Adrenocortical cancer

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Adrenocortical cancer at a glance

Adrenocortical carcinoma, often shortened to ACC, is a rare cancer arising in the outer part of an adrenal gland. There are two adrenal glands, one above each kidney. The outer layer, or cortex, makes steroid hormones. ACC can cause problems because of a growing tumour, excess hormone production, or both. Care is best coordinated by a team experienced in adrenal cancer.

Many adrenal lumps found on scans are non-cancerous growths called adenomas, and cancer from another organ can also spread to an adrenal gland. Neither is the same as ACC. Pheochromocytoma arises in the inner adrenal region and has a different pathway. This guide concerns adult ACC; children need a paediatric team with relevant expertise.

What matters first

The first priorities are to identify the nature of the adrenal mass, assess hormone production and determine whether complete surgical removal is possible. A suspicious adrenal tumour should not automatically undergo a needle biopsy. Unlike many other cancers, a biopsy may be unhelpful or unsafe when a potentially removable ACC is suspected. Specialist planning before a procedure is important.

Early disease can sometimes be cured by complete surgery. Advanced disease may be treated to control tumour growth and hormone effects. Because ACC is rare, there is uncertainty about some treatment choices. An experienced multidisciplinary review and a clear explanation of that uncertainty are particularly valuable.

Symptoms and warning signs

Symptoms may include persistent abdominal or back discomfort, a feeling of fullness, an abdominal mass or unexplained weight loss. Hormone-producing tumours can cause more distinctive changes, including rapid weight gain around the trunk, easy bruising, new diabetes, high blood pressure, muscle weakness, excess hair growth or changes in periods or sexual function.

Severe weakness, confusion, vomiting, collapse, chest pain or breathlessness needs urgent care. After adrenal surgery or during mitotane treatment, low cortisol can be dangerous, especially during infection or vomiting. If you have a steroid emergency plan, follow it while seeking urgent help; do not wait for the next clinic appointment.

Making this information useful to you

A cancer diagnosis can arrive before you have had time to understand the tests. You do not need to learn every treatment name at once. Begin with the exact diagnosis, where the cancer is, and what the team recommends next. A relative or friend can join the appointment if you would find that helpful. Ask for an interpreter when language makes a discussion harder; important decisions should not depend on guessing unfamiliar words.

This guide explains possibilities, not a prescription for your situation. Your pathology, scan findings, general health, previous treatment and priorities determine which possibilities apply. Two people with the same cancer name may appropriately receive different treatment. If a recommendation differs from something you have read, ask the team to explain the reason and the evidence behind it.

Keep a list of medicines, allergies and other health conditions, including non-prescription products, vitamins and herbal remedies. Tell the team about previous cancer treatment, pregnancy or breastfeeding, and anything that could affect attending appointments. Do not stop an established medicine without advice. Bring your questions in the order that matters most to you, so that the most important concern is discussed first.

Understand

Adrenal hormones and cancer

Cortisol helps the body respond to stress and influences blood pressure and glucose. Aldosterone helps regulate salt and potassium. The adrenal cortex also produces sex-hormone precursors. An ACC may produce too much of one or several hormones, or no obvious hormone excess. A non-functioning tumour can still be malignant and require treatment.

Excess cortisol can cause Cushing syndrome, with weakness, fragile skin, bruising, infection risk, high glucose and blood clots. Excess androgens can cause new hair growth, acne, voice changes or menstrual disturbance. Oestrogen production can cause breast enlargement or sexual changes in men. Aldosterone-related excess can cause high blood pressure and low potassium, although it is not the usual ACC presentation.

Hormone symptoms sometimes improve before the tumour shrinks, or need separate medicines even while cancer treatment is working. Monitoring both is important. A hormone test alone does not prove cancer, because benign adrenal conditions can also produce hormones.

Risk factors and inherited conditions

Most ACC occurs without a known inherited syndrome. Some cases are associated with conditions such as Li–Fraumeni syndrome, Lynch syndrome or other rare inherited disorders. Genetic assessment is especially relevant in younger patients, suggestive personal or family histories, and circumstances identified by the specialist team.

