Adrenal Cancer

Adrenal cancer is a rare malignancy that develops in the adrenal glands, small hormone-producing organs located above each kidney. This cancer can disrupt hormone production and spread to other parts of the body if not detected and treated early.

Overview

Adrenal cancer is an uncommon malignancy that originates in the adrenal glands, which are triangular-shaped organs situated on top of each kidney. These glands are part of the endocrine system and produce essential hormones including cortisol, aldosterone, and androgens. Adrenal cancer occurs when cells in the adrenal glands mutate and grow uncontrollably, forming tumors that can be either functional (hormone-producing) or non-functional.

The most common type of adrenal cancer is adrenocortical carcinoma (ACC), which develops in the outer layer (cortex) of the adrenal gland. Less commonly, cancers can develop in the inner part (medulla) of the gland, such as pheochromocytomas, though most pheochromocytomas are benign. Adrenal cancer affects approximately 1-2 people per million annually, making it one of the rarer forms of cancer. It can occur at any age but shows two peak incidences: in children under 5 years old and in adults between 40-50 years of age.

The prognosis for adrenal cancer varies significantly depending on the stage at diagnosis, tumor size, and whether the cancer has spread to other organs. Early-stage tumors that are completely removed surgically have the best outcomes, while advanced cases that have metastasized present greater treatment challenges. Understanding the signs and symptoms of adrenal cancer is crucial for early detection and improved treatment outcomes.

Symptoms

Adrenal cancer symptoms can vary widely depending on whether the tumor produces excess hormones (functional) or not (non-functional), as well as the size and location of the tumor. Many patients may not experience symptoms in the early stages, which can delay diagnosis.

General Symptoms

  • Dizziness - often related to hormonal imbalances or blood pressure changes
  • Diminished hearing - can occur due to metabolic changes
  • Abdominal pain or fullness
  • Unexplained weight loss
  • Loss of appetite
  • Nausea and vomiting
  • Back pain
  • Fever
  • A feeling of fullness in the abdomen

Hormone-Related Symptoms

When adrenal tumors produce excess hormones, they can cause specific syndromes:

Excess Cortisol (Cushing's Syndrome)

  • Weight gain, particularly in the face and upper back
  • Purple stretch marks
  • Easy bruising
  • Muscle weakness
  • High blood pressure
  • High blood sugar
  • Mood changes and depression
  • Osteoporosis

Excess Aldosterone (Conn's Syndrome)

  • High blood pressure
  • Low potassium levels
  • Muscle cramps and weakness
  • Frequent urination
  • Excessive thirst

Excess Sex Hormones

  • In women: Deepening voice, excessive facial and body hair, irregular periods
  • In men: Breast enlargement, decreased sex drive, impotence
  • In children: Early puberty, rapid growth followed by short stature

Causes

The exact causes of adrenal cancer are not fully understood, but researchers have identified several factors that may contribute to its development. Like other cancers, adrenal cancer results from genetic mutations that cause cells to grow and divide uncontrollably.

Genetic Mutations

Adrenal cancer develops when cells in the adrenal gland undergo genetic mutations. These mutations can be:

  • Sporadic: Random mutations that occur during a person's lifetime, accounting for most cases
  • Inherited: Genetic mutations passed down through families, responsible for a smaller percentage of cases

Hereditary Syndromes

Several inherited genetic syndromes increase the risk of developing adrenal cancer:

  • Li-Fraumeni syndrome: Caused by mutations in the TP53 gene
  • Beckwith-Wiedemann syndrome: Associated with overgrowth and increased cancer risk
  • Multiple endocrine neoplasia type 1 (MEN1): Affects multiple endocrine glands
  • Lynch syndrome: Primarily associated with colorectal cancer but increases adrenal cancer risk
  • Familial adenomatous polyposis (FAP): Causes numerous colon polyps and increases various cancer risks

Environmental Factors

While less clearly established than genetic factors, some environmental exposures may contribute:

  • Previous radiation exposure, particularly to the abdomen
  • Certain chemical exposures, though specific chemicals haven't been definitively linked
  • Some medications or hormonal treatments (under investigation)

Risk Factors

Several factors may increase the likelihood of developing adrenal cancer, though having risk factors doesn't mean cancer will definitely develop:

Genetic Risk Factors

  • Hereditary syndromes: Li-Fraumeni, Beckwith-Wiedemann, MEN1, Lynch syndrome
  • Family history: Having relatives with adrenal or other endocrine cancers
  • Genetic mutations: Specific gene mutations like TP53, CTNNB1, or IGF2

Demographic Factors

  • Age: Two peak incidences - children under 5 and adults aged 40-50
  • Gender: Slightly more common in women than men
  • Geographic location: Higher incidence in certain regions, possibly due to genetic factors

Medical History

  • Previous cancers: History of other cancers may increase risk
  • Benign adrenal tumors: While most don't become cancerous, they require monitoring
  • Hormone disorders: Certain endocrine conditions may be associated with increased risk

Diagnosis

Diagnosing adrenal cancer requires a comprehensive approach combining imaging studies, laboratory tests, and sometimes biopsy. Early and accurate diagnosis is crucial for determining the most appropriate treatment plan.

