign adrenal lesions on CT?
<4cm, <10Hu, >60% contrast washout
107
What enzymes are affected in CAH?
11a hydroxlase - aldo/test 17a hydroxylase - aldo 21ahydroxylase - test
108
What is the chloride to phosphate ratio in hyperparathyroidism?
Elevated, at >33
109
Which neuroendocrine tumours tend to present at youngest age?
Appendiceal - median 39 (39% male) Most others are mid-late 60s
110
What is the most frequent type of NET?
Lung > SB > Appendix
111
Which NETs are more common in women (3)?
Appendix, caecum and lung Women have a globally much better prognosis than males? why
112
What are the causes of diarrhoea in patients with NET (6)?
Pancreatic malabsorption short bowel drug related bile salt related bacterial overgrowth hormonal (gastrin or 5-HT)
113
How should patients with NETS be managed perioperatively?
Consider octreotide IV/infusion If non functioning - IV if major surgery If history carcinoid will need IV for minor surgery (inc biopsies) and preop infusion for major
114
Where can NETs occur (3)?
1) Isolated neuroendocrine cells (bronchopulmonary, GI tract, skin) 2) Aggregates of neuroendocrine cells (pancreatic islets) 3) Classic endocrine glands (adrenal, pituitary)
115
What proportion of gastrointestinal NETSs are 'functioning'?
about 30% This is a difficult distinction though because they often release hormones when handled anyway
116
What proportion of patients with Small bowel NETs have carcinoid disease?
20% - 20% of these have carcinoid heart disease (check pro-BNP)
117
How long should patients with small bowel NETs be followed up for?
Minimum of 8 years - life
118
What is the differential diagnosis of an adrenal incidentaloma?
Functional -cortex - adenoma, nodular hyperplasia, carcinoma -medulla - phaeochromocytoma, ganglioneuroma/blastoma Non-functional -Adrenal masses - lipoma, cyst, haematoma, harmartoma, teratoma, amyloid, neurofibroma -Metastases - breast, lung, lymphoma, renal -Leukaemia
119
What investigations should be performed for an adrenal incidentaloma?
-Standard bloods -Urinary/plasma metanephrines -Overnight dexamethasone suppression test -If hypertensive renin/aldosterone levels -If virilised plasma sex steroids
120
What are the characteristics of a benign adrenal lesion on CT?
HU<10 and 60% contrast washout at 15 minutes
121
What are the indications for completion thyroidectomy after lobectomy for T3F?
If confirmed follicular carcinoma and any of: Large tumour >4cm Extracapsular extension/angioinvasion Hurthle cell (secretes thyroglobulin)
122
What proportion of Insulinomas are benign?
About 90% therefore can be treated with enucleation. CF glucagonomas, which are usuallyy malignant Best diagnostic test is EUS 5-10% have MEN1 75% of those with MEN1 will have Pancreatic Islet tumours
123
How should adrenal incidentalomas be assessed for cortisol excess?
1mg overnight Oral Dexamethasone test If Cortisol <50 - normal 51-138 - possible >138nmol/L probable (low ACTH)
124
What additional treatment will patients receiving Mitotane require?
Steroids - as lyses normal side as well
125
What proportion of U5 thyroid lesions are malignant?
80%
126
In which situations is monitoring of thyroglobulin inaccurate?
Remaining thyroid Positive thyroid antibodies (can monitor trend)
127
What proportion of patents with Graves disease have eye signs?
30% - chemises, proptosis, opthalomplegia (nb lid lag present in all thyrotoxicosis)
128
What are the broad indications for surgery in primary hyperparathyroidism?
Any <50 >50 if symptomatic or end organ damage
129
How often are Thy3f malignant on final histology?
About 20%
130
From which thyroid cells does thyroglobulin originate?
Follicular
131
Where is the largest store of body Calcium?
Bones!
132
What are the effects of PTH?
-To increase Ca and decrease PO4 -Immediate action on osteoblasts to increase Ca, also they signal the osteoclasts to resorb bone -Increase renal tubular absorption of caclium -Increased synthesis of 1,25(OH)2D --> bowel absorption -Decreased renal phosphate
133
Where is Calcitonin made?
