Everything To Know About Pheochromocytoma

pheochromocytoma

Tumors of the chromaffin cells found in adrenal glands are called pheochromocytomas. The adrenal glands, located on top of the kidneys are responsible for the production of important stress, sex and steroid hormones. When tumors of such secretory cells occur, there is production of hormones and symptoms secondary to the high levels of these hormones. According to experts like Best Endocrinologist in Lahore, most cases of pheochromocytomas are unilateral. Read on to know more about pheochromocytomas, their symptoms, diagnosis and treatment options:

What are pheochromocytomas?

Pheochromocytomas are tumors of the chromaffin cells, as mentioned before. These cells are found in the medulla or the middle of the adrenal cortex, and responsible for production of stress hormones like adrenaline and noradrenaline. With pheochromocytomas, there is continuous overproduction of these hormones and the symptoms produced are due to their high levels. 

Pheochromocytomas are rarely cancerous, but may often be part of a syndrome linked with cancer of other organs—like the thyroid gland. Despite being benign, pheochromocytomas need treatment, as high levels of catecholamines are dangerous for the body—increasing the risk of outcomes like stroke and heart attack. 

What are the symptoms of pheochromocytomas?

The three main symptoms of pheochromocytoma are:

  • Headache
  • Excessive sweating 
  • Palpitations or increased heart rate

Apart from the primary symptoms, the other symptoms are:

  • Pallor 
  • Nervous shaking 
  • Upset stomach 
  • Weight loss 
  • Vision disorders 
  • Anxiety 
  • Seizures 
  • Pain in the abdomen or chest 
  • Feeling overheated
  • Spikes in blood pressure 
  • Vomiting 
  • Clammy skin 
  • Orthostatic hypotension 

What are the risk factors of pheochromocytomas?

The risk of pheochromocytoma is highest in:

  • ages between 20 and 50 years 
  • certain hereditary disorders like: 
    • multiple endocrine neoplasia (MEN) type 2
    • hereditary paraganglioma syndrome
    • neurofibromatosis type 1 
    • Von Hippel-Lindau (VHL) disease

What can trigger pheochromocytoma attacks?

The symptomatic spells or triggers of pheochromocytomas include:

  • Activities like physical hard work 
  • Change in body position like sitting to standing or lying to sitting 
  • Anxiety and stress 
  • States of stress like labor and delivery 
  • Surgical stress 
  • Anesthesia 
  • Foods with high tyramine content can also trigger symptoms of pheochromocytomas. Examples of such foods include: chocolate, smoked meat, cheeses and aged wine. 
  • Certain medications like antidepressants MAOIs, tranylcypromine, isocarboxazid, phenelzine, as well as, stimulants like cocaine or amphetamines can trigger symptoms. 

How are pheochromocytomas diagnosed?

The diagnosis of pheochromocytoma is based on high suspicion as per the history of the patient, along with investigations like:

  • 24-hour urine test: in this test, urine sample is collected for a period of 24-hours to check for the metabolites of catecholamines. They will be raised in pheochromocytomas. 
  • CT/MRI: this x-ray of CT and magnetic imaging is used to produce detailed imaging of the adrenal glands to check the location of the tumor, and its extent. Imaging tests also confirm the tumor, as pheochromocytomas have a characteristic appearance.   
  • MIBG imaging: in this test, a small dye is injected into the body, which is then taken up by the pheochromocytomas and paragangliomas.  
  • PET scan: in this test, radioactive dye is injected in the body, which is taken up by the tumor cells wherever they may be. Thus, this helps to detect metastatic tumors. 

What are the complications of pheochromocytomas?

With spikes of extremely high blood pressure, there is risk of:

  • Kidney damage
  • Stroke 
  • Heart attack 
  • Retinopathy and end organ damage 
  • Myocarditis 
  • Fluid buildup in the lungs
  • Cardiomyopathy 
  • Metastasis to liver, lungs, bones and lymph nodes

What are the treatment options?

The treatment options for pheochromocytoma are:

  • Medication: the initial control of blood pressure and palpitations is with medication. 
  • Surgery: the mainstay of treatment for pheochromocytomas is surgical excision. With surgical removal of the tumorous mass, the symptoms often go away. 
  • Radiation therapy: cancer cells are killed with radiation therapy; external beam radiotherapy is used in particular for pheochromocytomas. 
  • Chemotherapy: chemotherapeutic drugs are given intravenously to kill the cancer cells. The regimen most often used by Endocrinologist in Karachi for pheochromocytomas is: vincristine, dacarbazine and cyclophosphamide. 

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