Pheochromocytomas

Question 1

D?

Answer 1

A neuroendocrine tumour from catecholamine-producing enterochromaffin cells of the adrenal medulla that presents with several symptoms.

Question 2

RF?

Answer 2

• MEN 2A and 2B
• Von Hippel Lindau Disease
• SDH BCD mutations

Question 3

ddx?

Answer 3

• Anxiety and panic attacks
• Essential HT
• Hyperthyroidism
• Drugs
• Carcinoid syndrome-flushed skin
• Arrhythmias
• Menopause
• Pre-eclampsia

Question 4

Epidemiology?

Answer 4

Age: 30-50
Sex: same
Ethnicity:-
Prevalence:

Question 5

Aetiology?

Answer 5

Tumour of enterochromaffin cells , mostly in adrenal medulla

Question 6

CP?

Answer 6

• Episodic hypertension
• Throbbing ehadache
• Diaphoresis
• Palpitations
• Pallor
• Abdo pain
• Anxiety
• Weight loss
• Hyperglycaemia
• Signs of familial disorders

Question 7

Pathophysiology?

Answer 7

• Enterochromaffin cells undergo mutations that allow uncontrolled cell division
• They secrete excess catecholamines, which are converted by COMT into metanephrines and normetanephrines
• Increased symptoms due to a and b receptors stimulation.
• Extra-adrenal-arise in paraganglion chromaffin tissue of ANS-similar symptoms
Malignant-same structure but mroe malignant and mets to liver, LN and bone

Question 8

I-first line?

Answer 8

• 24 hr urine collection for catecholamines and metanephrines, normetanephrines (gold standard) and creatinine
• Serum free
• Plasma catecholamines
• Genetic testing

Question 9

I-second line?

Answer 9

• Serum electrolytes-high Ca, low K
• MRI/CT
• Chromogranin A-elevated
• Increased uptake of a radiolabelled catecholamine precursor in the site of the tumour
• meta-iodobenzyl guanidine (MIBG, I-131 or 123) scintigraphy has been used to identify metastases, recurrent tumors and tumors in rare sites such as the pericardium and urinary bladder

Question 10

M-hypertensive crisis?

Answer 10

• Anti-hypertensive agents

Phentolamine -CCB

Question 11

M-non-hypertensive crisis?

Answer 11

• Alpha blockers-phenoxybenzamine
• Beta blockers-atenolol
• Hydration and high salt diet
• CCB (nifedipine)/metirosine
• Surgical excision of tumour
  
  • Surgical treatment options include open or laparoscopic total adrenalectomy, adrenal-sparing, or partial adrenalectomy. Robot-assisted adrenalectomy is another safe and effective surgical treatment option
• Chemo and radiotherapy-cyclophosphamide
  
  • Iobenguane I-131

Question 12

Prognosis?

Answer 12

• 95% 5 yr survival rate if benign
• Will need follow-up for 10 yrs-BP management
42% 5yr survival rate if malignant-better if younger, female and diagnosed early

Question 13

Complications?

Answer 13

• HT crisis
• Neuro-altered status, strokes
• Post-op-hypotension, arrhythmias, hypoglycaemia