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Pheochromocytoma: Associations, Clinical features, Investigations Treatment (Rule of Ten+Mnemonics)

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Pheochromocytoma/ Phaeochromocytoma is an adrenal medulla tumor.

In this video we’re going to learn about phyochromocytoma this is the tumor arising from adrenal gland or suprarenal gland which lies above the kidney cross section of superannual gland shows an outer part called adrenal cortex and inner part called adrenal medulla the medulla is composed of chromafin cells that secrete catecholamines and the tumor arising from

These cells is called pheochromocytoma the chromafine cells are also found in other areas like organ of jocker candle urinary bladder periodic areas and sometimes in the neck as well now the tumor arising from chromophin cells on these areas are called paragangliomas or extra adrenal fuel chromocytoma few chromosytuma association with the following hereditary

Syndromes first we have multiple endocrine neoplasia 2a also known as sepal syndrome or multiple endocrine neoplasia 2b it is an autosomal dominant disorder associated with an activating germline mutation of the ret proton cuisine on chromosome 10. next one is von hipperlindo’s disease it is also an autosomal dominant disorder associated with an inactivating

Mutation of the one hippolyndo tumor suppressor gene on chromosome 3. next is von wreckling nelson’s disease or neurofibromatosis type 1. this is also an autosomal dominant disorder associated with an inactivating mutation of the nf1 tumor suppressor gene on chromosome 17. pheochromocytoma is a tumor arising from chromafine cells and chromophene cells secrete

Catecholamines so this is a catecholamine secreting tumor and catecholamines include adrenaline nor adrenaline and dopamine it could be benign or malignant however it’s very difficult to differentiate these two on the basis of histology the definitive diagnosis of malignancy in feochromocyte is based exclusively on the presence of metastases which may involve

Regional lymph nodes liver lung and bone something called a rule of ten can be applied in case of feochromocytoma forbid understanding of the tumor let’s see what are they ten percent cases of pheochromocytoma are malignant ten percent occur in children’s ten percent tumor calcified ten percent are not associated with hypertension which means ninety percent people

With fear chromocytoma have hypertension ten percent are extra adrenal that is found in other organs rather than adrenal gland like organ of jacquard candle bladder etc as i’ve explained early ten percent are familial cases and ten percent of your chromosytoma are bilateral that is it occurs in both adrenal glands and you can remember these as a mnemonic master

Safe britain now let’s move on to the clinical features clinical features of fear chromosytoma depends upon the pattern of catecholamine secretion and because it does the passion can be intermittently or continuously symptomatic or even asymptomatic hypertension is the dominant clinical manifestation of theochromocytoma and it usually demonstrates paroxysmal

Episodes associated with tachycardia palpitations headache sweating tremor and pallor or the easy way to remember the clinical features is as 5p which stands for tracer or increase in blood pressure pain stands for headache perspiration palpitations and pallor the presentation may also be with the complication of hypertension such as stroke myocardial infarction

Left ventricular failure and hypertensive retinopathy the tumor cells of pheochromocytoma may also secrete erythropoietin which is a hormone primarily secreted by the kidneys it plays a key role in the production of red blood cells hence this may leads to polycythemia which means increase in number of rbc now let’s move on to the investigation part measurement of

Metabolites of catecholamines in plasma or urine will allow confirmation of the diagnosis of geochromocytoma catecholamines are rapidly inactivated and metabolized by enzymes such as catechol methyl transferase and monoamine oxidase found in many tissues and the metabolic products like metanephrine nor metanephrine and vanily mandelic acid are produced production

Of catecholamines is increased in feochromocytoma hence the level of metabolites are also elevated measurement of such metabolites confirm the diagnosis but there is high false positive rate as metanephrine’s level may also be elevated in stressed persons or due to injection of drugs such as tricyclic antidepressants for this region a repeat sample test should

Usually be requested if elevated levels are found after the diagnosis is confirmed the tumor can be localized by ct scan or mri now on to the management as soon as the biochemical diagnosis is confirmed the patient should be commenced on alpha receptor antagonists phenoxy benzamine or doxazosin beta blockers are only indicated if tachycardia develops and should

Not be instituted until the passing is fully alpha blocked because of the risk of hypertensive crisis in surgery laparoscopic adrenalectomy for adrenal tumors performed by the transperitoneal or retroperitoneal root is the gold standard you

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Pheochromocytoma: Associations, Clinical features, Investigations Treatment (Rule of Ten+Mnemonics) By DipMED