Testing should be accompanied by counselling about what a result means for you and relatives. A gene alteration found only in a tumour is not necessarily inherited. Family members should not begin surveillance or assume they carry a mutation without appropriate advice and testing.

Why specialist pathology matters

Distinguishing a benign adrenal cortical tumour from ACC can require assessment of several microscopic features and the tumour's behaviour. The surgical specimen usually provides more information than a small needle sample. Expert review can clarify difficult or borderline findings and reduce the chance of using the wrong treatment pathway.

The report may describe a Weiss score, a system that combines microscopic features to help diagnose ACC. It also records Ki-67, whether cancer has entered blood vessels, and the margins: the edges of the removed tissue. Ki-67 estimates how actively cells are dividing and helps assess recurrence risk. No single number should be interpreted without the rest of the pathology and clinical information.

Tests and diagnosis

Hormone tests before treatment

An endocrine assessment looks for cortisol and sex-hormone excess and other abnormalities appropriate to the presentation. Blood pressure and potassium help determine whether aldosterone testing is needed. Tests for pheochromocytoma are important before an invasive procedure when an adrenal mass is being evaluated, because an unrecognised catecholamine-producing tumour can cause a dangerous crisis.

Some tests require particular timing, medicines or preparation. Follow the endocrinology team's instructions rather than stopping medicines independently. If cortisol is high, treatment may be needed before surgery to reduce infection, clotting and metabolic risks. Diabetes and blood pressure also need active management.

Scans and staging tests

Adrenal-focused computed tomography (CT) or magnetic resonance imaging (MRI) assesses size, appearance, local invasion and the relationship to the kidney, liver and major blood vessels. Chest imaging checks for lung spread. Additional scans, including positron emission tomography combined with CT (PET-CT), using a radioactive glucose tracer called FDG, in selected cases, help answer specific staging questions. The goal is to determine whether all visible disease can be safely removed and whether another diagnosis is more likely.

A suspicious large or invasive mass warrants specialist review, but size alone does not establish ACC. Scans should be compared with earlier studies if available. Growth rate, imaging characteristics and hormone findings are interpreted together. An adrenal mass discovered during follow-up of another cancer may represent a metastasis rather than a new adrenal primary.

When biopsy is and is not useful

A needle biopsy is generally avoided for a potentially resectable suspected ACC because it may not reliably distinguish benign from malignant cortical tissue and can risk tumour spillage. It is not a routine shortcut before referral. The team should first consider whether the result would change management and whether surgery is already indicated.

Biopsy may be considered when disease is unresectable or another diagnosis, such as metastasis or lymphoma, is suspected and tissue is necessary for systemic treatment. Pheochromocytoma must be excluded first. The safest sampling site may be outside the adrenal gland. This decision should be made by the specialist team, not applied as a blanket rule to every adrenal mass.

Understanding recurrence risk

After surgery, recurrence risk is influenced by stage, margins, Ki-67, tumour rupture and other features. R0 means no tumour at the microscopic resection margin; R1 means microscopic involvement, while visible residual disease is different again. Ask how confident the team is that the tumour was removed completely and which findings drive the recommendation for further treatment.

Preparing for your appointment

For your consultation or a second opinion, bring a CD containing your scan images (DICOM format) and the software needed to view them, along with the written scan reports. Bring your biopsy report and ask whether the pathology slides or tissue blocks are needed for specialist review or additional tests. Keep originals safe and agree how borrowed material will be returned. Having the actual images allows the team to compare changes and plan treatment accurately.

A repeat test is sometimes needed because an earlier sample was too small, a scan did not cover the necessary area, or a new result will change treatment. Ask what question the test will answer, how it may affect the plan, and when you should expect the result. A result described as uncertain does not automatically mean the cancer is worse. Sometimes discussion between the radiologist, pathologist and treating specialists resolves the uncertainty without another procedure.

