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Phaeochromocytoma

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Abstract

Phaeochromocytomas are rare adrenal tumours characterized by the overproduction of catecholamines. They are often misdiagnosed due to their non-specific clinical signs. This chapter focuses primarily on the canine disease as phaeochromocytoma is extremely rare in cats. The chapter explains the process of catecholamine synthesis and metabolism within the adrenal gland, as well as the clinical signs and diagnostic imaging techniques used to detect phaeochromocytoma. Treatment options are addressed, including adrenalectomy and medical management with tyrosine kinase inhibitors.

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Figures

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33.1 The biosynthetic pathway and metabolism of catecholamines within the adrenal gland. The catecholamines are metabolized by the catechol--methyltransferase (COMT) enzyme and can be measured as free normetanephrine and metanephrine in plasma. In the gastrointestinal tract, kidneys and liver, sulphate conjugation by sulphotransferase enzyme (SULTIA3) takes place. AADC = aromatic amino acid decarboxylase; DBH = dopamine beta-hydroxylase; -DOPA = 3,4-dihydroxyphenylalanine; PNMT = phenylethanolamine--methyltransferase; TH = tyrosine hydroxylase. (Modified from )
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33.2 The pathway of catecholamine synthesis, metabolism and secretion in adrenal chromaffin cells. The secretion of normetanephrine and metanephrine is a continuous action independent of adrenaline and noradrenaline release. -DOPA = 3,4-dihydroxyphenylalanine. (Modified from )
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33.4 (a) Transverse computed tomographic image of a phaeochromocytoma (arrowed) after intravenous administration of contrast medium in an 8‐year‐old dog. Severe ingrowth in the vena cava is visible (arrowheads). (bi) The tumour was successfully removed. (bii) The cut surface of the adrenal mass, comprising the adrenal gland and tumour thrombus removed from the vena cava.
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33.5 Urinary normetanephrine:creatinine in healthy dogs, dogs with hypercortisolism (HC), dogs with non-adrenal diseases (NAD) and dogs with phaeochromocytoma (Phaeo). Significantly higher urinary ratios were measured in dogs with phaeochromocytoma compared with healthy dogs and dogs with hypercortisolism.
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33.6 Scatter plots of free plasma (a) metanephrine and (b) normetanephrine concentrations in healthy dogs, dogs with non-adrenal disease, dogs with adrenocortical tumours (Adrenal) and dogs with phaeochromocytoma (Phaeo). The statistical significance between the phaeochromocytoma group and the other groups is shown below the X axis. (Modified from )
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