Imaging Studies

  • CT scan: The primary imaging tool for detecting adrenal masses and assessing size
  • MRI: Provides detailed images and helps distinguish benign from malignant tumors
  • PET scan: Useful for detecting metastases and staging the cancer
  • Chest X-ray or CT: To check for lung metastases
  • Bone scan: If bone metastases are suspected

Laboratory Tests

Blood and urine tests to measure hormone levels:

  • 24-hour urine collection: Measures cortisol, catecholamines, and metanephrines
  • Blood tests: Cortisol, ACTH, aldosterone, renin, DHEA-S, testosterone, estrogen
  • Dexamethasone suppression test: Evaluates cortisol production
  • Electrolyte panel: Checks for hormonal effects on sodium and potassium

Biopsy

Biopsy is not always performed for adrenal tumors due to risk of spreading cancer cells. When necessary:

  • Fine-needle aspiration: Using CT or ultrasound guidance
  • Core needle biopsy: For larger tissue samples
  • Surgical biopsy: During tumor removal surgery

Staging

Once diagnosed, adrenal cancer is staged to determine extent:

  • Stage I: Tumor ≤5 cm, confined to adrenal gland
  • Stage II: Tumor >5 cm, confined to adrenal gland
  • Stage III: Tumor has spread to nearby tissues or lymph nodes
  • Stage IV: Cancer has spread to distant organs

Treatment Options

Treatment for adrenal cancer depends on the stage of the disease, the patient's overall health, and whether the tumor is functional. A multidisciplinary approach involving surgeons, oncologists, and endocrinologists typically provides the best outcomes.

Surgery

Surgery is the primary treatment for localized adrenal cancer:

  • Adrenalectomy: Complete removal of the affected adrenal gland
  • En bloc resection: Removal of tumor with surrounding tissues if invaded
  • Lymph node dissection: Removal of nearby lymph nodes
  • Cytoreductive surgery: Removing as much tumor as possible in advanced cases
  • Minimally invasive surgery: Laparoscopic approach for small, contained tumors

Medical Therapy

  • Mitotane: The primary drug for adrenal cancer, both as treatment and adjuvant therapy
  • Chemotherapy: Combinations like EDP-M (etoposide, doxorubicin, cisplatin plus mitotane)
  • Targeted therapy: Drugs targeting specific genetic mutations
  • Immunotherapy: Emerging treatments using checkpoint inhibitors
  • Hormone blockers: To control symptoms from excess hormone production

Radiation Therapy

While adrenal cancer is relatively resistant to radiation, it may be used for:

  • Palliative treatment to relieve pain from metastases
  • Adjuvant therapy after surgery in high-risk cases
  • Treatment of inoperable tumors
  • Control of local recurrence

Supportive Care

  • Hormone replacement: If both adrenal glands are removed
  • Management of hormone excess symptoms: Blood pressure control, diabetes management
  • Pain management: For advanced disease
  • Nutritional support: To maintain strength during treatment
  • Psychological support: Counseling for patients and families

Clinical Trials

Patients may be eligible for clinical trials testing:

  • New drug combinations
  • Novel targeted therapies
  • Immunotherapy approaches
  • Combination treatments

Prevention

Since most cases of adrenal cancer occur sporadically without known causes, prevention strategies are limited. However, certain measures can help with early detection and risk reduction:

Genetic Counseling and Testing

  • Individuals with family history should consider genetic counseling
  • Testing for hereditary cancer syndromes in at-risk families
  • Regular screening for those with identified genetic mutations
  • Family planning counseling for carriers of genetic mutations

Surveillance for High-Risk Individuals

  • Regular imaging (CT or MRI) for those with hereditary syndromes
  • Biochemical screening with hormone level tests
  • Earlier and more frequent screening for children in affected families
  • Monitoring of benign adrenal tumors for changes

Lifestyle Factors

While not specifically proven for adrenal cancer, general cancer prevention strategies include:

  • Maintaining a healthy weight
  • Regular physical activity
  • Avoiding tobacco use
  • Limiting alcohol consumption
  • Protecting against unnecessary radiation exposure

When to See a Doctor

Early detection of adrenal cancer can significantly improve treatment outcomes. Seek medical attention if you experience:

Immediate Medical Attention

  • Severe abdominal or back pain
  • Rapid, unexplained weight loss
  • Severe high blood pressure not responding to treatment
  • Signs of severe hormonal imbalance
  • Persistent fever without obvious cause

Schedule an Appointment For

  • Persistent dizziness or lightheadedness
  • Unexplained diminished hearing
  • Unusual weight gain or distribution
  • New or worsening high blood pressure
  • Unexplained muscle weakness
  • Changes in sexual characteristics
  • Persistent abdominal fullness or discomfort

Frequently Asked Questions

Is adrenal cancer hereditary?

While most adrenal cancers occur sporadically, about 10-15% are associated with hereditary syndromes. Genetic counseling is recommended for patients with family history of adrenal or related cancers.

What is the survival rate for adrenal cancer?

Survival rates vary significantly by stage. Five-year survival rates range from 60-80% for stage I-II disease to 10-20% for stage IV. Early detection and complete surgical removal offer the best prognosis.

Can adrenal cancer come back after treatment?

Yes, adrenal cancer can recur, especially in advanced stages. Regular follow-up with imaging and hormone tests is essential. Recurrence is most common within the first two years after treatment.

What happens if both adrenal glands are removed?

If both adrenal glands are removed, lifelong hormone replacement therapy is necessary. Patients must take corticosteroids and mineralocorticoids daily and learn to adjust doses during illness or stress.

How is adrenal cancer different from metastases to the adrenal gland?

Primary adrenal cancer originates in the adrenal gland, while metastases are cancers that spread from other organs. Metastases to the adrenal glands are actually more common than primary adrenal cancer.