Before a scan or procedure, mention kidney problems, previous contrast reactions, implanted devices, diabetes medicines, blood-thinning medicines and the possibility of pregnancy. Follow the testing centre's instructions about food, drink and medicines. If you have not received those instructions, contact the centre rather than making changes yourself.

Planning your treatment

How your treatment plan is chosen

An experienced endocrine surgeon, endocrinologist, oncologist, radiologist and pathologist should review your diagnosis and treatment options together. The plan needs to address both cancer control and hormone safety. For a potentially curable tumour, the quality and planning of the first operation are especially important. Referral before an unplanned procedure can preserve treatment options.

Ask whether complete removal is realistic, whether nearby organs or vessels may need surgery, and how hormone problems will be controlled before and afterwards. If the cancer has spread, ask whether surgery or local treatment to selected sites is likely to help or whether systemic treatment should come first. More surgery is not automatically beneficial in widespread disease.

Understanding benefit and uncertainty

Evidence in rare cancers is often based on smaller studies than in common cancers. The team should distinguish an established approach from an option supported by limited data. Ask what the likely benefit is, how side effects will be monitored and what would lead to stopping or changing treatment. An expert second opinion can be helpful without implying distrust of the current team.

Questions specific to adrenal cancer

Ask whether hormone excess is present, whether genetic assessment is appropriate, and whether pathology has had expert review. If mitotane is proposed, ask about blood-level monitoring, steroid replacement and interactions. Make sure you understand the difference between treatment after complete surgery to reduce recurrence risk and treatment of visible advanced cancer.

Questions to ask your care team

• What is the exact cancer type and stage, and are any important results still pending?
• Is the aim to cure the cancer, reduce the chance of recurrence, control it, or relieve a particular symptom?
• What benefit do you expect from this option, and what are the reasonable alternatives for me?
• How will treatment affect everyday activities, fertility, sexual health and the things I most value?
• How will we know whether it is working, and when will we review the plan?
• Who should I contact between visits, including at night or over a weekend?

Clinical trials and newer treatments

A clinical trial can be worth considering at diagnosis or when treatment needs to change. Trials ask a defined question; some compare established treatments and others study a newer approach. Eligibility depends on the cancer, previous treatment, test results and health. Taking part is voluntary, and choosing not to participate does not remove your right to standard care. Ask about extra visits, possible randomisation, costs, travel, side effects and what happens when the study ends.

Newer treatments are considered using the clinical evidence, recognised guidelines and the exact cancer type, biomarkers and previous treatment. Some options have less mature evidence than established treatments. Ask what is known, what remains uncertain and whether a clinical trial would be appropriate.

Your priorities and supportive care

Tell the team what you are hoping treatment will make possible: returning to work, caring for family, keeping independence, reducing pain or attending an important event. These priorities can influence a choice between reasonable options. If the likely benefit is small and the burden is substantial, discussing a less intensive approach is appropriate. Decisions can be revisited when your health or circumstances change.

Supportive and palliative care help with symptoms, sleep, mood, family concerns and planning. They can be provided alongside treatment intended to cure or control cancer. A referral does not mean that active treatment is ending. If the cancer cannot be cured, ask what treatments may help you live longer or more comfortably and how their burdens compare with their likely benefits.

Treatment options

Surgery for localised disease

Complete removal with an intact tumour capsule offers the best chance of long-term disease control. The operation removes the affected adrenal gland and, when necessary, adjacent involved tissues. Avoiding rupture and spillage is important. Open surgery is often appropriate for a large or invasive suspected ACC; minimally invasive surgery is reserved for carefully selected cases in expert hands.

The surgical approach should be chosen for cancer safety, not only incision size or speed of recovery. Ask about lymph node assessment and the possibility of removing an adjacent organ if directly involved. A well-functioning opposite adrenal gland can often supply hormones after recovery, but cortisol-producing tumours can suppress it temporarily, making steroid replacement necessary.

