Adrenal Cortical Carcinoma Facts

Adrenal cortical carcinoma (ACC) is a rare type of childhood cancer that originates in the adrenal cortex, which is the outer layer of the adrenal glands. 

Adrenal Glands: The adrenal glands are small, triangular-shaped glands located on top of each kidney. They produce hormones that regulate various bodily functions, including metabolism, blood pressure and response to stress. The adrenal cortex, specifically, produces hormones such as cortisol, aldosterone and androgens.

  • Adrenal cortical carcinoma is considered rare, especially in children. 
  • While it can occur at any age, it is more commonly diagnosed in adults. However, when it does occur in children, it tends to present in early childhood or adolescence.
  • Adrenal cortical carcinoma arises from abnormal cells in the adrenal cortex.
  • These cancerous cells grow and divide uncontrollably, forming tumors within the adrenal glands. 
  • The tumors may produce excess hormones, leading to hormonal imbalances and associated symptoms.
  • The symptoms of adrenal cortical carcinoma can vary depending on several factors, including the size and location of the tumor and whether it produces excess hormones. Common symptoms may include:
    • Abdominal pain or discomfort
    • Mass or swelling in the abdomen
    • Unexplained weight loss
    • High blood pressure (hypertension)
    • Hormonal changes leading to symptoms such as virilization (masculinization) in females or precocious puberty in children
  • Diagnosis of adrenal cortical carcinoma typically involves imaging studies such as CT scan or MRI to visualize the adrenal glands and detect any abnormalities. 
  • Biopsy may be performed to confirm the diagnosis by examining the tumor tissue under a microscope.
  • Treatment usually involves surgical removal of the tumor, often followed by chemotherapy or radiation therapy to eliminate any remaining cancer cells.

The overall survival rate for adrenal cortical carcinoma in children is generally lower compared to some other childhood cancers, highlighting the importance of early detection and aggressive treatment.

The prognosis for children with adrenal cortical carcinoma depends on various factors, including the stage of the tumor, the extent of spread and the response to treatment.