Pheochromocytoma Disease

DESCRIPTION
Catecholamine-producing tumor. In 90% of cases, the tumors are found in the adrenal medulla, but may also be found in other tissues derived from neural crest cells.
  • System(s) affected: Endocrine/Metabolic
  • Genetics: N/A
  • Incidence/Prevalence in USA: 0.01% to 0.1% of the hypertensive population
  • Predominant age: Any age, peak incidence ages 30 to 60 years
  • Predominant sex: Male = Female
SIGNS AND SYMPTOMS
  • Paroxysmal spells (the "5 P's")
    • Pressure - sudden increase in blood pressure
    • Pain - headache, chest and abdominal pain
    • Perspiration
    • Palpitation
    • Pallor
  • Additional symptoms
    • Constipation
    • Tremor
    • Weight loss
    • Anxiety
  • Signs
    • Hypertension - paroxysmal in half of the patients
    • Orthostatic hypotension
    • Grade II to IV retinopathy
    • Fever
    • Hyperglycemia
    • Hypercalcemia
    • Erythrocytosis
    • 10% of patients are asymptomatic
CAUSES
  • Catecholamine-producing tumor - "Rule of 10:"
    • 10% are extra-adrenal
    • 10% are multiple or bilateral
    • 10% are malignant
    • 10% recur after surgical removal
    • 10% occur in children
    • 10% are familial
    • 10% present as adrenal incidentalomas
RISK FACTORS
  • Familial pheochromocytoma
  • Multiple endocrine neoplasia types II A and B
  • Neurofibromatosis
  • Von Hippel-Lindau syndrome
LABORATORY
  • Elevated 24-h urine metanephrine
  • Elevated 24-h urine or plasma catecholamines measured by high performance liquid chromatography (HPLC)
  • If results equivocal or normal, repeat 24-h urine collection with a spell
Drugs that may alter lab results:
  • Increased by:
    • Amphetamines
    • Tricyclic antidepressants
    • Clonidine or other drug withdrawal
    • Labetalol
    • Ethanol
    • Methyldopa
    • Sotalol
    • Levodopa
  • Decreased by:
    • Central alpha-2 agonists
    • Reserpine
Disorders that may alter lab results: Major physical stress (e.g., surgery, stroke)
PATHOLOGICAL FINDINGS
Catecholamine-producing tumor in the adrenal medulla, para-aortic sympathetic chain, wall of the urinary bladder, sympathetic chain in the neck or mediastinum
SPECIAL TESTS
Clonidine suppression test, suppression/provocative tests
IMAGING
  • Computerized abdominal imaging (MRI preferred over CT)
  • I-123: I-metaiodobenzylguanidine scan
  • In-111: pentetreotide scan
DIAGNOSTIC PROCEDURES

N/A

APPROPRIATE HEALTH CARE

Inpatient surgery

GENERAL MEASURES
  • Combined alpha- and beta-adrenergic blockade
  • Cardiovascular and hemodynamic variables must be monitored closely
SURGICAL MEASURES
  • High risk surgical procedure
  • Experienced surgeon/anesthesiologist team required
ACTIVITY
  • Exercise alone rarely causes significant weight loss, but may improve long term results of weight loss treatment and should be an integral part of any weight loss program.
  • Exercise regimens involve 30 minutes 3-5 times/week. Increasing calories expended in activities throughout the day is also important.
  • Many patients benefit from an "exercise prescription"
DIET

High in salt content preoperatively

PATIENT EDUCATION

National Adrenal Disease Foundation (NADF), 505 Northern Blvd, Great Neck, NY 11021; 516-407-4992; e-mail: nadf@aol.com

PREVENTION/AVOIDANCE

N/A

POSSIBLE COMPLICATIONS
  • Postural hypotension with alpha-adrenergic blockade
  • Pulmonary edema with beta-adrenergic blockade
  • Intra-operative hypertensive crisis
EXPECTED COURSE AND PROGNOSIS
  • The survival rate after removal of a benign pheochromocytoma is nearly that of age- and sex-matched controls
  • For malignant pheochromocytoma, the 5-year survival rate is less than 50%
ASSOCIATED CONDITIONS
  • Multiple endocrine neoplasia type IIA (medullary thyroid carcinoma and primary hyperparathyroidism)
  • Multiple endocrine neoplasia type IIB (medullary thyroid carcinoma and mucosal neuromas)
  • Neurofibromatosis
  • Von Hippel-Lindau syndrome (retinal angiomatosis and cerebellar hemangioblastoma)
  • Ataxia-telangiectasia
  • Tuberous sclerosis
  • Sturge-Weber syndrome
  • Cholelithiasis
  • Renal artery stenosis
AGE-RELATED FACTORS

Pediatric:N/A
Geriatric: N/A
Others: N/A

PREGNANCY
  • First and second trimester - surgical resection
  • Third trimester - cesarean section and removal of the pheochromocytoma in the same operation
OTHER NOTES

All patients with paroxysmal spells and hypertension or with difficult to control hypertension should be screened

ABBREVIATIONS

HPLC = high performance liquid chromatography

Clinical Investigations
  • Cardiology
  • Dermatology
  • General Destistry
  • Cardiology
  • Dermatology

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.

Facebook X-twitter Instagram Google-plus-g
QUICK LINKS
contact

Copyright © 2025 Selkey. All Rights Reserved.