Pheochromocytoma And Paraganglioma

Pheochromocytoma And Paraganglioma

Summarize

Pheochromocytoma and paraganglioma are rare tumors of the same type of nerve tissue.

50. Pheochromocytomas originate in the adrenal glands. Humans have two adrenal glands, located at the top of the upper abdomen. Each adrenal gland consists of two parts: the outer layer is the adrenal cortex, and the central part is the adrenal medulla. Pheochromocytomas are tumors that develop in the adrenal medulla. The adrenal glands produce a crucial hormone called catecholamines, which includes adrenaline and noradrenaline. These hormones help regulate heart rate, blood pressure, blood sugar levels, and the body's response to emergencies. Some pheochromocytomas release excessive amounts of adrenaline and noradrenaline into the bloodstream, leading to specific symptoms.
50. Paragangliomas are located outside the adrenal glands, near the carotid arteries, and along the nerve pathways of the head and neck. They may also be found in other parts of the body. Some paragangliomas produce excessive amounts of adrenaline and noradrenaline. When these excess hormones are released into the bloodstream, they can cause symptoms.

Epidemiological

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Etiology & Risk Factors

Any factor that increases the likelihood of disease is called a risk factor. Having a risk factor doesn't mean you will get cancer; not having a risk factor doesn't mean you won't get cancer. If you think your child may be at risk, consult a pediatrician.

        Children with any of the following genetic syndromes or gene changes are at increased risk for pheochromocytoma or paraganglioma:

50. Multiple endocrine neoplasia syndrome type 1.
50. Multiple endocrine neoplasia syndrome type 2 (multiple endocrine neoplasia syndrome type 2A and multiple endocrine neoplasia syndrome type 2B).
50. von Hippel-Lindau disease (VHL)
50. Type 1 neurofibromatosis (NF1)
50. Carney-Stratakis syndrome (simultaneous paraganglioma and gastrointestinal stromal tumor)
50. Carney triad (commonly known as Carney triad, with paraganglioma, gastrointestinal stromal tumor and pulmonary chondroma)

        Changes in certain genes, including VHL, NF1, RET, SDHD, SDHB, SDHA, MAX, and TMEM127.

        More than half of children and adolescents diagnosed with pheochromocytoma or paraganglioma have a hereditary syndrome or genetic change that increases the risk of cancer. Genetic counseling (discussing genetic diseases with trained professionals) and testing are an important part of the treatment plan for these patients.

Classification & Staging

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Clinical Manifestations

Some tumors do not produce additional adrenaline or noradrenaline and do not cause symptoms. These tumors, such as pheochromocytomas and paragangliomas, may be detected when a lump is found in the neck or during examinations or surgeries for other reasons. Symptoms of these tumors occur when excessive adrenaline or noradrenaline is released into the bloodstream. In addition to pheochromocytomas and paragangliomas, other conditions can also cause these symptoms. If your child exhibits any of the following symptoms, please consult a pediatrician:

50.  hypertension
50.  headache
50. Sweating a lot for no apparent reason
50. A strong, rapid or irregular heartbeat
50.  shake
50. Very pale
50.  dizzy
50. Irritable or tense,

        These signs and symptoms may come and go, but hypertension is more likely to persist for longer in younger patients. These symptoms can also occur in other situations, such as physical activity, injury, anesthesia, surgical removal of a tumor, eating foods like chocolate and cheese, or during urination (if the tumor is in the bladder).

Clinical Department

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Examination & Diagnosis

The tests needed for the diagnosis and staging of pheochromocytoma and paraganglioma depend on the patient's signs and symptoms, as well as their family history. These tests may include:

50. Physical exam and medical history
50. PET scan
50. CT scan (CAT scan)
50. MRI (magnetic resonance imaging)

For a description of these tests and procedures, see the basic information section above.

        Other tests used to diagnose pheochromocytoma and paraganglioma include:

50. Plasma free metanephrine test: This blood test measures the levels of metanephrines in the blood. Metanephrines are substances produced when the body breaks down adrenaline or noradrenaline. Pheochromocytomas and paragangliomas can produce large amounts of adrenaline and noradrenaline, leading to high levels of metanephrines in both blood and urine.
50. Blood catecholamine testing involves examining blood samples to measure the levels of certain catecholamines (epinephrine or norepinephrine) released into the bloodstream, as well as the breakdown products of these catecholamines. Abnormal levels (either higher or lower than normal) may indicate diseases in organs or tissues. Levels above the normal range can be a sign of pheochromocytoma or paraganglioma.
50. 24-hour urine test: A 24-hour urine is collected to measure the levels of catecholamines (epinephrine or norepinephrine) or metanephrines in the urine. It also measures the levels of these catecholamine breakdown products, which can indicate diseases in organs or tissues. Levels above normal may suggest conditions such as pheochromocytoma or paraganglioma.
50. MIBG scan: This test is used to detect neuroendocrine tumors, such as pheochromocytoma and paraganglioma. During the procedure, a small amount of radioactive MIBG is injected into the patient's vein, allowing it to circulate through the bloodstream. The tumor cells will absorb the radioactive MIBG, which can then be detected by a scanner. The scan typically takes 1-3 days. To prevent the thyroid from absorbing too much MIBG, patients may be given an iodine solution before or during the test.
50. Somatostatin receptor imaging: This is a radiopharmaceutical scan used to detect tumors. A very small amount of the radioactive hormone octreotide, which binds to tumors, is injected into a vein and circulates through the bloodstream. The radioactive octreotide attaches to tumors and can be detected using a special camera that detects radioactivity, revealing the tumor's location in the body. This procedure is also known as an octreotide scan or SRS.

Clinical Management

For information on the treatments listed below, see the treatment overview section above.

        Treatment of children with pheochromocytoma and paraganglioma may include the following:

50. The tumor was completely removed by surgery. ​
50. Combination chemotherapy, dose 131I-MIBG, and targeted therapy for tumors that have spread

        Before surgery, the medical staff will give the patient medication, including α receptor blockers to control blood pressure and β receptor blockers to control heart rate. If both adrenal glands are removed, lifelong hormone replacement therapy is required after surgery to replace the hormones produced by the adrenal glands.

        Children with recurrent pheochromocytoma and paraganglioma may be considered for clinical trials to test whether the genes in the patient's tumor samples have changed and to target the patients based on the type of gene change.

Prognosis

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Follow-up & Review

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Daily Care

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Cutting-edge therapeutic and clinical Trials

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References

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Audit specialists

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