C- cells of thyroid
134
Which type of parotid malignancy has the worst outcomes?
Adenoid cystic (5 year 35%) Acinic cell 80%, Adenocarcinoma up to 75%
135
Between which muscles does a pharyngeal pouch occur?
Thyropharyngeus and cricopharyngeus
136
From which branchial structure do branchial cysts originate?
2nd branchial cleft
137
What proportion of CO goes to the kidney?
About 25%
138
How is the GFR calculated?
(Conc of solute in urine x volume of urine/min)/plasma conc Total volume of plasma leaving capillaries --> Bowman's capsule
139
What substances are absorbed in the proximal convoluted tubule?
-Glucose, amino acid, phosphate co-transported with Na+ and water - Up to 2/3 of water
140
What substances are regulated at the distal convoluted tubule?
Sodium Potassium H+ Calcium
141
Describe the physiology of the Loop of Henle?
-Isotonic fluid enters LoH -Thin descending limb permeable to water but not Na/CL -Thin ascending limb impermeable to water but permeable to ions -Thick ascending limb active resorption of na and cl ions
142
What factors stimulate renin release?
From juxtaglomerular cells --> angiotensinogen to angiotensin 1 -Hypotension (reduced RPP) -Hyponatraemia -Sympathetic nerve stimuation -Catecholamines -Erect posture
143
What drugs reduce renin secretion?
Beta blockers NSAIDs
144
What factors are likely to make postoperative renal failure more likely?
-Elderly -PVD -High BMI -COPD -Vasopressors -Nephrotoxic medication -Emergency surgery
145
What are the characteristics of prerenal renal failure?
Kidneys retain sodium, Therefore Urine sodium low (<20mmol/l) Urine concentrated (>1.5 osmolality ratio) with high SG
146
What are the types of testicular cancer?
95% Germ cell tumours - Seminoma (commonest) -NSGT (42% - teratoma, yolk sac, choriocarcinoma, mixed germ cell)
147
What are the features of testicular seminoma?
Normal AFP HCG/LDH elevated in 10-20% Advanced disease 5- year 73% Sheet like lobular patterns of cells with fibrous component and septa.
148
What are the features of testicular NSGT?
Younger (20-30) Worse prognosis May need retroperitoneal lymph nod dissection AFP raised in 70% 40% HCG Heterogenous texture
149
What drug can characteristically cause epididymitis?
Amiodarone
150
What is the peak incidence of testicular torsion?
13-15 years
151
What is the most common cause of acute epidiymo-orchitis?
Chlamydia
152
What are indications for commencement of dialysis?
Intractable hyperkalaemia Acidosis Uraemia symptoms (nausea, pruritus, malaise) Therapy resistant fluid overload CKD 5
153
What are the borders of the anterior triangle of the neck?
Anterior border of SCM Lower border of Mandible Anterior midline
154
What sub triangles are contained within the anterior triangle?
Digastric Triangle (Submandibular gland/nodes, facial vessels, IX) Muscular triangle (Straps, EJV) Carotid Triangle (carotid sheath, ansa cervicali)
155
What is the blood supply of the parathyroids?
Inferior thyroid artery, inserting medially
156
What is the blood supply to the thyroid?
-Arterial Superior thyroid artery - 1st branch from external carotid artery (Superior Laryngeal Nerve) Inferior thyroid artery - thyrocervical trunk (RLN) -Venous Superior thyroid vein - IJV Middle thyroid vein - IJV Inferior thyroid vein - brachiocephalics
157
How does parasympathetic and sympathetic stimulation affect the parotid gland?
Para - water saliva Symp - enzymatic
158
What muscles are controlled by Trochlear and Abducens CNs?
SO 4 LR 6
159
Which is the largest cranial nerve?
Trigeminal - ophthalmic - maxillary - mandibular (sensory/motor)
160
What nerves supply taste to the tongue?
Anterior 2/3 - Facial Posterior 1/3 - Glossopharyngeal
161
What are the borders of the posterior triangle of the neck?
Anterior - post SCM Posterior - anterior Trap Base - middle 1/3 of clavicle
162
What are the contents of the posterior triangle of the neck?
