Adrenocortical carcinoma

Adrenocortical carcinoma

Summarize

We have two adrenal glands in our body. They are small, triangular, and located on the upper sides of the left and right kidneys. Each adrenal gland can be divided into two parts: the outer layer is the adrenal cortex and the inner core is the adrenal medulla.

        Adrenal cortical carcinoma in children is common in patients under 6 years of age or adolescents, and more common in females.

        The adrenal glands synthesize important hormones for the body to use:

Maintain the balance of water and salt in the body

Maintain normal blood pressure

Regulates the body's use of protein, fat and carbohydrates

Differentiate gender identity

Epidemiological

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Etiology & Risk Factors

The risk of adrenal cortical carcinoma is increased in the presence of certain specific genetic mutations or the following syndromes:

Li-Fraumeni syndrome (also known as Li-Frau syndrome of multiple endocrine neoplasia type I)

Beckwith-Wiedemann syndrome

 hemihypertrophy

        Adrenal cortical tumors can be categorized into functional tumors, which involve abnormal hormone secretion, and non-functional tumors, which do not produce hormones. In children, adrenal cortical tumors are predominantly functional. Depending on the type of hormone involved, the symptoms caused by excessive hormone secretion can vary. For example, an excess of androgens can cause male-like characteristics in both male and female children, such as excessive body hair, a deeper voice, rapid growth, and acne; an excess of estrogens can lead to breast development in male children.

Classification & Staging

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Clinical Manifestations

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Clinical Department

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Examination & Diagnosis

The methods for the diagnosis and staging of adrenal cortical carcinoma depend on the patient's symptoms, including:

Physical examination and medical history

Blood biochemical tests

Chest, abdominal and skeletal X-ray examination

CT scan

MRI check up

PET scan

Biopsy (surgical removal of a mass to check whether the specimen is cancerous)

        For a description of these inspection methods, see the basic information section above.

        Other methods used to diagnose adrenal cortical cancer are as follows:

24-hour urine test: A 24-hour urine is collected to measure the levels of cortisol and 17-ketosteroids. If these levels are above normal, it may indicate an adrenal cortex disorder. ​Ÿ Low-dose dexamethasone suppression test: The patient is given a low dose of dexamethasone once or multiple times, followed by continuous three-day monitoring of blood or urine samples. This test is used to check if the adrenal glands are overproducing cortisol. If the adrenal glands are overproducing cortisol, the cortisol levels in the plasma or urine will not decrease after taking the medication.

High-dose dexamethasone suppression test: Administer a high dose of dexamethasone to the patient once or multiple times, and monitor blood or urine samples for three consecutive days. This test helps determine whether the condition is due to an adrenal gland that secretes excessive cortisol or a pituitary gland that commands the adrenal glands to secrete too much cortisol. If it is adrenal cortical cancer, the cortisol levels in the plasma or urine will not decrease after taking the medication; if it is a pituitary disorder, the cortisol levels will decrease after taking the medication.

Blood hormone test: Blood samples are taken to measure the levels of certain hormones. If they are higher or lower than normal, it indicates that there is a lesion in an organ or tissue. If plasma testosterone or estrogen is higher than normal, it may indicate that the patient has adrenal cortical cancer.

Adrenal angiography: used to examine the arteries and blood flow adjacent to the adrenal gland. The contrast agent is injected into the adrenal artery, and when the contrast agent passes through the vessel, X-ray imaging is performed to see if there is an arterial obstruction.

Adrenal venography: This procedure is used to examine the veins and blood flow near the adrenal glands. Contrast agent is injected into the adrenal vein, and X-ray imaging is performed as the agent passes through the vein to check for any obstruction. Additionally, a catheter (a very fine tube) can be inserted into the vein to draw blood samples, which are then tested for abnormal hormone levels.

Clinical Management

For the treatment information below, see the treatment plan overview section above.

        Adrenal cortical cancer in children can be surgically removed and assisted chemotherapy or no chemotherapy can be selected according to the actual situation.

        Patients with recurrent pediatric adrenal cortical carcinoma may consider participating in clinical trials to test for changes in genes in tumor samples and to target the patient based on the type of gene change

Prognosis

If the tumor is small and can be completely removed by surgery, the prognosis is better. The prognosis is related to the following factors:

 gross tumor volume

Whether there is a genetic mutation

Whether the tumor has spread to other parts of the body, including lymph nodes

Age of the child

Whether the tumor capsule is ruptured when the tumor is removed by surgery

Whether the child has developed male characteristics

        The tumor can spread to the kidneys, lungs, bones and brain.

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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