Medical history (history of illness) represents an important component in the diagnosis of pheochromocytoma. Family history Are there any genetic diseases in your family? Do any people in your family suffer from kidney or adrenal diseases? Current medical history/systemic history (somatic and psychological complaints). What symptoms/complaints have you noticed? Abdominal pain? Flank pain? Persistently elevated … Pheochromocytoma: Medical History
Endocrine, nutritional, and metabolic diseases (E00-E90). In hyperglycemia (high blood sugar): think diabetes mellitus! Hyperthyroidism (hyperthyroidism). Cardiovascular system (I00-I99) Apoplexy (stroke) Hypertensive encephalopathy – hypertensive emergency characterized by an increase in intracranial (within the skull) pressure with consequent intracranial pressure signs. Hypertensive crisis (blood pressure values > 230/120 mmHg) of other genesis. Intracranial hemorrhage (bleeding … Pheochromocytoma: Or something else? Differential Diagnosis
General measures Nicotine restriction (refraining from tobacco use). Limited alcohol consumption (men: max. 25 g alcohol per day; women: max. 12 g alcohol per day). Aim for normal weight! Determination of BMI (body mass index, body mass index) or body composition using electrical impedance analysis. Falling below the BMI lower limit (from the age of … Pheochromocytoma: Therapy
Obligatory medical device diagnostics. Medical device diagnostics are used when a pheochromocytoma is confirmed by laboratory diagnosis and are used to determine the location of the pheochromocytoma. Computed tomography (CT) of the abdomen (abdominal CT) or magnetic resonance imaging (abdominal MRI) – sensitivity (percentage of diseased patients in whom the disease is detected by use … Pheochromocytoma: Diagnostic Tests
Initially, an attempt should be made to remove the pheochromocytoma laparoscopically (by laparoscopy), i.e., minimally invasively. This procedure can be used in the majority of cases. If this is not possible, for example, because of the tumor size or because it is difficult to access, invasive surgery will be required: Unilateral adrenalectomy (removal of the … Pheochromocytoma: Surgical Therapy
The following symptoms and complaints may indicate pheochromocytoma: Main symptoms Hypertension (high blood pressure) Paroxysmal hypertension (seizure-like increase in blood pressure) with hypertensive crises (high blood pressure crises) that can last from a few minutes to hours – 40-60% in adults Persistent (ongoing) hypertension – 50-60% in adults, up to 90% in children! Symptoms in … Pheochromocytoma: Symptoms, Complaints, Signs
Pathogenesis (disease development) Pheochromocytoma is a neuroendocrine (affecting the nervous system) catecholamine-producing tumor of the chromaffin cells of the adrenal medulla (85% of cases) or sympathetic ganglia (nerve cord that runs along the spine in the thoracic (chest) and abdominal (stomach) regions) (15% of cases). The latter is also called extraadrenal (outside the adrenal gland) … Pheochromocytoma: Causes
The following are the most important diseases or complications that may be contributed to by pheochromocytoma: Endocrine, nutritional, and metabolic diseases (E00-E90). Diabetes mellitus type 2 – favored by inhibition of insulin secretion by epinephrine. Cardiovascular system (I00-I99). Intracranial hemorrhage (hemorrhage within the skull; parenchymal, subarachnoid, sub- and epidural, and supra- and infratentorial hemorrhages)/intracerebral hemorrhage … Pheochromocytoma: Complications
Classification of familial pheochromocytoma. Syndrome Gene Locus Exons Phaeo Malignancy (malignancy) Multiple endocrine neoplasia type 2a/b RET 12q 11.2 21 50 % 3-5 % Von Hippel-Lindau syndrome VHL 3p 25-26 3 20 % 5 % Recklinghausen’s disease (neurofibromatosis type I) Nf1 17q 11.2 59 2 % 10 % Paraganglioma syndrome type 1 SDHD 11q 23 … Pheochromocytoma: Classification
A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body weight, height; further: Inspection (viewing) of skin and mucous membranes [pallor; sweating]. Auscultation (listening) of the heart [palpitations (heartbeat)] Examination of the abdomen (abdomen) (tenderness?, palpitation?, cough pain?, guarding?, hernial orifices?, renal bearing palpation?) … Pheochromocytoma: Examination
Biochemical screening to detect overproduction of catecholamines (epinephrine, norepinephrine) should be performed in patients with: New-onset refractory hypertension (hypertension that does not respond to medical therapy). Paradoxical blood pressure response during anesthesia or surgical procedures. Hereditary (congenital) predisposition regarding a pheochromocytoma. Sudden panic attacks As well as in asymptomatic patients with incidentaloma (accidentally discovered neoplasms) … Pheochromocytoma: Test and Diagnosis
Therapeutic targets Therapy and prevention of hypertensive crises (hypertension crises). Prevention of complications Therapy recommendations For acute hypertensive crises: Nitroprusside sodium (“nitro spray”). Preoperative blockade of alpha receptors using phenoxybenzamine (10 days before surgery). If the patient cannot undergo surgery: Therapy with alpha blockers (to prevent hypertensive crises): phenoxybenzamine, prazosin (via inhibition of tyrosine hydroxylase, … Pheochromocytoma: Drug Therapy