Adrenocortical carcinoma in children — adrenal cancer explained for parents

Adrenocortical carcinoma — often abbreviated ACC — is a cancer that arises from the outer layer of the adrenal gland, called the cortex. The adrenal glands are small, triangular organs that sit on top of the kidneys and produce hormones that regulate blood pressure, the stress response, salt and water balance, and sex hormones. ACC is an uncommon cancer overall, and it is particularly rare in children. However, it does occur in childhood and is actually more common in young children (under five years of age) than in older children or teenagers. Many families have never heard of this diagnosis before; that is not unusual, and you are right to seek clear, reliable information as early as possible.

The adrenal cortex produces sex hormones as well as cortisol and aldosterone, and in children with ACC the most common early signs are caused by abnormal hormone production rather than the tumour bulk itself. Parents often notice: unusual growth of pubic or underarm hair in a very young child; an enlarged clitoris in girls or premature penile growth in boys; unexpected acne; rapid growth in height; or a deepening voice — any of these in a child under eight years who has not started puberty should prompt a medical review. A smaller number of children develop signs of cortisol excess (weight gain around the trunk, a rounded face, stretch marks, high blood pressure, or mood changes). Some children, particularly older ones, have a tumour that does not produce hormones and may present with an abdominal lump, flank pain, or weight loss. Any unexplained hormonal change or abdominal swelling in a child should be assessed by a doctor.

In a significant proportion of children with ACC, a change in a gene called TP53 is found. TP53 is a tumour-suppressor gene — it normally helps the body prevent abnormal cells from becoming cancer. When both copies of TP53 are not working normally, cells in the adrenal cortex may develop into a tumour. This pattern is associated with a genetic condition called Li-Fraumeni syndrome, which predisposes individuals to several different cancers. In certain parts of the world — notably southern Brazil — a specific inherited change in TP53 is found in a high proportion of children with ACC, and genetic research in those populations has contributed enormously to understanding this disease. When a child is diagnosed with ACC, genetic counselling and testing for TP53 changes are recommended for the child and, depending on results, for family members. Finding a genetic cause does not change the immediate treatment, but it is important for long-term cancer surveillance planning for the family. Please ask your child’s oncology team about a referral for genetic counselling.

When a doctor suspects an adrenal tumour, the diagnostic process typically involves two things in parallel: imaging to identify and characterise the tumour, and hormone blood and urine tests to find out whether the tumour is producing hormones and which ones. Imaging usually starts with an ultrasound of the abdomen, followed by a CT scan or MRI to precisely map the tumour’s size and position and to look for any spread to nearby lymph nodes or other organs. Blood tests measure levels of cortisol, androgens (sex hormones), aldosterone, and related hormones. Urine collected over 24 hours may be tested for cortisol and its breakdown products. A chest CT is usually done to check whether there are any spread spots in the lungs. Biopsy before surgery is generally avoided in ACC because the tumour capsule must not be broken; instead, the entire tumour is usually removed surgically and then examined by a pathologist. The pathologist’s report — particularly a scoring system called the Weiss score or a paediatric-specific modification — helps confirm that the tumour is truly malignant (a carcinoma) rather than a benign adrenocortical adenoma.

Surgery is the cornerstone of treatment for adrenocortical carcinoma in children. The goal is complete removal of the tumour in one piece, without rupturing the capsule — because capsule rupture is associated with a higher risk of local recurrence. The operation removes the adrenal gland along with the tumour (adrenalectomy), and sometimes nearby lymph nodes are removed as well. After surgery, whether additional treatment is needed depends on the stage of disease (how far the tumour had spread at diagnosis), whether the surgeon was able to achieve complete removal, and the pathology findings. For children with fully resected early-stage disease, careful follow-up surveillance may be sufficient without additional systemic therapy. For children with more advanced disease, or where the tumour was not completely removed, the oncology team will discuss other treatment options. Every decision at CION is made by a multidisciplinary tumour board — medical, surgical, and radiation oncologists together — before anything is started. We do not make rushed decisions.

Yes — long-term follow-up is an important part of care after treatment for childhood ACC. The oncology team will set up a structured surveillance schedule involving regular imaging (typically CT or MRI of the abdomen and chest) and hormone measurements to detect any sign of recurrence early. The frequency of these checks is usually highest in the first two years after treatment, then gradually reduces over time if the child remains well. In addition, because many children with ACC carry a TP53 genetic change (whether or not this was known before diagnosis), genetic counselling for the child and family is recommended — and the child may benefit from a broader cancer surveillance programme throughout their life. This can feel daunting, but it is also a form of protection: cancers detected early at follow-up imaging are generally more treatable than those discovered because a symptom reappears. The team at CION will walk this journey with you at every step.