Adrenal Gland Cancer

Adrenal gland cancer, or by its other name adrenal cancer, is a rare but important group of tumours developing in the adrenal glands located above the kidneys. The adrenal glands secrete hormones that play a part in many vital functions such as the stress response, blood pressure control, metabolism, salt balance and the production of sex hormones. For this reason, tumours developing in the adrenal glands do not only create a mass effect; at the same time they can also disrupt the hormone balance.

The great majority of adrenal cancers arise from the cortex, the outer layer of the adrenal gland, and for this reason are called adrenocortical carcinoma. More rarely, tumours developing from the medulla, the inner part of the gland, can also be seen. While some adrenal tumours do not produce hormones, some can secrete excessive hormones and lead to serious clinical pictures.

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Adrenal cancer is seen quite rarely. However, because it can follow an aggressive course, early diagnosis is important. Today, thanks to advanced imaging methods, hormone analyses, minimally invasive surgical techniques and multidisciplinary treatment approaches, the management of adrenal tumours can be carried out more successfully.

The Adrenal Glands and Their Functions

The adrenal glands, located at the upper part of both kidneys, are small structures approximately a few centimetres in size. However, because of the hormones they secrete they have very important functions in the body.

The adrenal glands consist of two main parts:

• The adrenal cortex: it produces the hormones cortisol, aldosterone and androgen.
• The adrenal medulla: it produces adrenaline and noradrenaline.

These hormones have a direct effect on:

• Blood pressure
• Blood sugar balance
• The stress response
• Salt-potassium balance
• Sexual development
• Metabolism

Adrenal Cancer Symptoms

The symptoms of adrenal gland cancer can vary according to the size of the tumour and whether or not it produces hormones. Small tumours may give no symptoms for a long time and can sometimes be detected incidentally during a CT or MRI taken for other reasons.

However, as the tumour grows or when it begins to produce hormones, certain complaints can emerge.

Symptoms Due to the Mass

• Abdominal or flank pain
• A feeling of fullness in the abdomen
• A palpable mass in the abdomen
• Loss of appetite
• Weight loss

Symptoms Due to Androgen Excess

Excessive androgen production can be marked particularly in women.

• Increased hair growth on the face and body
• Deepening of the voice
• Increased acne
• Menstrual irregularity
• Signs of early puberty in children

Symptoms Due to Oestrogen Excess

• Breast enlargement in men
• A decrease in sexual desire
• Erectile dysfunction
• Abnormal vaginal bleeding in women

Symptoms of Cortisol Excess

Some adrenal tumours produce excessive cortisol and can lead to a picture similar to Cushing's syndrome.

• Rapid weight gain
• Rounding of the face
• Fat accumulation in the abdominal region
• Stretch marks on the skin
• Muscle weakness
• Easy bruising
• High blood pressure
• High blood sugar
• Depression and mood changes

Symptoms of Aldosterone Excess

• Resistant high blood pressure
• Muscle cramps
• Muscle weakness
• Low potassium
• Excessive thirst
• Frequent urination

The Causes and Risk Factors of Adrenal Cancer

The exact cause of adrenal cancer is not always clear. However, certain genetic syndromes and hereditary diseases can increase the risk.

The conditions associated with an increased risk are as follows:

• Li-Fraumeni syndrome
• Beckwith-Wiedemann syndrome
• Carney complex
• Multiple endocrine neoplasia (MEN)
• Familial adenomatous polyposis
• Lynch syndrome

In people with these genetic diseases the risk of developing an adrenal tumour can be higher than the population average.

Types of Adrenal Cancer

Tumours developing in the adrenal glands can be benign or malignant.

Malignant Tumours

• Adrenocortical carcinoma: it develops from the adrenal cortex. It is the most frequently seen type of adrenal cancer.
• Malignant phaeochromocytoma: it arises from the adrenal medulla.
• Neuroblastoma: it is seen mostly in childhood.
• Malignant paraganglioma: it can develop from the nerve tissues around the adrenal gland.

