Phaeochromocytoma is a neuroendocrine tumour that arises from chromaffin cells of the adrenal medulla . It is characterised by excessive catecholamine production , which causes the clinical triad of headache, diaphoresis, and tachycardia .
10% occur in children, 10% are bilateral, 10% are malignant, and 10% are extra-adrenal (paragangliomas). The most common extra-adrenal site is the organ of Zuckerkandl, which lies adjacent to the aortic bifurcation.
Clinical features of phaeochromocytoma
Category Clinical features Cardiovascular Tachycardia, palpitations, ventricular tachycardia, dyspnea, fainting, angina , myocardial infarction , left ventricular hypertrophy, cardiomyopathy Neurological Headache, visual disturbance, dizziness, tremor, numbness, convulsions, encephalopathy, Horner’s syndrome (due to a paraganglioma), subarachnoid hemorrhage Psychological Anxiety, panic, hyperactivity, confusion, episodic psychosis Gastrointestinal Diarrhoea, vomiting, abdominal pain, abdominal mass, mesenteric vasoconstriction Others Sweating, flushing, heat intolerance, pallor, hyperthermia, backache, haemoptysis
Associated conditions
Sporadic (90%)
MEN2
Neurofibromatosis
Von Hippel-Lindau Syndrome
Signs and symptoms
Asymptomatic (incidentaloma)
Resistant hypertension
Sustained or paroxysmal (fluctuating blood pressure)
Precipitated by exertion, stress, abdominal palpation, or beta-blockers
Headache
Paliptations
Diaphoresis
Anxiety
Pallor or flushing
Tremor
A sense of impending doom
Investigations
Plasma free metanephrines and normetanephrines for screening
24-hour urinary collection of metanephrines/metadrenalines
High-resolution CT scan or MRI for localization
Metaiodobenzylguanidine (MIBG) scintigraphy or PET scan for metastatic or extra-adrenal disease
Genetic testing
Treatment
Blockade of catecholamines
Alpha-blockade with phenoxybenzamine, prazosin, or doxazosin first to prevent unopposed vasoconstriction
Beta-blockade with propranolol once blood pressure has been normalized
Adrenalectomy
Pre-operative blockade of catecholamines
Biochemistry
ACTH P: <80 ng/L ALT P: 5–35 U/L Albumin P: 35–50 g/L Aldosterone P: 100–500 pmol/L Alk. phosphatase P: 30–130 U/L α-Amylase P: 0–180 IU/dL α-Fetoprotein S: <10 kU/L Angiotensin II P: 5–35 pmol/L ADH P: 0.9–4.6 pmol/L AST P: 5–35 U/L Bicarbonate P: 24–30 mmol/L Bilirubin P: 3–17 μmol/L BNP P: <50 ng/L CRP P: <10 mg/L Calcitonin P: <0.1 mcg/L Calcium (ionized) P: 1.0–1.25 mmol/L Calcium (total) P: 2.12–2.60 mmol/L Chloride P: 95–105 mmol/L Cholesterol P: <5.0 mmol/L VLDL P: 0.128–0.645 mmol/L LDL P: <2.0 mmol/L HDL P: 0.9–1.93 mmol/L Cortisol AM P: 450–700 nmol/L Cortisol Midnight P: 80–280 nmol/L CK ♂ P: 25–195 U/L CK ♀ P: 25–170 U/L Creatinine P: 70–100 μmol/L Ferritin P: 12–200 mcg/L Folate S: 2.1 mcg/L FSH P: 2–8 U/L ♂; >25 menopause GGT ♂ P: 11–51 U/L