What to know about adrenocortical carcinoma staging

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The American Cancer Society (ACS) explains that doctors will use one of the two staging systems to describe how far ACC has spread or metastasized.

These are the American Joint Committee on Cancer (AJCC) staging system and the European Network for the Study of Adrenal Tumors (ENSAT).

Both systems stage ACC according to the TNM categories, which stand for “tumor, nodes, and metastases”:

• Tumor (T): The size of the primary tumor and whether it has grown into nearby tissues.
• Nodes (N): Whether the cancer has spread to nearby lymph nodes.
• Metastases (M): Whether the cancer has spread or metastasized to distant organs, lymph nodes, or other tissues in the body.

Each TNM category will also feature a number. The higher this number, the more advanced the cancer.

Tests that can help determine the stage include physical examinations, imaging tests, and biopsies.

The four stages of ACC are as follows:

Stage 1 ACC represents the earliest stage of the disease. The TNM categories associated with this stage are as follows:

• T1: The tumor is approximately 5 centimeters (cm) or less and has not grown into nearby tissues.
• N0: The tumor has not spread to nearby lymph nodes.
• M0: The tumor has not spread to distant parts of the body.

Stage 2 ACC is more severe than stage 1 because the tumor is larger. The TNM categories associated with this stage are as follows:

• T2: The tumor is greater than 5 cm in size but has not grown into nearby tissues.
• N0: The tumor has not spread to nearby lymph nodes.
• M0: The tumor has not spread to distant parts of the body.

Stage 3 ACC may involve changes to the T and N categories, but the M category remains unchanged.

T category

In stage 3 ACC, the tumor category may range anywhere from T1–T4:

• T1: The tumor is less than 5 cm and has not spread to nearby tissues.
• T2: The tumor is larger than 5 cm but has not spread to nearby tissues.
• T3: The tumor may be any size and is growing into the fat surrounding the adrenal gland.
• T4: The tumor may be any size and is growing into nearby organs, such as:
  • the kidney
  • the pancreas
  • the liver
  • the spleen
  • large blood vessels, such as the renal vein, or the vena cava

N category

In stage 3 ACC, the nodes category may appear as either “N1” or “Any N.”

The N1 category indicates that the cancer has spread to nearby lymph nodes, while the “Any N” category simply states that the cancer may or may not have spread to nearby lymph nodes.

M category

Stage 3 ACCs do not involve metastases to different parts of the body. As such, the metastases category is always M0.

Stage 4 ACC is the most advanced stage of the disease.

Here, the tumor category will appear as “Any T,” indicating that the tumor can be any size and that it may or may not have spread into nearby tissues.

Likewise, the nodes category will appear as “Any N,” indicating that the cancer may or may not have spread to nearby lymph nodes.

Finally, the metastases category will appear as “M1,” indicating that the cancer has spread to distant sites, such as the liver or lungs.

Below are answers to some questions that are usually asked about ACC.

How quickly does adrenal cancer progress?

A 2019 review assessed CT scans of adrenal masses to look into the growth rate of adrenal cancer tumors and adrenal adenomas (a type of benign adrenal tumor).

Of the 26 adrenal cancer tumors in this study, all grew at a rate of 5 millimeters (mm) per year. For comparison, only one-third of the 105 adrenal adenomas grew, and each of these did so at a rate of less than 3 mm per year.

There is little information on how quickly ACC may metastasize. Firstly, ACC is a rare disease, affecting an estimated 200 people per year in the United States. Secondly, doctors can rarely detect ACC in its early stages. These factors make it difficult for scientists to study the rate of ACC progression.

What is the life expectancy for people with adrenocortical cancer?

When discussing cancer outlook, doctors may refer to the 5-year relative survival rate (RSR) for a particular cancer type. This is the percentage of people who were living 5 years after diagnosis.

As the ACS explains, the 5-year RSR for individuals living with ACC depends upon how far the cancer has spread at the time of diagnosis.

For this, medical professionals refer to the Surveillance, Epidemiology, and End Results (SEER) database, which classifies cancers into localized, regional, and distant stages.

The table below shows each of the three SEER stages along with their corresponding descriptions and 5-year RSRs.

Adrenocortical carcinoma (ACC) is a rare type of cancer that develops in the outermost part of the adrenal gland called the adrenal cortex.

ACC is so rare, which is why researchers are still working to understand more about its progression.

As with other types of cancer, doctors will stage ACC to determine whether and to what extent the cancer has spread. Staging also helps doctors to make informed decisions about treatment options and outlook.

The cancer stages for ACC range from 1 through 4, with stage 1 representing the earliest stage of the disease and stage 4 the most advanced.

Stage 4 ACC is that which has metastasized to distant parts of the body. Early stage ACC is associated with a higher 5-year relative survival rate.