What Is Rare Adrenal Gland Cancer Actress Emilie Dequenne Was Suffering From?

What Is Rare Adrenal Gland Cancer Actress Emilie Dequenne Was Suffering From? (Image Credits: Instagram)

Belgian actress Emilie Dequenne, known for her award-winning role in Rosetta, tragically passed away on Sunday at the age of 43. Her family and agent confirmed to AFP that she had been battling adrenocortical carcinoma (ACC), a rare and aggressive cancer affecting the adrenal glands, since her diagnosis in October 2023.

Understanding Adrenocortical Carcinoma (ACC)

Adrenocortical carcinoma is a rare malignancy originating in the cortex of the adrenal glands, which sit atop each kidney. These glands produce crucial hormones such as cortisol, aldosterone, and androgens, which regulate metabolism, blood pressure, and immune function. ACC is aggressive and often diagnosed late, leading to poor survival rates.
According to the National Cancer Institute (NCI), ACC occurs in approximately 1 to 2 people per million each year, making it an exceptionally rare condition.

Symptoms and Diagnosis

The symptoms of ACC vary, depending on whether the tumour is functional (hormone-producing) or non-functional. Common symptoms include:
    Hormonal Imbalances: Overproduction of cortisol can lead to Cushing’s syndrome, causing rapid weight gain, high blood pressure, and muscle weakness. Excess androgens may result in abnormal hair growth and menstrual irregularities.
  • Abdominal Pain: As the tumour grows, it can cause pain or discomfort in the abdomen or back.
  • Unexplained Weight Loss: Sudden, unexplained weight loss is a key warning sign of aggressive cancers.
  • Due to its rarity, ACC is often diagnosed at an advanced stage. According to a study published in the Journal of Clinical Endocrinology and Metabolism, early detection remains a major challenge due to the tumour’s asymptomatic nature in the initial stages.

    Treatment Options

    Treatment for ACC depends on the stage of diagnosis and the patient’s overall health. The main treatment approaches include:
      Surgical Resection: If diagnosed early, surgery to remove the tumour offers the best chance of survival. However, because ACC is often aggressive, achieving complete removal can be difficult.
  • Mitotane Therapy: Mitotane is an adrenolytic drug specifically used for ACC to suppress adrenal gland activity and target cancer cells. The American Society of Clinical Oncology (ASCO) recommends mitotane as the first-line treatment for patients with unresectable or metastatic ACC.
  • Chemotherapy and Radiation: Advanced cases may require chemotherapy with drugs like etoposide, doxorubicin, and cisplatin, often combined with mitotane. Radiation is sometimes used for localized control.
  • Dr Gary Hammer, an expert in adrenal cancers at the University of Michigan, explains the importance of specialized treatment: “ACC is a rare and complex disease that requires a multidisciplinary approach, ideally in specialized cancer centres.”

    Prognosis and Ongoing Research

    The prognosis for ACC remains poor, with five-year survival rates ranging from 20 per cent to 35 per cent, according to the National Organization for Rare Disorders (NORD). The outcome depends largely on the stage at diagnosis and whether the tumour can be surgically removed.
    Researchers are exploring genetic mutations in ACC to develop targeted therapies. Recent studies published in The Lancet Oncology suggest that immunotherapy and molecular-targeted treatments may offer new hope for ACC patients in the future.
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