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Paraganglioma

more about Paraganglioma


Pheochromocytoma, PC, Tumors of Adrenal Glands, and Ten Percent Tumors


  • There are two adrenal glands located above the kidneys.  Pheochromocytomas are catecholamine-producing tumors of the adrenal glands.  The brain uses Catecholamines chemicals known as epinephrine (adrenaline) and norepinephrine to process and transmit information to other organs.  Adrenal glands are the site where Catecholamines are normally made.  PCs are tumors that can secrete excess Catecholamines , which can produce a variety of symptoms.
  • They are called 10% tumors because:
      1. 10% are grown outside the adrenals in other tissues derived from neural crest cells of the developing embryo (baby).
      2. 10% are multiple in one or both adrenal glands.
      3. 10% are in children.
      4. 10% are cancerous tumors (malignant) and can spread to other sites in the body.
      5. 10% are familial (found in other family members), and may be inherited (passed down through abnormal genes).
      6. 10% return after being removed.
      7. 10% of patients with Strokes have Pheochromocytomas.
      8. 10% have no symptoms.

  • Symptoms appear suddenly -- paroxysmal
  • Pain -- head, chest, and abdomen
  • Pain may be sudden
  • Pressure in the head or Blurred Vision
  • Palpitations -- heart racing or pounding
  • Increased sweating
  • Pallor -- pale, ghost color of skin
  • Anxiety
  • Nausea
  • Vomiting
  • Fever
  • Flushing is rare -- redness of the face
  • Weightless

  • Heredity -- a gene may be passed down from only one parent (autosomal dominant) that can give rise to PC.
  • May be associated with other diseases of the skin, nerves, and hormone producing cells
  • Neurofibromatosis
  • Tuberous sclerosis
  • Sturge -- Weber syndrome
  • Von Hippel -- Lindau syndrome
  • Multiple endocrine neoplasia -- types II (Thyroid Cancer and parathyroid gland involvement) and III.

  • Your doctor may consult with an endocrinologist.
  • History:
    1. Symptoms
    2. Medications
    3. Illnesses
    4. Allergies
    5. Habits
    6. Job
    7. Family
  • Exam:
    1. Fever, High Blood Pressure, and a fast heart rate may be present.
    2. The examination of the back of the eyes (retina) may show long-term damage from High Blood Pressure.
  • Blood levels of Calcium and glucose may be elevated.
  • There may be an increased number of red blood cells.
  • Diagnosis of PC requires the extracting of excess Catecholamines.
  • Urine samples are collected for 24 hours (outpatient or inpatient) and increased levels of metanephrine (a breakdown product of Catecholamines) is diagnostic of PC.
  • Blood levels of Catecholamines can also be measured.
  • Tests such as Glucagon stimulation (a hormone given into the veins) and Clonidine (blood pressure medicine taken orally) suppression are helpful in diagnosing PC.
  • Special scans can take pictures of the tumor.  These include MRIs, CAT scans and In-111 pentetreotide and I-123 I-metaidobenzylguanidine scans.

  • Male > females
  • Patients between 30-60 years of age

  • Surgery can remove the tumors and cure PC.
  • Before surgery, the patient's symptoms are controlled with the use of specific medications, such as alpha and beta adrenergic blockers (Dibenzyline and Inderal).
  • Very high and dangerous blood pressure (hypertensive crisis) is treated with Phentolamine or Nitroprusside intravenously (in the veins).

  • Contact your doctor for appropriate tests and referrals to specialists.




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