Adrenocortical Carcinoma: Causes,Symptoms & Treatment

Written by Medicover Team and Medically Reviewed by Dr Neelesh Kanderi , Oncologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Adrenocortical carcinoma is a rare cancer that begins in the outer layer (cortex) of the adrenal glands, which are small glands located above the kidneys. These glands produce hormones that regulate metabolism, blood pressure, and stress response. When cancer develops in this region, it may disrupt hormone production or grow as a mass affecting nearby organs.

It originates from the adrenal cortex, the hormone-producing outer layer of the adrenal gland.
The tumour may be functional (producing excess hormones) or nonfunctional.
It can affect hormonal balance and may spread to nearby tissues or distant organs.
Because it is rare, early diagnosis can sometimes be challenging.

What Types of Adrenocortical Carcinoma are there?

Adrenocortical carcinomas are commonly categorized based on whether they produce hormones. This classification helps doctors understand symptoms and plan appropriate treatment.

Functional tumours: These tumours produce excess hormones such as cortisol, aldosterone, or androgens, leading to noticeable hormonal symptoms.
Nonfunctional tumours: These tumours do not produce hormones and are often detected later when they grow large enough to cause pressure-related symptoms.

What Are the Symptoms of Adrenocortical Carcinoma?

Adrenocortical carcinoma can produce a variety of symptoms depending on whether the tumour releases excess hormones or grows large enough to press on surrounding tissues.

What hormonal symptoms can occur in Adrenocortical Carcinoma?

Functional ACCs may lead to overproduction of hormones such as cortisol, aldosterone, and androgens. This hormonal imbalance may lead to several noticeable changes in the body.

Symptoms of Cushing's syndrome such as weight gain, high blood pressure, and glucose intolerance.
Excess androgen production leading to virilization.
Hirsutism (excess facial or body hair) in females.
Acne and menstrual irregularities such as amenorrhea.

What local and systemic symptoms may appear?

Nonfunctional tumours or larger masses may produce symptoms due to pressure on nearby organs or general effects on the body.

Abdominal pain or discomfort.
A noticeable abdominal mass.
Unexplained weight loss.
Persistent fatigue.
Occasional fever or general weakness.

What Causes Adrenocortical Carcinoma?

The precise cause of adrenocortical carcinoma is not fully understood. However, certain genetic mutations and inherited conditions are known to increase the risk of developing this cancer.

Mutations in the TP53 gene that regulates cell growth and division.
Inherited conditions such as Li-Fraumeni syndrome.
Beckwith-Wiedemann syndrome.
Multiple endocrine neoplasia type 1 (MEN1).
Possible exposure to certain industrial chemicals, although evidence is still limited.

These factors may contribute to abnormal cell growth in the adrenal cortex, eventually leading to tumour formation.

When Should You See a Doctor for Adrenocortical Carcinoma?

Early medical evaluation is important if symptoms related to hormonal imbalance or abdominal masses appear. Oncologists trained in endocrine disorders and cancer management are usually involved in diagnosis and treatment.

Persistent abdominal pain or a noticeable abdominal mass.
Unexplained weight gain or weight loss.
Symptoms of hormonal imbalance such as high blood pressure or abnormal hair growth.
Chronic fatigue or unexplained weakness.
Consult an endocrinologist, oncologist, or endocrine surgeon for proper evaluation.

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How is Adrenocortical Carcinoma Diagnosed?

Early and accurate diagnosis is essential for effective treatment. Doctors usually combine imaging tests, hormonal evaluations, and pathological examination to confirm the condition.

What imaging tests help diagnose Adrenocortical Carcinoma?

Imaging studies help evaluate the size, location, and spread of the tumour.

Computed tomography (CT) scans to visualize adrenal gland structures.
Magnetic resonance imaging (MRI) to assess tumour characteristics and possible spread.
Imaging also helps distinguish malignant tumours from benign adrenal growths.

What biochemical tests are used?

Hormonal testing helps determine whether the tumour is producing excess hormones.

Measurement of cortisol levels.
Testing for elevated aldosterone.
Assessment of androgen levels.
Hormonal assays to identify endocrine imbalances caused by the tumour.

How does histopathological analysis confirm the diagnosis?

A definitive diagnosis requires examining tumour tissue under a microscope.

Tissue samples obtained during surgery.
Biopsy in selected cases.
Microscopic evaluation to distinguish carcinoma from benign adrenal tumours such as adenomas.

What Are the Treatment Options for Adrenocortical Carcinoma?

The management of adrenocortical carcinoma typically requires a multidisciplinary approach involving surgery, medications, and sometimes radiation therapy.

Is surgery used to treat Adrenocortical Carcinoma?

Surgical removal of the tumour is the primary treatment for localized disease.

Adrenalectomy to remove the affected adrenal gland.
En bloc removal of nearby tissues if required.
The goal is complete tumour removal with clear surgical margins.

What adjuvant or neoadjuvant therapies are used?

Additional treatments may be recommended depending on the tumour stage and recurrence risk.

Mitotane therapy to destroy remaining adrenal cancer cells.
Neoadjuvant treatment to shrink the tumour before surgery.
Post-surgical therapy to reduce the risk of recurrence.

Are chemotherapy or radiotherapy used?

Advanced or metastatic cases may require systemic treatments.

Chemotherapy drugs such as etoposide, doxorubicin, and cisplatin.
Radiotherapy mainly for symptom relief or local recurrence.
Combination treatment for advanced disease management.

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What Are the Stages of Adrenocortical Carcinoma?

The staging of adrenocortical carcinoma follows the TNM (Tumor, Node, Metastasis) classification, which evaluates the size of the tumour, lymph node involvement, and distant spread.

Stage I: Tumour confined to the adrenal gland and ?5 cm in size.
Stage II: Tumour larger than 5 cm but still limited to the adrenal gland.
Stage III: Tumour spreads to nearby tissues or regional lymph nodes.
Stage IV: Cancer has spread to distant organs.

Accurate staging helps determine the most appropriate treatment approach and assess prognosis.

What is the Recovery Process After Adrenocortical Carcinoma treatment?

Recovery after treatment depends on the stage of the cancer, the treatment method used, and the patient's overall health. Continuous monitoring is important to detect recurrence or manage hormone levels.

Regular follow-up imaging to monitor recurrence.
Hormone level monitoring after adrenal gland removal.
Medications to manage hormonal balance if required.
Long-term follow-up care with oncology and endocrinology specialists.

What is the Prognosis of Adrenocortical Carcinoma?

The prognosis of adrenocortical carcinoma varies significantly depending on tumour stage, the possibility of complete surgical removal, and response to treatment.

Localized disease: Patients with stage I or II disease who undergo complete surgical removal may have a 5-year survival rate of around 60-80%.
Advanced disease: Stage III and IV cancers often have a poorer prognosis, with survival rates below 20%.

Additional prognostic indicators include the Ki-67 proliferation index, mitotic rate, and specific histopathological features.