Steroid care around surgery

If the tumour produces cortisol, the body may not immediately make enough cortisol after it is removed. The team may provide perioperative hydrocortisone and a gradual replacement plan. Blood tests and clinical assessment guide recovery. Do not stop steroids simply because the tumour has been removed or because you feel well.

Ask for a steroid emergency card, sick-day instructions and an emergency injection plan when indicated. Family members should know what to do if you are vomiting or unable to take tablets. This safety planning is as important as the wound-care instructions.

Mitotane after surgery

Mitotane can be considered after complete surgery when recurrence risk is high, for example with concerning stage, margins or Ki-67. It is not automatically required for every resected ACC. Treatment can be prolonged and requires close monitoring because blood levels rise slowly and side effects can be substantial.

The ADIUVO trial did not show a significant recurrence-free survival benefit in a selected low-to-intermediate-risk group after complete resection and found treatment-related burden. The trial was small and stopped early, so it does not prove that mitotane can never help. It supports an individual discussion about observation in lower-risk disease rather than routinely treating everyone. Its findings should not be transferred to clearly high-risk patients.

How mitotane is monitored

Mitotane affects adrenal tissue and alters the metabolism of many medicines. Blood-level testing helps balance activity and toxicity; the dose is adjusted by the specialist team rather than by symptoms alone. Liver tests, blood counts, cholesterol, thyroid and other hormone assessments may be needed. Side effects can persist because the medicine remains in the body for a long time.

Most patients need glucocorticoid replacement during treatment unless ongoing cortisol excess changes the plan. Replacement requirements can be higher than usual because mitotane accelerates steroid breakdown. Some also need mineralocorticoid replacement. The endocrinology team should explain the regimen and how to respond to illness, surgery or missed doses.

Radiotherapy and local treatment

Radiotherapy may be considered for selected high-risk local situations after surgery, residual disease or symptom control. It is not a routine substitute for complete resection of a removable tumour. Treatment to painful bone metastases or another threatening site can help quality of life. Ablation or other local approaches may be considered for a limited number of lesions in selected patients.

If recurrence is confined to a small area after a substantial disease-free interval, further surgery may be worthwhile when complete removal is feasible. The likelihood of benefit depends on disease pace, distribution and prior treatment. Repeated procedures should have a clear purpose and be weighed against recovery time and complications.

Cancer that has spread or cannot be removed

Systemic treatment is considered when disease cannot be completely removed or is progressing. A commonly used combination is etoposide, doxorubicin and cisplatin with mitotane, often called EDP–M. The choice depends on fitness, kidney and heart function, disease pace and hormone problems.

Mitotane alone may be considered in selected slower-growing or less aggressive situations. Other guideline-supported chemotherapy choices include cisplatin or carboplatin with etoposide, with doxorubicin, mitotane or both added when appropriate. These combinations have different burdens and should be selected by a team experienced in ACC.

Treatment may shrink or stabilise cancer and help hormone symptoms, but benefit is not guaranteed. Scans and clinical review assess whether it is helping. If a good response changes the possibility of surgery or local treatment, the multidisciplinary team can reassess. It is important not to promise that chemotherapy will make every unresectable tumour operable.

Later treatment and clinical trials

Options beyond the initial plan include other chemotherapy, cabozantinib, mitotane, or pembrolizumab with or without mitotane in appropriate patients. Nivolumab with ipilimumab is another option in selected circumstances. The evidence, side effects and suitability of these treatments differ; they are not interchangeable or a universal replacement for platinum-based chemotherapy.

A clinical trial remains valuable. A biomarker such as mismatch-repair deficiency or microsatellite instability may provide additional support for an immune-treatment choice, but is not the only context in which immunotherapy may be considered for ACC.

Evidence for pembrolizumab with mitotane is particularly limited, including a published report of six patients. That report did not compare the combination with pembrolizumab alone.

