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Endocrine > Adrenocortical Neoplasms > Flashcards

Adrenocortical Neoplasms Flashcards

1
Q

What is a phaeochromocytoma?

A

Catecholamine producing tumours that usually arise from the chromaffin cells of the adrenal medulla; derived from neural crest. Extra-adrenal in ~10% of cases.

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2
Q

What is a paraganglioma?

A

Extra-adrenal phaechromocytoma.
Also used to describe non-catecholamine-secreting tumours derived from parasympathetic paraganglia (e.g. carotid body chemodectomas).

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3
Q

Pattern of phaeochromocytoma secretion cf adrenal medulla?

A

Phaeochromocytoma: mainly secretes noradrenaline.
Adrenal medulla: mainly secretes adrenaline.
EXCEPT: familial phaeochromocytomas ==> may secrete large amounts of adrenaline.

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4
Q

Do phaechromocytomas show familail predisposition?

A

YES! Up to 30% of cases.

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5
Q

Percentage of HTN attributable to phaeochromocytomas?

A

0.2%

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6
Q

Conditions predisposing to familial phaeochromocytoma?

A
  • Multiple endocrine neoplasia (MEN 2a/2b)
  • von Hippel Lindau (VHL) syndrome
  • Succinate dehydrogenase (mitochondrial enzyme) gene mutations
  • Neurofibromatosis type 1
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7
Q

What is multiple endocrine neoplasia type 2a associated with?

A
  • Medullary thyroid carcinoma

- Hyperparathyroidism

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8
Q

What is multiple endocrine neoplasia type 2b associated with?

A
  • Medullary thyroid carcinoma

- Mucosal neuromas

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9
Q

Common clinical symptoms in patients with phaeochromocytoma?

A

COMMON =

i) Headache
ii) Sweating
iii) Palpitations
- CV: CP, dyspnoea (ischaemia / HF)
- GI: nausea, epigastric pain, constipation
- Neuropsychiatric tremor, weakness, anxiety

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10
Q

Clinical signs in patients w/ phaeochromocytoma?

A
  • Pallor
  • Tachycardia
  • Hypertension
  • Postural hypotension (due to reduced plasma volume)
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11
Q

Screening in suspected phaeochromocytoma?

A
  • MEN type 2: measure serum calcium and calcitonin
  • VHL: opthalmoscopy, MRI of posterior fossa and renal ultrasound
  • Neurofibromatosis type 1: examine for neurofibromas, cafe-au-lait spots, axillary frecling
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12
Q

What is a paraganglioma?

A

Extra-adrenal phaechromocytoma.
Also used to describe non-catecholamine-secreting tumours derived from parasympathetic paraganglia (e.g. carotid body chemodectomas).

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13
Q

Pattern of phaeochromocytoma secretion cf adrenal medulla?

A

Phaeochromocytoma: mainly secretes noradrenaline.
Adrenal medulla: mainly secretes adrenaline.
EXCEPT: familial phaeochromocytomas ==> may secrete large amounts of adrenaline.

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14
Q

Do phaechromocytomas show familail predisposition?

A

YES! Up to 30% of cases.

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15
Q

Conditions predisposing to familial phaeochromocytoma?

A
  • Multiple endocrine neoplasia (MEN 2a/2b)
  • von Hippel Lindau (VHL) syndrome
  • Succinate dehydrogenase (mitochondrial enzyme) gene mutations
  • Neurofibromatosis type 1
How well did you know this?
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16
Q

What is multiple endocrine neoplasia type 2a associated with?

A
  • Medullary thyroid carcinoma

- Hyperparathyroidism

17
Q

What is multiple endocrine neoplasia type 2b associated with?

A
  • Medullary thyroid carcinoma

- Mucosal neuromas

18
Q

Common clinical symptoms in patients with phaeochromocytoma?

A

COMMON =

i) Headache
ii) Sweating
iii) Palpitations
- CV: CP, dyspnoea (ischaemia / HF)
- GI: nausea, epigastric pain, constipation
- Neuropsychiatric tremor, weakness, anxiety

19
Q

Clinical signs in patients w/ phaeochromocytoma?

A
  • Pallor
  • Tachycardia
  • Hypertension
  • Postural hypotension (due to reduced plasma volume)
20
Q

Ix in suspected phaeochromocytoma?

A
  • 24h urinary catecholamines and metanephrines
  • Plasma free metanephrines
  • Imaging: CT / MRI
21
Q

Screening in suspected phaeochromocytoma?

A
  • MEN type 2: measure serum calcium and calcitonin
  • VHL: opthalmoscopy, MRI of posterior fossa and renal ultrasound
  • Neurofibromatosis type 1: examine for neurofibromas, cafe-au-lait spots, axillary frecling
22
Q

Pre operative preparation in patients with phaeochromocytoma?

A

-alpha blocker (e.g. phenoxybenzamine 40-100mg daily ==> increase dose until normotensive + postural hypotension + nasal stuffiness)
-followed by B-blocker (e.g. propranolol NEVER pre alpha)
-rehydration
2-3 weks pre surgery

Endocrine (21 decks)

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