Cancer in the adrenal gland is rare.

The most common cancer is a metastasis from another cancer that spread to the adrenal gland. Much less common is Adrenal Cortical Carcinoma (ACC), a rare, usually aggressive cancer that is diagnosed in 1-2% of all adrenal masses. It arises from the cortex, or outer layer of the gland and most commonly occurs in the very young (< 10 years of age) or older (>50/60 years of age) individuals.

Symptoms

Symptoms of Adrenal Cancer and Metastases

If the ACC is producing adrenal hormones, then patients can experience various symptoms relating to the particular hormone, most commonly excess cortisol, and less commonly aldosterone or sex hormones.

Occasionally, large tumors can also produce back or abdominal pain because of impingement on the diaphragm or other abdominal organs.

Signs

  • High blood pressure
  • Diabetes
  • Moon facies (round facial shape)
  • Buffalo hump (mound of fat at the back of the neck)
  • Stretch marks
  • Abnormal hair growth

Symptoms

  • Easy bruising
  • Fatigue
  • Weight gain (particularly around the mid-section)
  • Leg swelling
  • Mood swings
  • Irregular periods
  • Muscle weakness
Risk Factors

Risk Factors

  • The majority of ACCs are sporadic and without risk factors. There are a few rare genetic syndromes that are associated with ACC, including Li-Fraumeni, Lynch, and Beckwith-Wiedemann Syndromes.
  • The most common cancers that metastasize to the adrenal gland are lung, breast, kidney, gastrointestinal, melanoma, and lymphoma.
Diagnostics

Diagnostic Tests for Adrenal Cancer and Metastases

  • The diagnostic workup of adrenal cancer consists of blood tests, sometimes urine tests, and imaging which determine if the adrenal mass is producing hormones and the extent of the tumor spread.
  • Definitive diagnosis is only made on final pathology. However, certain characteristics of the tumor on imaging studies make it highly suspicious for ACC.
Treatment

Treatment of Adrenal Cancer

  • If surgically resectable: open adrenalectomy and en-bloc removal of the tumor, the surrounding tissues, any invading structures and the regional lymph nodes.
  • Adjuvant therapy with mitotane and possibly chemotherapy is usually recommended.
  • Unresectable tumors: mitotane, chemotherapy, and clinical trials with newer biologic agents

Adrenal Cancer (ACC) Prognosis

  • Adult adrenal cortical cancer is an aggressive tumor that is not usually found until it has advanced to a later stage and therefore has a poor prognosis.
  • Prognosis for smaller tumors that are confined to the adrenal gland and removed early can have a five year survival of up to 65%.
  • Since ACC is an aggressive tumor, it can definitely come back either locally (in the same location) or in a distant location as metastatic disease.

  • Completeness of surgical resection, size, stage at diagnosis, and tumor biology are the most important factors for recurrence.

  • You will have lifelong follow-up with an oncologist, surgeon, and endocrinologist.
  • Monitoring will consist of periodic imaging and blood tests to monitor for recurrence and/or disease progression.