-Nerves Accessory nerve Phrenic nerve Three trunks of brachial plexus - Vessels EJV SCA -Muscles Inferior belly of omohyoid Scalene
163
Which embryological structures does the vagus supply?
4th/6th pharyngeal arches (fore/midgut sections of embryonic gut tube)
164
What is the lymphatic drainage of the tongue?
Anterior 2/3 ipsilateral submental --> deep cervical Posterior 1/3 bilateral submandibular --> deepcervical
165
From which structures are the coverings of the spermatic cord formed?
Internal spermatic fascia --> Transversalis Cremastic fascia --> IO External spermatic fascia --> EOA
166
What are the contents of the spermatic cord?
Three vessels - Testicular artery - cremasteric artery - artery to vas Three other - vas deferens - pampiniform plexus - lymphatics Three nerves - genital branch of genitofemoral - sympathetic - ilioinguinal (on outside)
167
What are the four parts of the male urethra?
Pre-prostatic Prostatic Membranous (narrowest, external sphincter) Penile (longest) Transitional urothelium --> squamous distally
168
What are the histological findings for anaplastic thyroid carcinoma?
Rhabdoid cells, multi nucleation, nuclear pleomorphism
169
what is the most common pancreatic tumour in MEN1?
Gastrinoma > Insulinoma
170
On which bones is a DXA scan performed?
Radius Upper Lumbar spine Lower Lumbar spine Hip
171
What can increase 5 HIAA levels?
-Food Avocados Bananas Kiwi Plantains Tomatoes -Medicines Paracetamol
172
What can decrease 5HIAA levels?
Heparin MAOIs Aspirin
173
What is the agent of choice for metastatic medullary thyroid cancer?
Vandetanib (TKI) also cabozantinib
174
How should patients with adrenal cancer be followed up?
3 monthly CT for 2 years
175
What adjuvant treatment should be considered for adrenal cancers?
Mitotane if Ki67>10% + Radiotherapy if Stage 3/R1
176
In thyroidectomy, what factors increases the risk of postoperative hypocalcaemia?
Central node dissection (20-->35%)
177
What is the result of a low dose dexamethasone suppression test in Cushings?
Lack of suppression of cortisol
178
How are the results of the high dose dexamethasone suppression test interpreted?
If suppresses - Pituitary tumur (CD) If not then: -Ectopic ACTH -Adrenal adenoma
179
What is the most common presentation of hyperparathyroidism?
Fatigue (>stones/asymptomatic)
180
What happens to potassium with Cushings?
Hypokalaemia - weak mineralocorticoid effect
181
What is the most common cause of Cushings after steroids?
Pituitary adenoma
182
How frequently is a serum calcium of <2.1 on POD 1 seen after thyroidectomy?
25%
183
When should a paediatric hydrocele be fixed?
IF not going by 2-3 years
184
How should Thy1 results be managed?
Repeat FNAC, usually after 3 months to allow inflammation to settle
185
With a normal calcium on POD 1 after thyroidectomy, what supplemental calcium is required?
None
186
How should patients be prepared for radio iodine remnant ablation post thyroidectomy?
Suppression of TSH, so: -Levothyroxine (e.g. 100mcg OD) then stop 4 weeks prior -Liothyronine (e.g. 20mcg TDS) then stop 2 weeks prior -Low iodine diet post stopping -Could have levo followed by Lio if timings work -Could continue Levo then give recombinant TSH at 48/24hours prior
187
How do the parathyroids relate to the RLN?
Superior above and lateral Inferior below and medial
188
In which patients after thyroidectomy is hypocalcaemia most common?
Hungry bone syndrome - screen with ALP (also check and replace Vit D)
189
What is the blood supply to the adrenal?
Superior adrenal artery from inferior phrenic Middle adrenal artery from aorta Inferior adrenal artery from renal artery Right --> IVC Left --> Renal vein
190
What preoperative treatment should patients undergoing pituitary surgery receive?
Steroids
191
What history and examination findings are relevant in patients with adrenal incidentilomas?
