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Phaeochromocytoma

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Phaeochromocytoma

  1. 1. Phaeochromocytoma
  2. 2. <ul><li>This us a rare catecholamine- producing tumour, </li></ul><ul><li>and a treatable cause of hypertension (incidence <1%). </li></ul><ul><li>90% are in the adrenal medulla, 90% benign . </li></ul><ul><li>and these are usually unilateral 90%). </li></ul><ul><li>Sometimes inherited - autosomal dominant. </li></ul>
  3. 3. Association: <ul><li>multiple endocrine neoplasia (Men Type Il). </li></ul><ul><li>Tumours outside the medulla may be in paraganglia ( = phaeochrome bodies, i.e collections of epinephirine - secreting chromaffin cells) - typically by the aortic bifurcation (organ of Zuckerkandl). </li></ul>
  4. 4. Symptoms and signs: <ul><li>Episodic hypertension, </li></ul><ul><li>chest tightness, </li></ul><ul><li>restlessness, </li></ul><ul><li>anxiety, </li></ul><ul><li>pallor, </li></ul><ul><li>and weakness. </li></ul>
  5. 5. Test: <ul><li>Glycosuria during attacks in 30%. </li></ul><ul><li>Screening: </li></ul><ul><li>24h urine collection for </li></ul><ul><li>4-OH-3 methoxymandelate (HMMA, VMA) or total ( or free) metadrenalines . </li></ul>
  6. 6. Full investigations: <ul><li>consult specialist centre: </li></ul><ul><li>consider MIB scan (metadrenalines benzylguanidine) or clonidine suppression test & abdomen CT/MRI Scan. </li></ul>
  7. 7. Treatment Surgery: <ul><li>Careful BP control for 2 weeks pre-alfa-blocker (phenoxybenzamine before B-blockers (Propranolol). </li></ul><ul><li>Consult anaesthetist. </li></ul><ul><li>Post operative collection 24 h urine as above; monitor BP (risk of BP↓↓) </li></ul>
  8. 8. The clinical features of Phaeochromocytoma <ul><li>features including: </li></ul><ul><li>• Chest tightness • Skin mottling. </li></ul><ul><li>• Abdominal pain • Palpitations </li></ul><ul><li>• Spots b the e • Weight loss </li></ul><ul><li>• Tremor • Pallor </li></ul><ul><li>• Pins and needles • Dyspnoea </li></ul><ul><li>• Cold feet • Claudicarion </li></ul>
  9. 9. <ul><li>a Herninopia </li></ul><ul><li>Purpura </li></ul><ul><li>a Vomiting </li></ul><ul><li>• Flushing </li></ul><ul><li>• Pulsatile scotomas </li></ul><ul><li>• Sweating </li></ul><ul><li>• Faints (postural at drop) </li></ul>
  10. 10. <ul><li>Symptoms are precipitated by stretching, </li></ul><ul><li>sneezing, </li></ul><ul><li>stress, </li></ul><ul><li>sex, </li></ul><ul><li>smoking surgery, </li></ul><ul><li>or parturiti by agents such as cheese, </li></ul><ul><li>alcohol, </li></ul><ul><li>or the ti-i cyclic you so kindly prescribed, </li></ul><ul><li>thinking that the patient’s bizarre symptoms were only explicable by depression. </li></ul>
  11. 11. <ul><li>These causes may last for a few minutes or a week. </li></ul><ul><li>Suddenly the patient may feel ‘as it about to die’—and then gets better, </li></ul><ul><li>or goes on to stroke or cardiovascular collapse. </li></ul><ul><li>On examination, there may be no signs, </li></ul><ul><li>or hypertension (± signs of cardiomyopathy or heart failure) and thyroid swelling (episodic) and glycosuria during an attack—or terminal haematuria from a bladder phaeochromocytoma. </li></ul>

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