Adrenal Gland Cancer: A Patient Guide

Adrenal gland cancers, including Adrenocortical Carcinoma (ACC) and malignant Pheochromocytoma/Paraganglioma (PPGL), are exceptionally rare and complex diseases. Because these tumors develop in the glands that produce the body’s essential hormones, they do more than just grow as masses; they can fundamentally alter your body’s chemistry. This dual nature—being both a cancer that can spread and a source of powerful hormones—means that every step of your care must be carefully sequenced to protect your safety ^[1][2].

Which Pathway Applies to Me?

• Adrenocortical Carcinoma (ACC): Often produces excess cortisol or androgens. Treatment relies heavily on specialized surgery (en-bloc resection) and frequently the medication mitotane.
• Pheochromocytoma/Paraganglioma (PPGL): Tumors that often produce adrenaline-like hormones (catecholamines). Treatment requires strict blood pressure preparation (alpha-blockade) before surgery, and the condition carries a very high rate of hereditary (genetic) risk.

The cornerstone of safe care is a “hormone-first” approach to diagnosis. Before a biopsy or surgery is even considered, doctors must use biochemical testing to rule out a pheochromocytoma. If a biopsy is performed on an unrecognized pheochromocytoma, it can trigger a sudden, life-threatening release of hormones known as a hypertensive crisis. (Note: biopsies are generally not a routine part of diagnosing a resectable adrenal tumor; they are mostly reserved for when doctors suspect metastasis from another cancer).

Once the hormone status is clear, the focus shifts to an expert review of imaging and pathology. Because these tumors are so rare, getting a precise diagnosis—including critical markers like the Weiss score and Ki-67 index—requires an expert adrenal pathologist working within a multidisciplinary team of specialists ^[3][4].

Treatment for localized adrenal cancer is primarily surgical, but it is highly specialized. The goal is an en-bloc resection, where the surgeon removes the tumor intact along with involved surrounding tissue to ensure no cancer cells are left behind. For those with a catecholamine-secreting pheochromocytoma, surgery can only happen after a period of “alpha-blockade” to stabilize blood pressure.

For those with ACC, the multidisciplinary team will evaluate the risk of recurrence to decide if “adjuvant” therapy is needed. This often involves mitotane, a medication that helps control the disease but also suppresses the body’s ability to produce cortisol. This makes long-term steroid replacement and “sick-day” education a vital part of daily life ^[5][6].

Because many adrenal cancers (especially PPGL) have a strong hereditary component, your diagnosis is often a doorway to protecting your family through genetic cascade testing and counseling. Living with adrenal cancer means committing to long-term, individualized surveillance through regular imaging and blood tests to catch any changes early. While the path is complex, being cared for at a high-volume expert center ensures that you have access to the specialized knowledge needed to manage both the cancer and your hormone health ^[7][8].

Glossary of Key Terms

• Catecholamines / Metanephrines: Adrenaline-like hormones produced by PPGLs, measured in blood or urine.
• Hounsfield Units (HU): A measurement on a CT scan that tells radiologists how dense a tumor is.
• Weiss Score / Ki-67: Pathology markers used to evaluate ACC. Ki-67 measures how fast cells are dividing.
• En-bloc Resection: Surgical removal of a tumor in one intact piece.
• R0 / R1 / R2: Margins after surgery. R0 means clear margins (no visible cancer cells left).
• Germline Mutation: An inherited genetic change that can be passed down in families.