Phaeo - anxiety, palpitations, headaches, hypertension Hyperaldosteronism - hypokalaemia, hypertension Hypercortisolism - diabetes, weight gain, hypertension FH - Endocrinopathy (MEN) Examination - obesity, facial plethora, hirsutism, proximal muscle weakness
192
How may adrenal incidentalomas be classified?
Functional Medulla- phaeo Cortex - adenoma, carcinoma Non-functional Metastasis, lipoma, cyst, haematoma
193
What features are important in a history and examination of thyroid goitre?
History --Upper airway symptoms (Cough, stridor, SOB, change in voice) --Dysphagia --Rapid growth --History of irradiation --Thyroid symptoms (heat/cold intolerance, weight loss/gain, insomnia/tiredness) --FH of MEN Examination - Dominant nodule - Cervical lymphadenopathy - retrosternal extension - bruit - tracheal deviation, signs of SVC/thoracic outlet obstruction - thyroid eye signs
194
How Is a thyroid uptake scan helpful in acute thyrotoxicosis?
Uptake bilateral - Graves Uptake unilateral - toxic nodule/adenoma No uptake - thyroiditis Carbimazole for 6 weeks, if urgent need then potassium iodide and propranolol for 7-10 days
195
What is Ki-67?
Marker of cell proliferation
196
What is the differential diagnosis of a parotid lump?
Benign --Pleomorphic adenoma --Warthin tumour --Lipoma --Neuroma Malignant Primary -- Adenoid cystic -- Adenocarcinoma -- Mucoepidermoid -- SCC -- Acinic cell carcinoma Malignant Secondary (SCC, Lymphoma)
197
How is MEN1 hyperparathyroidism characterised?
--Multigland disease, hyperplasia and adenomas. --Often ectopic and supernumerary --Subtotal/total + cervical thymectomy
198
What is the risk of complications after thyroidectomy?
Hypoparathyroidism/hypocalcaemia 20% transient, 4% permanent RLN damage 5-10% temporary, 2% permanent, 0.5% bilateral ((can treat with injection of vocal cords with autologous fat)) Superior laryngeal N. 0.5% Haemorrhage --> theatre 1-2%
199
How is recurrent Medullary Thyroid cancer managed?
Surgery if feasible Chemotherapy and radiotherapy can be used but less effective Tyrosine Kinase inhibitors e.g. Cabozantinib and Vandetinib now licensed
200
How is recurrent differentiated Thyroid cancer managed?
Papillary or follicular Surgery if feasible Chemotherapy or radio-iodine Can use Lenvatinib (VEGFR)/ sorafenib, then cabozantinib
201
What are the complications of adrenalectomy?
Overall about 10% Mortality <0.5% Major haemorrhage requiring transfusion 5% Damage to viscera - pancreas, liver, kidneys, diaphragm 2% Addisonian crisis <1%
202
What are the subdivisions of the anterior triangle of the neck?
Muscular Carotid (posterior belly of digastric) Submandibular Submental
203
What are the boundaries of level 6 neck nodes?
Inf- manubrium Sup - hyoid Ant - platysma Post - prevertebral fascia/trachea
204
What are branchial cleft cysts?
Anomalies arising from 1-4th branchial clefts Most common is 2nd (anteromedial to superior SCM), may be associated with IX and XII 1 - Parotid area, 3-middle SCM, 4 lower SCM Present as cysts, sinuses or fistulae Do USS +/- CT Excise (infection, malignancy), can do blue dye or fistula probes
205
What is the mechanism of carbimazole and how can it cause agranulocytosis?
Thyroid peroxidase inhibition (iodination of tyrosine residues in thyroglobulin) Causes agranulocytosis via direct toxicity with reactive oxygen species and possibly by immune reactions with ANCA
206
What are the types of nerve injury?
Sunderland Classification Grade 1 - neuropraxia Grade 2 - axontmesis Grade 3-5 - neurotmesis
207
How should you examine a neck?
--Inspection General – scars, cachexia, dyspnoea/stridor/hoarseness/exophthalmos Neck Lump – anterior/posterior triangle, movement on swallowing or tongue protrusion --Assessment of lump Site, size, shape, consistency, mobility/fixity Pulsatility/bruit, tenderness, transillumination --Palpate all lymph node basins --Examine thyroid from behind – general, tongue, swallow