GGT ♀ P: 7–33 U/L Glucose (fasting) P: 3.5–5.5 mmol/L Growth hormone P: <20 mu/L HbA1C (DCCT) B: 4–6% HbA1C (IFCC) B: 20–42 mmol/mol Iron ♂ S: 14–31 μmol/L Iron ♀ S: 11–30 μmol/L Lactate (venous) P: 0.6–2.4 mmol/L Lactate (arterial) P: 0.6–1.8 mmol/L LDH P: 70–250 U/L LH P: 3–16 U/L Magnesium P: 0.75–1.05 mmol/L Osmolality P: 278–305 mosmol/kg PTH P: 0.8–8.5 pmol/L Potassium P: 3.5–5.3 mmol/L Prolactin ♂ P: <450 U/L Prolactin ♀ P: <600 U/L PSA P: 0–4 mcg/mL Protein (total) P: 60–80 g/L Red cell folate B: 0.36–1.44 μmol/L Renin (erect) P: 2.8–4.5 pmol/mL/h Renin (recumbent) P: 1.1–2.7 pmol/mL/h Sodium P: 135–145 mmol/L TBG P: 7–17 mg/L TSH P: 0.5–4.2 mU/L T4 P: 70–140 nmol/L Free T4 P: 9–22 pmol/L TIBC S: 54–75 μmol/L Triglycerides P: 0.50–2.3 mmol/L T3 P: 1.2–3.0 nmol/L Troponin T P: <0.1 mcg/L Urate ♂ P: 210–480 μmol/L Urate ♀ P: 150–390 μmol/L Urea P: 2.5–6.7 mmol/L Vitamin B12 S: 0.13–0.68 nmol/L Vitamin D S: 50 nmol/L
Arterial Blood Gases
pH 7.35–7.45 PaCO₂ 4.7–6.0 kPa PaO₂ >10.6 kPa Base excess ±2 mmol/L
Urine
Cortisol (free) <280 nmol/24h Hydroxyindole acetic acid 16–73 μmol/24h Hydroxymethylmandelic acid 16–48 μmol/24h Metanephrines 0.03–0.69 μmol/mmol cr. Osmolality 350–1000 mosmol/kg 17-Oxogenic steroids ♂ 28–30 μmol/24h 17-Oxogenic steroids ♀ 21–66 μmol/24h 17-Oxosteroids ♂ 17–76 μmol/24h 17-Oxosteroids ♀ 14–59 μmol/24h Phosphate (inorganic) 15–50 mmol/24h Potassium 14–120 mmol/24h Protein <150 mg/24h Protein/creatinine ratio <3 mg/mmol Sodium 100–250 mmol/24h
Haematology
WCC 4.0–11.0 ×10⁹/L RBC ♂ 4.5–6.5 ×10¹²/L RBC ♀ 3.9–5.6 ×10¹²/L Hb ♂ 130–180 g/L Hb ♀ 115–160 g/L PCV ♂ 0.4–0.54 L/L PCV ♀ 0.37–0.47 L/L MCV 76–96 fL MCH 27–32 pg MCHC 300–360 g/L RDW 11.6–14.6% Neutrophils 2.0–7.5 ×10⁹/L (40–75%) Lymphocytes 1.0–4.5 ×10⁹/L (20–45%) Eosinophils 0.04–0.44 ×10⁹/L (1–6%) Basophils 0–0.10 ×10⁹/L (0–1%) Monocytes 0.2–0.8 ×10⁹/L (2–10%) Platelets 150–400 ×10⁹/L Reticulocytes 0.8–2.0% / 25–100 ×10⁹/L Prothrombin time 10–14 s APTT 35–45 s
Paediatric
Pulse Rate (bpm) Neonate 140–160 Infant <1yr 120–140 1–5 years 110–130 5–12 years 80–120 >12 years 70–100 Respiratory Rate (tachypnoea) 0–2 months ≥60/min 2–12 months ≥50/min 1–5 years ≥40/min >5 years ≥30/min Blood Pressure (mmHg) Term 65/45 1 year 75/50 4 years 85/60 8 years 95/65 10 years 100/70 Weight Formulas 3–12 months (a + 9)/2 kg 1–6 years 2a + 8 kg >6 years (7a − 5)/2 kg Haemoglobin (g/dL) Term newborn 13–20 1 month 11–18 2 months 10–15 1–2 years 10–13 >2 years 11–14 MUAC (6 months–5 years) Obese >17.5 cm Normal 13.5–17.4 cm At risk 12.5–13.4 cm Moderate malnutrition 11.5–12.4 cm Severe malnutrition <11.5 cm Developmental Milestones Social smile 1.5 months Head control 4 months Sits unsupported 7 months Crawls 10 months Stands unsupported 10–12 months Walks 12–13 months Talks 18 months CSF WBC (/mm³) Term newborn 0–25 >2 weeks 0–5
