Pheochromocytoma is a tumor that develops in the medulla of the adrenal gland (the inner part of the adrenal gland). Adrenaline (epinephrine) and noradrenaline are produced by the adrenal medulla (norepinephrine).
A tumor in this location can cause an excess of these hormones. Thus, it causing incredibly high blood pressure. It occurs most frequent in individuals between 30 and 50, although it can affect anyone of any age. Children account for around 10% of all instances.
About 10% to 15% of these tumors are malignant, and they have the potential to spread to other regions of your body. However, most pheochromocytoma tumors are benign, which means they aren't malignant.
It is essential to identify this issue as soon as possible. It may cure high blood pressure by locating and treating it. If not addressed, it can cause a heart attack, a stroke, and impaired artery function.
Causes:
People who inherit Pheochromocytoma from their parents may acquire genetic disorders related to it. These are some of the conditions:
- Von Hippel-Lindau disease is a non-cancerous disorder in which cysts and tumors form in the central nervous system, kidneys, adrenal glands, and other body parts.
- Type 1 neurofibromatosis is characterized by the growth of tumors on the skin and optic nerves.
- Pheochromocytoma is associated with multiple endocrine neoplasia type 2 (MEN2), a thyroid cancer.
Signs Or Symptoms:
Pheochromocytoma can cause severe symptoms in some people but not in others because it depends upon disease stage and varies from person to person. Symptoms might appear numerous times per day to a few times each month.
Some of the signs and symptoms include:
- Dizziness
- Racing heartbeat
- Anxiety
- Shortness of breath
- Constipation
- High blood pressure
- Weakness
- Fatigue
- Severe headache
- Pale skin
- Vomiting
- Stomach pain
- Tremors
- Excessive sweating
Possible Treatment:
- Medications: Your doctor may prescribe medications to help you manage your symptoms, such as alpha and beta-blockers blood pressure medications.
- Surgery: Surgery is utilized to remove the tumor as much as possible. It may lead to removing the entire adrenal gland in rare circumstances.
- Radiation therapy and chemotherapy: When pheochromocytoma has spread to other body regions, radiation and chemotherapy treatments are performed.
- Other treatments include targeted cancer therapies, MIBG therapy, Peptide receptor radionuclide therapy (PRRT).
Risk Factor:
Exact cause of this is not known.
Pheochromocytoma and paraganglioma are both caused by some of the genetic abnormalities include:
- Von Hippel-Lindau syndrome (VHL).
- Neurofibromatosis type 1 (NF1)
- Carney triad (paraganglioma, GIST, and pulmonary chondroma)
- Hereditary paraganglioma syndrome
- Multiple endocrine neoplasia type 2 (MEN2)
- Succinate dehydrogenase (SDHx) syndromes.
- Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST])
Diagnosis and Test:
- Lab Tests: If they have pheochromocytoma symptoms, the doctor will request urine and blood tests to examine your hormone levels. 24-hour urine testing to detect hormone levels and biochemical analysis to test plasma metanephrines.
- Imaging tests: The doctor will use imaging tests such as CT, MRI, M-iodobenzylguanidine (MIBG) imaging, and Positron emission tomography (PET) to determine the location and size of the tumor. They'll look for symptoms that cancer has spread to other places of the body, as well.
- Epidemiology: It is a rare disease. The majority of malignancies appear sporadically throughout the fourth and fifth decades. Pheochromocytoma affects less than one in every 100 persons with high blood pressure, and more than 95 percent of those instances aren't malignancy. Hereditary syndromes are closely connected to around 1/3 of the cases.
According to estimations, 95% of patients diagnosed with a malignant type of pheochromocytoma that hasn't migrated to other regions of their body live for at least another five years. About 50 percent to 60 percent of malignancies that have spread or returned after therapy live for at least five years following diagnosis.
Expected Prognosis:
Prognosis is a prediction of how a condition will affect you in the long term. Every person is unique, and the prognosis is influenced by a variety of circumstances, including:
- How much of the tumor was removed during surgery
- Where the tumor present is in your body
- If the disease has spread to other regions of your body
Doctors calculate pheochromocytoma survival rates by looking at how previous pheochromocytoma patients fared. The five-year survival percentage for patients with a small pheochromocytoma that has not migrated to other regions of the body is around 95 percent.
Whatever therapy they choose, they need to see the doctor regularly to ensure that pheochromocytoma doesn't resurface. They can also assist you in managing any treatment-related long-term adverse effects.
Pathophysiology:
- Excessive catecholamine production and subsequent activation of adrenergic receptors are found to cause pheochromocytoma. It emerges from the sympathetic ganglia and adrenal medulla's chromaffin cells.
- According to researchers, Pheochromocytoma is mainly caused by mutations in roughly 20 distinct genes. It can be caused by mutations in the genes RET, VHL, NF1, SDHA, SDHB, SDHC, SDHD, SDHAF2, MDH2, IDH1, PHD1/PHD2, HIF2A/EPAS1/2, TMEM127, MAX, HRAS, MAML3, and CSDE1. It can be grouped into three clusters of mutations based upon the function and regulation of genes.
Possible Complications:
The following list comprises of the possible complications of pheochromocytoma:
- Stroke
- Heart attack ( Angina pectoris )
- The disease of the Heart muscle (Cardiomyopathy)
- Accumulation of fluid in lungs (Pulmonary edema)
- Kidney failure
- Optic Nerve damage
- Brain Hemorrhage
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