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PubMed This is a summary of 23 peer-reviewed journal articles Updated
Endocrinology · Adrenocortical Carcinoma

Understanding Your Adrenocortical Carcinoma Diagnosis

At a Glance

Adrenocortical carcinoma is evaluated with hormone testing and specialized CT or MRI review rather than routine needle biopsy of a potentially removable primary tumor. A high-volume multidisciplinary team helps distinguish cancer from benign adrenal growths and plan safe surgery.

Receiving a diagnosis of Adrenocortical Carcinoma (ACC) can feel overwhelming, especially when you learn how rare it is. Most local doctors may never see a case in their entire career [1]. Because ACC is so uncommon—affecting only about 0.5 to 2 people per million each year—it is vital to be seen by a specialized team that understands the unique complexities of this disease [2][3].

Understanding the Adrenal Glands

Your adrenal glands are small, triangle-shaped organs sitting on top of each kidney. They are part of your endocrine system, responsible for making hormones that regulate your metabolism, blood pressure, and response to stress [4].

ACC begins in the adrenal cortex (the outer layer of the gland). These tumors are divided into two main types:

  • Functioning Tumors: These produce an excess of one or more hormones [5]. About 60–70% of ACCs are functioning [6]. Tumors that co-secrete both cortisol and androgens are particularly suggestive of ACC.
  • Non-functioning Tumors: These do not produce hormones in excess and are often found because they grow large enough to cause pain or are discovered by chance during a scan for another reason [7].

Symptoms and Hormone Excess

When a tumor is “functioning,” it floods your body with hormones, leading to specific sets of symptoms that can differ by sex and age:

  • Cortisol Excess (Cushing Syndrome): This is the most common hormone overproduced [5]. It can cause rapid weight gain (especially in the face and trunk), a “buffalo hump” on the upper back, easy bruising, and high blood pressure [4][8].
  • Androgen Excess (Virilization): If the tumor produces male-type hormones, women and children may experience rapid hair growth on the face or body (hirsutism), voice deepening, and changes in menstrual periods [9][10].
  • Estrogen Excess (Feminization): Though rare, these tumors can cause breast tissue growth (gynecomastia) in men [11][12].
  • Aldosterone Excess: This can lead to very high blood pressure and low potassium levels (hypokalemia), causing muscle weakness [12][13].

Why a Biopsy Is Rarely Recommended

If your doctor suspects an adrenal mass might be ACC, your first instinct might be to ask for a needle biopsy to “be sure.” However, in the case of a potentially resectable primary adrenal tumor, a biopsy is rarely recommended and can be dangerous for several reasons:

  1. Tumor Seeding: There is a risk that the needle could “seed” the cancer, accidentally spreading cancer cells along the needle’s path into the abdominal wall or surrounding tissue [14].
  2. Diagnostic Accuracy: Biopsies are often unreliable for distinguishing ACC from benign adenomas. Diagnostic performance varies, but a small needle sample might miss malignant cells entirely because ACC tumors are often inhomogeneous (patchy) [15][16][17].
  3. Pheochromocytoma Risk: Before any biopsy or operation is considered, doctors must biochemically rule out a pheochromocytoma—a different type of adrenal tumor that produces adrenaline. If a needle enters or a surgeon operates on an undetected pheochromocytoma, it can trigger a “hypertensive crisis,” a life-threatening spike in blood pressure [15][18].

Note: While biopsy of a primary tumor is usually avoided, biopsy of a metastatic or unresectable lesion may be appropriate when tissue confirmation will directly change your systemic treatment.

Distinguishing ACC from Benign Growths

Many people have small, harmless growths on their adrenal glands called incidentalomas or adenomas [19]. Doctors use probabilistic clues on noncontrast CT or MRI scans, alongside hormone panels, to assess the risk of malignancy:

  • Size: Most benign adenomas are small. Tumors larger than 4 cm (about 1.5 inches) are treated with more suspicion, though size alone is not diagnostic [20].
  • Density (Hounsfield Units): Radiologists measure how “dense” a tissue is on a noncontrast CT using Hounsfield Units (HU). A very low score (below 10 HU) usually suggests a lipid-rich benign adenoma [20]. A score above 20 HU, or a tumor that looks “patchy” or “ragged” (inhomogeneous), raises suspicion for ACC [21][17].
  • Washout: ACC and other concerning masses tend to hold onto the “contrast dye” used in scans longer than benign tumors do. Delayed washout is a key factor assessed by specialists, though these calculations have limitations [20][22].

No single imaging feature is a hard rule. If your imaging and hormone tests suggest ACC, the goal is careful evaluation by a multidisciplinary team to plan a complete, en-bloc surgery to ensure the cancer is removed safely [23].

Common questions in this guide

What is adrenocortical carcinoma?
Adrenocortical carcinoma is a rare cancer that begins in the outer layer of an adrenal gland. The tumor may produce excess hormones, or it may be nonfunctioning and found because it grows, causes pain, or appears unexpectedly on a scan.
What symptoms can a hormone-producing adrenal cancer cause?
Symptoms depend on which hormone the tumor produces. Excess cortisol can cause rapid weight gain, easy bruising, and high blood pressure, while excess androgen can cause new facial or body hair, voice deepening, or menstrual changes. Less commonly, excess estrogen or aldosterone can cause breast growth in men or high blood pressure with low potassium and muscle weakness.
Why is a needle biopsy usually avoided for suspected adrenocortical carcinoma?
For a potentially removable primary adrenal tumor, a needle biopsy can spread tumor cells along the needle path and may miss cancer in a patchy mass. Doctors also need to rule out pheochromocytoma first, because puncturing one can trigger a dangerous surge in blood pressure. A biopsy of cancer that has spread or cannot be removed safely may be considered when the tissue result would change treatment.
What do Hounsfield units and contrast washout mean on an adrenal scan?
Hounsfield units measure how dense an adrenal mass looks on a noncontrast CT scan. A score below 10 often supports a benign, fat-containing adenoma, while a score above 20, an uneven appearance, or delayed contrast washout can raise concern for cancer. No single scan feature proves whether a mass is cancerous, so specialists interpret the findings together with hormone tests.
Why must doctors test for pheochromocytoma before a biopsy or operation?
Pheochromocytoma is a different adrenal tumor that can release adrenaline. If it is not recognized, a needle or operation can trigger a life-threatening spike in blood pressure. Biochemical testing helps doctors identify this risk before an invasive procedure.
Why should I see a high-volume adrenal cancer team?
Adrenocortical carcinoma is rare, so a high-volume center may have more experience evaluating its hormone patterns, imaging, and surgical risks. A multidisciplinary team can include endocrine specialists, an experienced surgeon, a radiologist, and a pathologist who review the case together. This helps guide a complete and safe surgical plan when surgery is appropriate.
What does en-bloc surgery mean for adrenal cancer?
An en-bloc resection removes the tumor as one piece, with the aim of avoiding rupture of its capsule and removing all visible cancer safely. Whether this approach is appropriate depends on the tumor’s size, location, spread, and your specialist team’s assessment.

Get notified when new evidence is published on adrenal carcinoma.

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References

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This page is for informational purposes only and does not constitute medical advice. Your specialist adrenal team should interpret your hormone tests and scans and advise whether biopsy or surgery is appropriate for you.

Guide contents adrenal carcinoma
Research & Literature
02 Staging and Your Pathology Report