Adrenal Cortical Carcinoma: Understanding Your Pathology Report

Section Editor: Jason Wasserman MD PhD FRCPC
October 7, 2026

Adrenal cortical carcinoma is a rare cancer that starts in the adrenal cortex, the outer layer of the adrenal gland. The body has two adrenal glands, one on top of each kidney. The adrenal cortex makes hormones that help control blood pressure, blood sugar, and the body’s response to stress.

Many of these tumors also make hormones, and the extra hormones can cause symptoms of their own. Your report may use the name adrenocortical carcinoma, which means the same thing.

This article explains how adrenal cortical carcinoma is diagnosed and what the findings in your pathology report mean. It also notes which findings appear in every report and which are optional.

Who gets adrenal cortical carcinoma?

Adrenal cortical carcinoma most often affects adults between about 40 and 60 years old. Fewer cases occur in young children. It is slightly more common in women than in men.

What causes adrenal cortical carcinoma?

In most adults, the cause of adrenal cortical carcinoma is not known. Some people develop it because they were born with a change in a gene that raises the risk of cancer. An inherited cause is much more common in children than in adults. The inherited conditions linked to this cancer include:

Li-Fraumeni syndrome. An inherited change in the TP53 gene causes this. Our article on Li-Fraumeni syndrome explains more.
Lynch syndrome. Lynch syndrome is best known for colon and uterine cancer. A small number of people with adrenal cortical carcinoma have it.
Beckwith-Wiedemann syndrome. This growth condition starts before birth.
Multiple endocrine neoplasia type 1 (MEN1). Adrenal tumors are common in MEN1, but most are not cancer.

What are the symptoms?

The symptoms of adrenal cortical carcinoma depend on whether the tumor makes extra hormones.

Extra cortisol. This causes Cushing syndrome. Symptoms include abdominal weight gain, a rounded face, muscle weakness, easy bruising, and high blood pressure.
Extra male-type hormones (androgens). In women, these can cause new facial or body hair, acne, a deeper voice, or changes in periods.
No extra hormones. The tumor may cause no symptoms until it is large. It can then cause abdominal or side pain, or a feeling of fullness.

Some adrenal cortical carcinomas are found by accident on a scan done for another reason.

How is the diagnosis made?

The diagnosis of adrenal cortical carcinoma is usually made after the tumor is removed and examined under the microscope by a pathologist. A needle biopsy is usually avoided when this cancer is suspected, because it can spread tumor cells along the needle path.

Before surgery, blood and urine tests show whether the tumor is making extra hormones. Our article on adrenal hormone tests explains these results. A CT or MRI scan shows the size of the tumor.

Under the microscope, the pathologist must decide whether the tumor is a cancer or a noncancerous tumor called an adrenal cortical adenoma. The two can look alike, so no single finding decides it.

The pathologist looks at several features together. These include how many cells are dividing, whether parts of the tumor have died, and whether the tumor has grown into blood vessels or nearby tissue. The more of these features a tumor has, the more likely it is to be a carcinoma.

Once adrenal cortical carcinoma is confirmed, scans of the chest and abdomen look for spread. The next section describes the special stains sometimes used to support the diagnosis.

Immunohistochemistry

Immunohistochemistry is a test that uses antibodies to show which proteins the tumor cells make. For adrenal cortical carcinoma, it helps confirm that the tumor started in the adrenal cortex and not somewhere else.

Laboratories use different stains, and no fixed set is required. Your report may include some of the following:

SF1. A positive result supports a tumor that started in the adrenal cortex. Not every laboratory has this stain.
Inhibin, melan-A, and calretinin. These are often positive in adrenal cortical tumors. They are also positive in some other tumors.
Chromogranin. This is usually negative. A positive result would suggest a different adrenal tumor called pheochromocytoma.

The stains on your report may be different from these, and some reports list very few. These stains show where the tumor started. They do not show how it will behave.

Grade

The grade of adrenal cortical carcinoma describes how quickly the tumor cells are dividing. The pathologist counts the dividing cells, called mitotic figures, in a set area of the tumor. This number is the mitotic count, and it should be in every report.

Low grade. There are 20 or fewer mitotic figures in 10 square millimeters. Some reports describe this area as 50 high-power fields.
High grade. More than 20 mitotic figures are present in the same area.

High-grade tumors tend to grow faster and are more likely to come back or spread. Your team uses the grade when deciding about treatment after surgery.

Ki-67

Ki-67 is a protein found in cells that are getting ready to divide. The Ki-67 index is the percentage of tumor cells that contain it. Like the mitotic count, it should be in every report for adrenal cortical carcinoma. Some reports call the test MIB-1.

A higher Ki-67 index is linked to a higher chance of the cancer coming back after surgery. No cut-off is agreed on, but 10% or less is often treated as lower risk.

