Adrenocortical Carcinoma: Symptoms, Causes, and Treatment Options

Adrenocortical carcinoma is a rare cancer that starts in the outer layer of the adrenal gland and can produce excess hormones or grow silently until it becomes large. Symptoms, diagnosis, and treatment vary, but care usually involves specialist evaluation, imaging, hormone testing, and often surgery.

Overview

Adrenocortical carcinoma is a rare cancer that begins in the adrenal cortex, the outer layer of the adrenal gland. The adrenal glands sit above the kidneys and help make hormones that regulate blood pressure, metabolism, stress response, salt balance, and some sex-related functions. When cancer develops in this area, it may either produce too much hormone or grow as a mass that causes pressure-related symptoms.

This condition is different from the more common benign adrenal adenoma, which is often found incidentally and may never cause harm. It is also different from pheochromocytoma, a tumor of another part of the adrenal gland. Because adrenocortical carcinoma is uncommon, diagnosis and treatment are best guided by teams experienced in endocrine tumors and cancer care.

Some people are diagnosed because they develop clear hormone-related changes, while others learn about the tumor after imaging for another reason. The outlook depends on factors such as tumor size, whether it has spread, whether hormones are being overproduced, and whether complete surgical removal is possible.

How adrenocortical carcinoma can affect the body

Adrenocortical carcinoma may behave in two broad ways. A functioning tumor makes excess hormones, which can create noticeable changes in the body even when the tumor is not very large. A nonfunctioning tumor does not produce significant hormone excess, so symptoms may come mainly from the tumor pressing on nearby structures.

Excess cortisol is one of the more common hormone patterns. This can lead to rapid weight gain, easy bruising, muscle weakness, high blood pressure, high blood sugar, mood changes, and a rounded face. In some people, the tumor may also produce androgens or estrogens, leading to acne, excess facial or body hair, menstrual changes, reduced libido, breast enlargement in men, or other signs of hormone imbalance.

When the tumor grows without major hormone production, symptoms can be less specific. People may notice abdominal pain, back pain, a sense of fullness, early satiety, or an enlarging abdomen. This is one reason the condition can be challenging to recognize early.

Because hormone excess can affect many organs at once, care often includes both cancer treatment and management of the body-wide effects of abnormal hormone levels. Evaluation may overlap with assessment for related adrenal conditions such as adrenal gland disorders.

Symptoms and signs

Symptoms of adrenocortical carcinoma depend on whether the tumor is making hormones and how large it has become. Some people have a combination of hormone-related changes and mass-related discomfort, while others have only one pattern. Symptoms can develop gradually or become more noticeable over a relatively short period.

Possible symptoms and signs include:

• Unexplained weight gain, especially with swelling of the face or abdomen
• High blood pressure or worsening blood sugar control
• Muscle weakness, fatigue, or easy bruising
• New or worsening acne
• Increased facial or body hair growth, especially in women
• Menstrual irregularities or fertility changes
• Breast enlargement or reduced sex drive in men
• Abdominal pain, back pain, or a feeling of fullness
• An abdominal mass discovered on examination or imaging

Children can present differently, often with signs of early puberty or unusual growth patterns related to hormone production. In adults, symptoms may be mistaken for more common endocrine problems at first. Any combination of rapid hormone-related changes and an adrenal mass deserves careful evaluation.

Causes and risk factors

In many people, there is no single clear cause of adrenocortical carcinoma. Cancer develops when cells in the adrenal cortex acquire changes that allow them to grow out of control. Researchers have identified genetic changes linked to this process, but most cases are not caused by anything a person did or did not do.

A small number of cases are associated with inherited cancer syndromes. These may include Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia type 1, and familial adenomatous polyposis. A personal or family history of rare endocrine tumors can therefore be relevant during assessment.

Age can influence presentation, with cases occurring in both children and adults, though patterns differ by age group. Most adrenal masses found in adults are not adrenocortical carcinoma, which is why imaging findings must be interpreted carefully rather than assumed to be cancer.

There are no proven lifestyle measures that specifically prevent this cancer. Still, regular medical follow-up for inherited syndromes and timely evaluation of suspicious symptoms may help lead to earlier diagnosis.

Diagnosis and staging

Diagnosis usually starts with a review of symptoms, a physical examination, and blood pressure and metabolic assessment. If hormone excess is suspected, doctors may order blood and urine tests to look at cortisol, androgen, estrogen, aldosterone, and related hormone patterns. These tests help show whether the adrenal mass is functioning and can also guide symptom control.

Imaging plays a central role. CT and MRI are commonly used to assess the adrenal gland, estimate tumor size, and look for features that suggest cancer rather than a benign growth. Chest or abdominal imaging may also be used to check whether the cancer has spread. In selected situations, PET imaging may be considered.

Biopsy is not routinely the first step for an adrenal mass suspected to be adrenocortical carcinoma. That is because hormone-secreting tumors and certain adrenal tumors require careful handling, and biopsy may not always provide the most useful or safest path to diagnosis. Treatment planning often proceeds from imaging, hormone studies, and surgical evaluation.