Benign Tumours

• Adrenal adenoma
• Benign phaeochromocytoma
• Benign paraganglioma

Not every mass seen in the adrenal gland means cancer. Because of the CT and MRI examinations frequently used today, small adrenal masses detected incidentally ("adrenal incidentaloma") are quite common. However, whether these masses produce hormones and their risk of cancer must without fail be assessed.

The Diagnosis of Adrenal Cancer

In the assessment of adrenal tumours both hormone analyses and imaging methods are important.

Physical Examination and Medical History

The patient's complaints, signs of hormone excess and family history are assessed.

Blood and Urine Tests

The levels of the hormones secreted by the adrenal gland are measured.

• Cortisol
• Aldosterone
• Catecholamines
• Metanephrines
• Androgen hormones

can be examined.

Imaging Methods

To assess the size and spread of adrenal tumours:

• Computed tomography (CT)
• Magnetic resonance imaging (MRI)
• PET-CT
• Ultrasonography

can be used.

Particularly in adrenal masses over 4 cm, with irregular borders or growing rapidly, the possibility of cancer is assessed more carefully.

Laparoscopy

In some situations a laparoscopic examination can be performed for direct assessment of the inside of the abdomen.

Biopsy

A biopsy is not performed on every adrenal mass. Particularly if there is a suspicion of phaeochromocytoma, a biopsy can be risky. For this reason the decision on biopsy must be planned carefully.

The Stages of Adrenal Cancer

When staging is carried out in adrenal cancers:

• The size of the tumour
• Involvement of neighbouring organs
• Lymph node spread
• The presence of distant metastasis

are assessed.

Liver, lung and bone metastases can be seen in advanced-stage disease.

Adrenal Cancer Treatment Options

Surgery

The most important method in the treatment of adrenal cancer, and the one that can potentially provide a cure, is surgery.

According to the location and size of the tumour:

• Laparoscopic adrenalectomy
• Robotic adrenalectomy
• Open surgery

can be applied.

In recent years robotic surgical methods in particular have been used more frequently in adrenal gland operations in suitable patients. Thanks to robotic surgery, advantages such as a smaller incision, less pain and faster recovery can be provided.

Radiotherapy

Radiotherapy can be applied to reduce the risk of recurrence after surgery or with the aim of controlling complaints in metastatic disease.

Drug Treatments

In some adrenal cancers, medicines that suppress hormone production can be used.

In particular the medicine called mitotane is one of the agents used for many years in the treatment of adrenocortical carcinoma.

In some patients:

• Targeted treatments
• Immunotherapy
• Biological agents

can be considered.

Chemotherapy

In advanced-stage or metastatic adrenal cancer, chemotherapy may be necessary. The treatment plan is determined according to the biological behaviour of the tumour.

Tumour Ablation

In some patients who are not suitable for surgery or who are metastatic, tumour control can be attempted with methods such as:

• Radiofrequency ablation
• Cryotherapy
• Microwave ablation

Follow-Up After Adrenal Cancer

Because there is a risk of recurrence in adrenal cancers, regular follow-up is important.

In the follow-up process:

• Hormone tests
• CT or MRI imaging
• PET-CT
• Blood tests

can be carried out at specific intervals.

Adrenal Cancer Survival Time and Survival

Survival time in adrenal cancer can vary according to the stage of the tumour, whether or not it produces hormones, the success of the surgery and the state of metastasis.

In adrenal tumours at an early stage and able to be completely removed, survival rates are markedly higher. However, in advanced-stage disease treatment can become more complex.

Today, thanks to advanced imaging methods, modern surgical techniques and systemic treatments, more successful results are being achieved in the management of adrenal cancer compared with past years.

Frequently Asked Questions

Is every mass seen in the adrenal gland cancer?

No. Many masses seen in the adrenal gland are benign. However, hormone production and imaging characteristics must be assessed carefully.

At what age is adrenal cancer seen?

Although it can be seen at any age, it is mostly detected in the adult age group.

Does adrenal cancer have to produce hormones?

No. While some adrenal tumours produce hormones, some can be non-functional.

Can robotic surgery be used in adrenal cancer?

Yes. In suitable patients robotic adrenalectomy can be a minimally invasive option.

Can adrenal cancer be completely cured?

In tumours diagnosed at an early stage and able to be completely removed, successful treatment is possible.