Tumor type

Most reports give the diagnosis as adrenal cortical carcinoma with no subtype. This is the usual, or conventional, type. Three less common subtypes are named for how the tumor cells look under the microscope:

Oncocytic. The tumor cells are large and pink. Pathologists use a different set of rules to assess oncocytic tumors, and as a group they tend to grow more slowly than the usual type. Our article on oncocytic adrenal cortical carcinoma describes this subtype in detail.
Myxoid. The tumor cells sit in a gel-like material. The word myxoid describes that material. Naming this subtype helps other pathologists recognize the tumor if it is ever sampled again.
Sarcomatoid. Some of the tumor cells are long and thin and no longer look like adrenal cells. Sarcomatoid tumors are rare. They tend to grow quickly and are more likely to spread.

The subtype does not change how we stage the tumor. Your team considers it, along with the grade and stage, when planning your care.

Tumor size and extension

Your report will give the size of the adrenal cortical carcinoma, measured across its widest point in centimeters (cm). Size matters for the stage. A tumor of 5 cm or smaller is in a lower stage than a larger one.

Your report will also describe tumor extension, which means how far the tumor has grown. This helps determine the tumor stage.

Confined to the adrenal gland. The tumor has not grown outside the gland. This is the most favorable result. A tumor that is still inside the gland is more likely to be removed completely.
Invasion into extra-adrenal tissue. The tumor has grown beyond the edge of the gland into the surrounding fat. This raises the stage, and it means the cancer is more likely to come back than a tumor confined to the gland.
Invasion into adjacent organs. The tumor has grown directly into a nearby organ, such as the kidney or liver, or into a large vein. This is the highest tumor stage. The surgeon may have removed part of that organ along with the tumor.

Other features your report may list

The pathologist records several other features of adrenal cortical carcinoma. Most are reported as present or not identified. They help the pathologist confirm that the tumor is a cancer. A carcinoma does not need to show all of them, and no single one changes your treatment on its own.

Tumor necrosis. Necrosis means areas of dead tumor cells. It happens when a tumor grows faster than its blood supply can support. Necrosis is common in adrenal cortical carcinoma and uncommon in noncancerous adrenal tumors.
Atypical mitotic figures. An atypical mitotic figure is a cell that is dividing abnormally. Normal cells do not divide like this, so finding even one supports a cancer diagnosis.
Capsular invasion. Most of these tumors are surrounded by a thin layer of tissue called a capsule. Capsular invasion means tumor cells have grown into or through it. This is a sign of cancer. It is not the same as growing outside the adrenal gland, and it does not change the stage.
Nuclear grade. The nucleus is the part of a cell that holds its DNA. Nuclear grade describes how abnormal the nucleus looks, and it is reported as low or high. Most adrenal cortical carcinomas have a high nuclear grade. This is not the same as the tumor grade, which is based on the mitotic count.
Architecture. This describes how the tumor cells are arranged. Normal adrenal cells grow in neat cords and nests. “Diffuse” means the tumor cells grow in sheets with no pattern, which is typical of carcinoma.
Clear cells. Normal adrenal cells are filled with fat, which makes them look pale or clear. Your report may call them lipid-rich cells. A tumor with few clear cells looks less like normal adrenal tissue, and this is typical of carcinoma.

Scoring systems

Some pathologists combine the features above into a score. The score helps decide whether a tumor is an adrenal cortical carcinoma or a noncancerous adenoma. These scores are optional. Your report may name one, more than one, or none.

Weiss score. This is the best-known system. The pathologist checks for nine features and gives one point for each one found. A score of 3 or more supports carcinoma. A higher score does not mean a more advanced cancer. Stage, grade, and Ki-67 index are better guides to outlook.
Modified Weiss score. This shorter version uses five of the nine features. A score of 3 or more supports carcinoma.
Helsinki score. This combines the mitotic count, necrosis, and the Ki-67 index into one number. A score above 8.5 supports carcinoma. Higher scores are linked to a higher chance of the cancer spreading.
Reticulin. Reticulin is a stain that shows the fine fibers that hold groups of cells in place. In a noncancerous tumor, these fibers form a regular mesh, and the report describes them as intact. In a carcinoma, the mesh is broken up, and the report may describe it as altered or disrupted.
Lin-Weiss-Bisceglia system. This system is used only for oncocytic tumors, because the Weiss score does not work well for them. It does not give a number. It sorts the tumor into benign, uncertain, or malignant.
Wieneke criteria. This system is used for children. Adrenal tumors in children often behave better than they look, so the adult scores can overstate the risk.

A report without a score is not incomplete. The score adds up the features, and the features themselves matter.

Vascular and lymphatic invasion

Vascular invasion means cells from the adrenal cortical carcinoma are seen inside a blood vessel. Tumor cells inside a vein, called venous invasion, are linked to a higher chance of the cancer spreading.

