“Here I go again for chemotherapy that I experienced a year ago,” said Émilie Dequenne on Sunday on the set of TF1. The 43-year-old Belgian revealed in October 2023 that she had a rare cancer, diagnosed two months earlier, and which had kept her away from film sets ever since. When the announcement was made, the 43-year-old actress had “the impression that we were giving (her) six months to live”. Today, she explains: “Deep down, I know very well that I will not live as long as I expected. » But what is this rare and aggressive cancer, often diagnosed at a late stage?
What is adrenocorticaloma?
This cancer affects the adrenal glands, which are located above the kidneys. Each gland is made up of two parts, an internal and an external. The latter is called the cortex or adrenal cortex and it is this which is affected. Its role is in particular to secrete steroids, such as cortisone.
Adrenocortical carcinoma is a rare, even very rare, cancer. “There are one to two new cases per million inhabitants per year,” specifies Eric Baudin, head of the endocrine tumors committee at the Gustave-Roussy Institute. In France, there are therefore between 65 and 135 people diagnosed each year. Patients who are mainly between 25 and 60 years old. Although a few cases exist among children, they are ten times less affected than adults. “There are slightly more women affected than men, around 1.3 women for every man,” adds Jérôme Bertherat, head of the endocrinology department at Cochin hospital.
The causes of adrenocortical carcinoma remain obscure, apart from a few hereditary cases. “Predisposition syndromes are extremely rare but in these patients, the adrenal gland will be one of the organs screened,” assures the doctor.
How is this cancer diagnosed?
This type of cancer can be discovered in two ways. The first will realize the presence of an adrenal mass incidentally, by doing a CT scan or MRI of the abdomen for another indication. “In only 2% of cases it will be a corticoadrenaloma and not benign lesions,” continues Jérôme Bertherat, head of the endocrinology department at Cochin hospital in Paris. A painful or bloating lump can also prompt a consultation.
But if the cancer is linked to an excess of cortisol or androgen, it will give rise to clinical manifestations. “Significant weight gain, a tendency to hypertension, diabetes, a drop in potassium, more fragile skin, which marks more easily, with edema, swelling are all symptoms,” lists Eric Baudin. In this case, a hormonal assessment and then a CT scan are carried out to confirm the diagnosis.
What is the treatment?
“Half of adrenocortical carcinomas appear in an operable form and the other half in an inoperable form, most of the time metastatic,” emphasizes Dr. Baudin. Even when it is localized, it is often ten, 15 or even 20 centimeters in diameter. From the outset the tumor will be advanced, with a high rate of recurrence. »
Once there is a localized tumor, treatment consists of surgically removing it. Due to the rarity of this type of cancer, treatment must be done in a specialized center (listed by the “Endocan Comete” rare cancers network). “To limit the risk of recurrence, we add a drug treatment, mitotane,” explains Professor Bertherat. But if it is insufficient, or in the event of a recurrence, the patient will have to resort to chemotherapy.
Our file on cancer
In order to assess the risk of relapse as early as possible, post-treatment monitoring remains very tight. “During the first five years, patients are checked every three months, with imaging and sometimes hormone checks, when they have a hormonal abnormality,” emphasizes Eric Baudin.
What is the prognosis?
“It’s an aggressive tumor which has a severe prognosis,” says Eric Baudin. But the latter varies depending on the initial stage. “When the tumor is very localized and treated early, we have an 80% survival rate at five years,” Professor Bertherat tries to reassure. But when you have a metastatic stage from the start, the prognosis is less than 35% survival at five years. » Finally, according to Eric Baudin, in 5 to 15% of patients, the disease will become chronic, with a slightly more indolent form and/or a response to chemotherapy.