What is adrenocorticaloma, this very rare cancer from which actress Emilie Dequenne suffers?

In October 2023, actress Emilie Dequenne announced that she was suffering from a rare form of cancer which affects the adrenal glands (above the kidneys): adrenocorticaloma. Despite an initial remission which had raised hopes, she announced a recurrence “more aggressive” on August 27 which forced him to put his career on hold. Since then, the news on the state of health of the Belgian actress has not been good. His cancer is resistant to treatment. “Part of my cancer responds to it and part of it doesn't respond to it or even progresses and the part that progresses is bigger than the part that shrinks. I'm off again for the chemo that I went through with an extra dose of something new”she confided to our colleagues from Sept à Huit during a program broadcast on TF1 on Sunday 1^is December.

If we know little about this cancer it is because it is one of the rarest. Each year in France, it is estimated that it affects only 1 to 2 people in a million, which represents 60 to 120 new cases per year. Although it can occur in children, patients are mainly aged 40 to 50 years, with a higher incidence in women. Due to its rarity, its causes also remain very little known. In less than 5% of cases, cancer has a genetic origin, being associated with rare hereditary diseases such as Li-Faumeni syndrome, Wiedemann-Beckwith syndrome or Gardner syndrome. “For the remaining 95%, the cause often remains unidentified. », Estimates Éric Baudin, head of the endocrine tumors committee at the Gustave Roussy Institute.

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An overproduction of the stress hormone

The disease develops in the adrenal glands, located above the kidneys, whose role is essential in the production of hormones involved in the regulation of blood sugar, blood pressure and in the development of sexual characteristics. “Adrenocortical carcinoma is a subtype of adrenal cancer that more specifically affects the external part of the adrenal gland – the adrenal cortex – and is responsible for excessive secretion of cortisol, known as the stress hormone”explains Dr. Baudin. Symptoms include weight gain, hypertension, muscle wasting, bruising and frequent edema due to weakened skin. In some cases it can also be associated with increased production of a sex hormone produced by the adrenal gland, androgen: “we will then have additional symptoms such as excessive hair growth and masculinization in women”indicates Éric Baudin.

In all cases, this cancer being rare, it requires specialized care in a certified center, within the national ENDOCAN-COMETE network, specifically dedicated to the management of adrenal cancers. Treatment typically includes a combination of surgery, chemotherapy and radiotherapy. “If the cancer is detected early and remains limited to the adrenal gland, the tumor can be operated on, with a possibility of cure in 15 to 20% of cases”underlines Éric Baudin. The prognosis, however, is poorer in the event of a recurrence or if the cancer has spread to other organs in the form of more aggressive metastases and potentially become resistant to chemotherapy.

Recently, however, research has made progress and could pave the way for more personalized management of adrenocortical carcinoma. Several studies have notably made it possible to highlight two frequent pathways of alteration, in approximately two thirds of patients: “These are mutations in the TP53 gene and an alteration of the Wnt/β-catenin pathway, which promotes cell proliferation, but their role remains poorly understood and we unfortunately have no treatment against these specific alterations”explains Éric Baudin. Regardless, the identification of these pathways is a first step toward the development of new drugs and fuels the hope that one day this cancer could benefit from targeted therapies, like many other cancers.