Broad molecular testing can sometimes help identify trials or uncommon actionable findings, but a test may not produce a useful treatment. Discuss cost and what results would change management before ordering it. Not every drug studied in ACC has sufficient evidence for routine use. Mitotane remains a central established medicine; newer approaches need careful assessment of the evidence and suitability.

Controlling hormone excess

Hormone control is an active part of treatment even if the tumour itself cannot be cured. Medicines that reduce cortisol production may be needed, sometimes urgently, with monitoring for interactions and adrenal insufficiency. Blood pressure, potassium, glucose, infection risk and clot risk require coordinated management. The specific medicine depends on severity and the treatment plan. Osilodrostat is one option for controlling excess cortisol in selected cases; hormone control is its purpose, rather than directly treating tumour growth.

Severe cortisol excess can make someone very unwell without a dramatic change in tumour size. Conversely, reducing cortisol too far can also be dangerous. Never adjust hormone-blocking medicines or replacement steroids independently. Ask how the team will distinguish symptoms of excess hormone from symptoms of too little hormone.

Managing side effects

Recognising adrenal insufficiency

Low cortisol can cause profound fatigue, nausea, vomiting, abdominal pain, dizziness or low blood pressure. During illness it can progress to adrenal crisis with collapse or confusion. If you cannot keep prescribed steroids down, follow your emergency injection instructions if you have them and seek emergency care. Tell emergency staff that you have adrenal insufficiency or are receiving mitotane.

A normal-looking wound or a recent reassuring scan does not protect against this problem. Carry your steroid information and keep replacement medicines available when travelling. The oncology and endocrine teams should agree who is responsible for dose adjustments and emergency advice.

Mitotane and chemotherapy effects

Mitotane can cause nausea, diarrhoea, loss of appetite, dizziness, drowsiness, poor concentration or problems with balance. Report neurological symptoms promptly because they can indicate excessive drug exposure. Avoid driving or hazardous activities if affected. Blood-level and laboratory monitoring are important even when you feel reasonably well.

EDP chemotherapy can cause low blood counts, infection risk, nausea, hair loss and fatigue. Cisplatin can affect kidneys, hearing and nerves, while doxorubicin requires attention to heart function. The team may use hydration, anti-sickness medicines and other supportive treatment. Tell them about hearing change, numbness, swelling or breathlessness rather than waiting for a severe problem.

Medicines, contraception and everyday safety

Mitotane has many interactions, including with steroids and hormonal contraceptives. Ask the team or oncology pharmacist to review every medicine and supplement. If pregnancy is possible, use effective non-hormonal contraception during treatment and after stopping until blood tests confirm that mitotane is no longer detectable. Ask your specialist to confirm this plan and when it is safe to consider pregnancy, because mitotane remains in the body after treatment. Do not rely on a general rule from a different cancer medicine.

If appetite and strength are poor, endocrine control and nutrition need to be reviewed together. Muscle weakness from cortisol excess may take time to improve. Rehabilitation should be adapted to fracture risk, blood pressure and current fitness. Recovery is often gradual rather than an immediate return to normal after tumour removal.

Keeping treatment manageable

Keep the team's contact details available and know where to go outside clinic hours. Report side effects early rather than waiting until the next appointment. A short record of symptoms, temperature, fluid intake and medicines can help the team decide what is needed. Do not change cancer medicine doses, restart a stopped treatment, or use leftover antibiotics without instructions. A planned delay or dose adjustment can be an important part of safe treatment.

If you receive treatment that can lower white blood cells, a temperature of 38°C (100.4°F) or higher needs urgent advice. Follow any lower threshold or additional instructions your team gives you. Chills, sudden weakness, confusion or feeling very unwell also need urgent assessment, even without a fever. Do not wait for a blood test appointment or try to hide the fever with medicine before speaking to the team.

Severe breathlessness, chest pain, a new seizure, sudden weakness, heavy bleeding or collapse require emergency care. New severe back pain with leg weakness, numbness around the bottom, or loss of bladder or bowel control also needs immediate assessment. Contact the nearest emergency service when necessary; a website contact form or routine messaging service is not an emergency pathway.

