PHEOCHROMOCYTOMA - Causes and Risk Factors, Clinical Manifestations, Diagnostic Evaluations and Management
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PHEOCHROMOCYTOMA  - Causes and Risk Factors, Clinical Manifestations, Diagnostic Evaluations and Management 

PHEOCHROMOCYTOMA  - Causes and Risk Factors, Clinical Manifestations, Diagnostic Evaluations and Management

Pheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that control heart rate, metabolism, and blood pressure. A pheochromocytoma can develop at any age, but most commonly occurs in middle age. Most of the time, a pheochromocytoma is noncancerous (benign).

 

CAUSES AND RISK FACTORS

Pheochromocytoma may occur as a single tumor or as more than one growth. It usually develops in the center (medulla) of one or both adrenal glands. Rarely, this kind of tumor occurs outside the adrenal gland, usually somewhere else in the abdomen.

Factors associated with pheochromocytoma include: A family history of pheochromocytoma, and Genetic diseases including: Von Hippel-Lindau disease, Multiple endocrine neoplasia, type 2, Neurofibromatosis type 1, Paraganglioma syndromes and Li-Fraumeni syndrome.

 

RISK FACTORS

  • Dopamine antagonists
  • Cold medications
  • Radiographic contrast media
  • Drug that inhibit catecholamine reuptake, such as tricyclic antidepressants and cocaine
  • People with this rare multisystem disorder are at high risk of pheochromocytoma, kidney cancer and brain and eye tumors


 

CLINICAL MANIFESTATIONS

Someone with a pheochromocytoma usually  has three classic symptoms  - headache, sweating and heart palpitations (a fast heart beat) in association with markedly elevated blood pressure (hypertension). The heart beat might be rapid, the patient might feel cold and clammy, and a drop of blood pressure with postural changes (postural hypotension) might make the patient dizzy or faint.

Some abdominal symptoms like increased appetite, abdominal pain, nausea, vomiting or constipation might also be present. Other symptoms include chest pain, breathing problems (shortness of breath), fatigue, weight loss, sleeping difficulty, vision problems (blurred vision), seizures, tingling, burning, or numbness in the legs, involuntary trembling (tremor) and feet anxiety with a strong feeling of impending doom and loss of skin feeling in different regions could also be present.

 

DIAGNOSTIC EVALUATIONS

The history of symptoms and the physical exam will make a healthcare provider suspect a pheochromocytoma. The tumor is often found in people with very high blood pressure.

  • 24 hour urine testing – to measure amounts of catecholamine, including epinephrine and norepinephrine and their byproduct (metanephrines).
  • Clonidine suppression test – a dose of the drug clonidine is given, and blood levels of norepinephrine are tested.
  • CT scan – a type of x-ray that uses a computer to make pictures of structures inside the body; used to diagnose the presence of the tumor
  • Ultrasound – a noninvasive test using sound waves, which may be used to help detect adrenal tumors
  • MRI scan – a test that uses magnetic waves to produce cross-sectional or three dimensional images of the body tissues
  • Meta-iodobenzylguanidine (MIBG) scanning – an imaging test for diagnosing adrenal tumors, in which tiny amounts of radioactive materials are injected into the body
  • Flurodopamine PET scan – used when the blood tests indicate presence of pheochromocytoma, but other imaging tests do not reveal the presence of the tumor


STAGES OF CANCERS

Stage I: the cancer is smaller than 5 cm and has not spread outside of the adrenal gland

Stage II: the cancer is larger than 5 cm and has not spread outside of the adrenal gland

Stage III: the cancer has spread into the fat surrounding the adrenal gland or has spread to lymph nodes or other organs near the adrenal glands

Stage IV: the cancer has spread to other parts of the body

 

MANAGEMENT

Surgery is the treatment of choice to remove the pheochromocytoma (adrenalectomy). Medications are used to help control the high blood pressure before and during surgery. Prior to surgical removal, the high blood pressure will need to be brought under control, using first the medication phenoxybenzamine, followed by beta-blocking antihypertensive agents.

If the tumor is smaller than 5 cm and, based on size or the way it looks on imaging scans, shows no evidence that it is cancerous, and then laparoscopic surgery may be possible. Laparoscopic surgery is a minimally invasive technique in which a surgeon operates with small telescopes and long instruments.

Some people cannot tolerate surgery, or surgery cannot remove the entire tumor. In this case, blood pressure medications and chemotherapy may be needed. Chemotherapy may be used if the tumor is cancerous and has spread outside of the adrenal glands. It involves the use of: Docarbazine, Vincristine and Cyclophosphamide. Radiation may also be used. It is used to destroy blood vessels that may be feeding the tumor. This process is called ablation.