Adrenocortical Tumors

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What is an adrenocortical tumor (ACT)?

ACTs are a group of tumours originating from the adrenal cortex- that is the outerlayer of the adrenal gland. They rarely occur in children and adolescents.

Who gets an ACT and why?

There are two peaks of incidence in young people, the first in children < 3 years of age, the second during adolescence. It is more common in girls. The cause is unknown.

Is there a risk of brothers / sisters developing the same cancer or other types of cancer?

While ACTs may occur sporadically, there is a significant association with other tumor syndromes (i.e. Li Fraumeni Syndrome, MEN1, and Beckwith-Wiedeman syndrome.). For this reason genetic review and investigations are recommended for all patients and their relatives.

Are all ACTs the same?

ACTs maybe benign (adenomas) or malignant (adrenocortical carcinomas-ACC) but they often present in a similar way clinically. However ACCs are larger than adenomas, grow faster, involve the surrounding organs and may spread, mostly to the lung, liver and bone. The pathological distinction between the two entities is difficult as they often look the same under the microscope but the size of the tumour, or evidence of spread helps to separate out adenomas from ACC.

What are the typical symptoms and signs of ACTs?

Most ACTs (80-90%) secrete in excess the same hormones normally produced by the adrenal cortex (steroids, androgens and aldosterone): common symptoms are hypertension- high blood pressure, virilization (hairiness or hirsutism, premature pubic hair), obesity, acne, moon face and skin striae. This symptom is called “Cushing syndrome” if they include steroid over secretion. If the tumor doesn’t secrete hormones, an abdominal mass may be the only symptom.

Which investigations are necessary to study a child with ACTs?

Two groups of investigations are indicated:

1. Hormone levels of the adrenal cortex-if elevated they support the diagnosis. Theymay be useful after the treatment as markers of tumor surveillance.

2. Imaging investigations (abdominal ultrasound, and CT/MRI scans): they evaluate the size of the mass and the involvement of adjacent organs, to help surgeons to discuss the resectability of the mass. Lung CT Scan and bone scan are recommended if an ACC is diagnosed.

Are there different stages of the disease?

The stage is taken into consideration only for ACCs. Stage is based on the tumor size, the involvement of regional lymph nodes (N), the presence of distant metastases (spread or M) and the results of surgery at diagnosis, and how much of the tumour has been removed. Also postoperative decrease of the hormone values are taken into account.

What is the treatment for ACTs?

Complete resection/removal of the tumour is the mainstay of treatment, and is curative alone for adenoma and localized small ACCs In patients with large ACCs (also after a complete resection), in those with tumor residuals after primary surgery, and in those with tumours that have spread, chemotherapy and /or mitotane are recommended. Experts in multidisciplinary meetings will usually discuss if there is a role for this in your child’s case.

What are the results of treatment?

Complete removal of the tumour can be curative and outcome is excellent for adenomas.The results of the treatment for ACCs depends on the extent of disease and if the tumour has been completely removed: the chances of cure vary, being low in those with spread but much higher in those with localized and completely removed disease.

What research is happening for ACTs?

ACTs are rare in childhood: most research studies come from countries where the incidence is higher (such as Brazil) or from adult series. Studies are evaluating a possible relation with environmental as well as genetic factors. Molecular studies have shown that there are abnormalities of the Tp53 gene in about 50% of ACTs, suggesting that ACTs are part of a familial cancer syndrome.

What EXPert is doing for children with ACTs?

The EXPert group is working for children with ACTs in many ways:

  • Collecting data of children with ACT among all European countries
  • Creating guidelines for diagnosis and treatment with the goal to optimize the chance of cure for all children
  • Giving advice in difficult cases to the responsible clinician at a European level and worldwide
  • Collaborating with other Groups to improve knowledge on ACTs