Eating, drinking and energy

Treatment may change appetite, taste, bowel habits and energy. Small, frequent meals and foods containing protein can be easier than large meals. Ask for dietitian support if weight is falling, swallowing is difficult, or you are avoiding many foods. There is no single diet that treats cancer, and severe restriction or prolonged fasting can make it harder to tolerate treatment. Check supplements with your team because some interact with medicines.

Take anti-sickness medicine as prescribed and report vomiting that prevents you keeping down fluids or essential medicines. Seek advice for persistent diarrhoea, dizziness, very little urine or increasing weakness. Fluid recommendations may need adjustment if you have heart or kidney disease. Mouth ulcers, pain or infection that interferes with eating should be treated promptly rather than accepted as unavoidable.

Gentle movement, short walks and planned rest may help fatigue, but activity should match your condition and any surgical or bone-safety restrictions. Fatigue can also reflect anaemia, infection, hormone changes, pain, poor sleep or low mood. Let the team assess a marked or persistent change. Accepting help with shopping, transport or meals can conserve energy for the activities that matter to you.

Live well

Follow-up after treatment

ACC requires close follow-up because recurrence can occur after apparently complete surgery. Imaging commonly assesses the chest, abdomen and pelvis, with frequency tailored to risk and time since treatment. Hormone tests are repeated when they were informative or symptoms suggest a change. Follow-up also monitors steroid replacement, mitotane effects and recovery. Imaging intervals may range from every three months to annually as risk and time since treatment change. Monitoring commonly continues for up to ten years and sometimes longer.

Ask for a plan extending beyond the first few visits and a named contact for new symptoms. Increasing abdominal pain, a new mass, unexplained weight change, recurrent hormone symptoms or breathlessness should be reported. A rising hormone result may be a clue, but no single test replaces the full assessment.

Living with a rare cancer can feel isolating. Specialist centres, appropriate patient organisations and genetic counselling can provide additional support. Ask the team to explain uncertain results in plain language and to coordinate appointments where possible, especially when oncology and endocrinology are both involved.

Emotional and practical support

Feeling frightened, angry, sad or uncertain is understandable. You do not have to appear positive all the time. Ask about counselling, psycho-oncology, support groups or a social worker if worry is affecting sleep, relationships or daily life. Persistent depression, panic or thoughts of self-harm need prompt professional help. Caregivers may need their own support and breaks; one person should not have to manage every appointment and household task alone.

It can help to agree who will update relatives and how much information you want shared. Children usually benefit from honest, age-appropriate explanations and reassurance about who will care for them. Ask permission before discussing private medical information with others. If concentration or memory is difficult, use reminders, a calendar and a trusted companion at important consultations.

Relationships, fertility and sexual health

Cancer and treatment can affect desire, comfort, fertility, body image and confidence. These are valid medical concerns. Raise fertility questions before treatment when possible, because some protective options need to be arranged early. The right approach depends on age, the treatment and how urgently it must start. Do not assume that absent periods or reduced fertility means pregnancy is impossible. Ask about contraception and the safe timing of pregnancy for your specific medicines.

Explain any pain, dryness, erection difficulties or changes in intimacy to the team. Appropriate treatments, rehabilitation and counselling can help. You can choose how much to involve a partner. Advice about hormone-containing treatments needs to take the cancer type and treatment into account; do not start them without discussing suitability.

Planning ahead while living well

Ask for a clear follow-up plan and know which symptoms should be reported between visits. Continue care for other conditions and discuss vaccinations with your oncology team; the timing and suitability of live vaccines can change during treatment. Avoid tobacco and ask for practical cessation support if needed. Activity, food and sleep plans should be realistic for your health rather than another source of pressure.

If treatment is long term, arrange periodic discussions about what is helping, what is difficult and what you want next. Planning for future care can protect your preferences and reduce uncertainty for family. You can discuss who should help with decisions if you become too unwell to speak for yourself, while continuing treatment and everyday life now.

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