Lymphatic invasion means tumor cells are seen inside a thin channel that carries fluid to the lymph nodes. Some reports call this sinusoidal invasion. Both findings are reported as present or not identified.

Margins

A margin is the edge of tissue the surgeon cuts when removing the tumor. The pathologist examines the margins to see whether any tumor cells are present at the cut edge.

Negative margin. No tumor cells are seen at the cut edge. This suggests the tumor was completely removed. Some reports call this R0.
Positive margin. Tumor cells are seen at the cut edge. This suggests some tumor may remain. Some reports call this R1.

Your report will also say whether the tumor arrived in one piece. Terms such as “capsule disrupted” or “fragmented” mean the surface of the tumor was opened, or the tumor was in pieces. This raises the chance of the cancer coming back in the same area, and it can make the margins hard to examine.

Pathologic stage

The pathologic stage describes the size of the adrenal cortical carcinoma and whether it has spread. It is assigned after the tumor is removed, using the AJCC staging system, 8th edition. The tumor stage (pT) is:

pT1. The tumor is 5 cm or smaller and has not grown outside the adrenal gland.
pT2. The tumor is larger than 5 cm and has not grown outside the adrenal gland.
pT3. The tumor has grown into the surrounding tissue.
pT4. The tumor has grown into a nearby organ or a large vein.

Lymph nodes are not removed in every operation for this cancer. If they were, the report states how many were examined and how many contain cancer. The nodal stage is pN0 when none contain cancer and pN1 when at least one does. If no lymph nodes were removed, your report may say pNX or that the nodal stage was not assigned.

Your doctors combine the pathologic stage with your scans to give an overall stage from I to IV. Stages I and II are tumors confined to the adrenal gland. Stage III means growth outside the gland or cancer in nearby lymph nodes. Stage IV means spread to distant organs, most often the liver or lungs.

Genetic testing

Because adrenal cortical carcinoma can be linked to an inherited condition, you may be offered genetic counseling and a blood test. This is discussed most often with children, younger adults, and people with a family history of cancer. The result is reported separately from your pathology report.

Some laboratories also test the tumor itself. They may list stains for p53, beta-catenin, or mismatch repair proteins. These tests are optional, and many reports do not include them. An abnormal mismatch repair result does not confirm Lynch syndrome, but it leads to a referral for genetic counseling.

What is the prognosis?

The outlook for adrenal cortical carcinoma depends mostly on how far the cancer had spread when it was found and whether all of it could be removed.

Stage I. About 8 in 10 people are alive five years after diagnosis.
Stage II. About 6 in 10 people are alive five years after diagnosis.
Stage III. About 5 in 10 people are alive five years after diagnosis.
Stage IV. About 1 in 10 people are alive five years after diagnosis.

These numbers are averages from large groups of people, many of whom were treated years ago. They cannot tell you what will happen to you.

Features in your report that are linked to a higher chance of the cancer coming back include:

Margins. A positive margin or a tumor that was not removed in one piece means some tumor cells may have been left behind.
Ki-67 and grade. A high Ki-67 index or a high grade means the tumor cells are dividing quickly.
Venous invasion. Tumor cells inside a vein can travel to other parts of the body.
Stage. Growth outside the adrenal gland or cancer in lymph nodes means the cancer has started to spread.

What happens after the diagnosis?

A team at a center experienced with adrenal tumors usually plans the care for adrenal cortical carcinoma. Your team uses the stage, margins, grade, and Ki-67 index to decide which options to discuss with you.

Follow-up. Follow-up includes regular scans and hormone tests. This may be all that is needed when the chance of the cancer coming back is lower.
Mitotane. Mitotane is a drug that acts on adrenal cortical cells. It may be offered after surgery when the chance of the cancer coming back is higher. It is also used when the cancer cannot be removed or has spread.
More surgery or radiation. These may be considered when a margin is positive.
Chemotherapy. Chemotherapy may be offered, often together with mitotane, when the cancer has spread.
Steroid tablets. A tumor that makes extra cortisol can switch off the other adrenal gland. Steroid tablets are often needed until that gland recovers.

The FDA and Health Canada have approved mitotane for adrenal cortical carcinoma that cannot be removed by surgery. Expert guidelines support its use after surgery. Access to treatment differs between countries, and your team can explain what applies where you live.

Questions to ask your doctor

How large was my tumor, and was it removed in one piece?
Had the tumor grown outside the adrenal gland?
Is my tumor low grade or high grade?
What was my Ki-67 index?
Was venous invasion found?
Were the margins negative?
Were any lymph nodes examined?
What is the stage of my tumor?
Did my tumor make hormones, and will I need steroid tablets after surgery?
Is mitotane recommended for me, and why or why not?
Should I be referred for genetic counseling?
How often will I need follow-up scans and hormone tests?