Staging describes whether the cancer is confined to the adrenal gland, has spread to nearby tissues or lymph nodes, or has metastasized to distant organs such as the liver or lungs. This staging is important because it helps shape the goals of care and the combination of treatments that may be appropriate.

Treatment options

The main treatment for localized adrenocortical carcinoma is surgery to remove the tumor completely when this can be done safely. Complete removal offers the best chance for long-term disease control. The operation may also involve nearby tissues or lymph nodes if they are affected. For many patients, evaluation by teams experienced in oncology care and endocrine surgery is important before proceeding.

If the tumor is producing excess hormones, treatment may also include medicines to lower hormone levels and reduce symptoms before or after surgery. This can help stabilize blood pressure, blood sugar, potassium balance, and other hormone-related effects. Ongoing follow-up is needed because hormone levels may change after treatment.

When the cancer has spread or cannot be completely removed, treatment may include systemic therapy, such as adrenal-directed medicines, chemotherapy, or other cancer drugs depending on the individual situation. Some patients may also receive chemotherapy or radiation to control symptoms or lower the chance of local recurrence after surgery in selected cases.

Supportive care is also an important part of treatment. It may involve pain control, nutritional support, management of treatment side effects, and help with blood pressure or diabetes related to hormone excess. In complex cases, patients may be assessed through advanced cancer screening and staging services and multidisciplinary review. Near the end of the treatment pathway, some international patients may choose centers such as Acibadem International, where multidisciplinary specialists in JCI-accredited hospitals diagnose and treat adrenal cancers.

Follow-up, self-care, and living with the condition

After treatment, regular follow-up is essential because adrenocortical carcinoma can recur. Follow-up often includes physical examination, blood or urine hormone testing, and repeat imaging at intervals chosen by the treating team. Monitoring also helps identify treatment side effects and hormonal changes that may need long-term management.

Self-care does not replace medical treatment, but it can support recovery. Patients may benefit from taking medicines exactly as prescribed, attending all follow-up visits, and reporting new symptoms promptly. Good nutrition, gradual return to activity, blood pressure and blood sugar monitoring when advised, and emotional support can all be helpful.

People recovering from cortisol excess may feel weak or tired for some time even after the tumor is treated. This does not always mean the cancer is worsening, but it should still be discussed with a doctor. Some patients need help from endocrinology, nutrition, rehabilitation, or mental health professionals during recovery.

Because adrenal tumors can overlap with other endocrine disorders, it may also help to understand the broader category of adrenal cancer when discussing follow-up and long-term outlook with a specialist.

When to seek medical care

Medical attention is important if a person develops rapid, unexplained hormone-related changes such as sudden weight gain, severe acne, increased facial hair, menstrual changes, unusual bruising, muscle weakness, or difficult-to-control blood pressure or blood sugar. These symptoms do not always mean cancer, but they should be assessed, especially when several occur together.

A doctor should also review persistent abdominal pain, back pain, a feeling of fullness, or an adrenal mass found unexpectedly on an imaging scan. If there is a family history of inherited cancer syndromes or rare endocrine tumors, this information should be shared during the visit because it may affect testing and follow-up.

Urgent care may be needed for severe weakness, very high blood pressure, sudden worsening blood sugar control, or other symptoms that suggest significant hormone imbalance. Early specialist review can help clarify the cause and guide the safest next steps.

Frequently asked questions

Is adrenocortical carcinoma the same as adrenal cancer?

Adrenocortical carcinoma is one type of adrenal cancer. It starts in the adrenal cortex, the outer layer of the adrenal gland that produces several important hormones. Not all adrenal tumors are adrenocortical carcinoma, and many adrenal masses are benign.

What are the first symptoms of adrenocortical carcinoma?

Early symptoms can vary widely. Some people notice hormone-related changes such as weight gain, high blood pressure, acne, increased body hair, or menstrual changes, while others mainly have abdominal or back discomfort. In some cases, there are no obvious symptoms at first.

Can adrenocortical carcinoma be cured?

Some patients can be treated successfully, especially when the tumor is found before it has spread and can be removed completely with surgery. However, the outlook depends on stage, hormone activity, and how fully the cancer can be treated. A specialist team can explain the likely goals of treatment in an individual case.

Do all adrenal masses need surgery?

No. Many adrenal masses are benign and may only need monitoring or hormone testing. Surgery is considered when imaging or hormone results suggest cancer, significant hormone excess, or other risks that make removal the safest option.

How is adrenocortical carcinoma diagnosed?

Diagnosis usually combines a medical history, physical examination, blood and urine hormone tests, and imaging such as CT or MRI. Doctors also use these results to stage the cancer and plan treatment. Biopsy is not always the first or best step for an adrenal mass.

Is adrenocortical carcinoma hereditary?

Most cases are not inherited, but some are linked to rare genetic syndromes. A family history of certain cancers or endocrine tumors may prompt genetic counseling or additional testing. This is especially important for younger patients or families with multiple related conditions.

References

• National Cancer Institute
• American Cancer Society
• National Organization for Rare Disorders
• European Society for Medical Oncology
• Endocrine Society

This article is for general information only and is not a substitute for professional medical advice. Please consult a qualified doctor about your individual situation.