Adrenal cancer is rare. Exceptional patient care and innovative treatment for adrenal cancer shouldn't be. 

As part of the Rogel Cancer Center, our Endocrine Oncology Program brings experts in related fields together to provide coordinated, exceptional patient care to those diagnosed with adrenocortical carcinoma (adrenal cancer). Patients benefit from the experience and expertise of many physicians, not just one.

Our multidisciplinary tumor board meets on a regular basis to discuss every patient who comes through our doors. A room full of specialists sit together to talk through the best treatment options, including clinical trials, for each individual. Patients get the benefit of several expert opinions.

Services include:

• Multidisciplinary team with one of the most active adrenal cancer research programs in the world
• See more than 200 adrenal patients per year
• Recognition as an international center of excellence for treatment of adrenal cancer
• Availability of state-of-the-art genetic counseling
• Advanced diagnostics
  • Diagnosis and therapy of benign and malignant adrenal tumors, pheochromocytoma, paraganglioma and neuroendocrine tumors
  • Diagnosis and therapy of hormone excess syndromes due to adrenal adenoma, adrenal cancer, pherochromocytoma, paraganglioma or neuroendocrine tumors
  • Dedicated radiology and nuclear medicine imaging
• Specialized therapies
  • Stereotactic Body Radiation Therapy for adrenal metastasis
  • Dedicated adrenal and endocrine surgeons
• Innovative care models
  • Remote Second Opinion Program for adrenal cancer
  • Pathology Consultation
  • Therapy and surveillance of endocrine issues in cancer survivors

We offer diagnosis and treatment for those with adrenal cancer.  If you have been diagnosed, or suspect you have adrenal cancer, patients, please call 734-647-8902.

Healthcare professionals, please contact our M-LINE service: 800-962-3555.  Our Endocrine Oncology Program also offers a consultation with one of our Pathology experts.  Please visit the M-Labs Consult page for more information.

Remote Second Opinion

The U-M Health's Remote Second Opinion is available to those with adrenal cancer or are suspected to have adrenal cancer. With our remote second opinion, you can obtain a medical consultation regarding your diagnosis and treatment plan by allowing our physicians to review your medical records and remotely interact with you and your local doctor. So, even if you can’t travel to Michigan for an appointment, you can still get an opinion from our medical specialists.

Requesting a Remote Second Opinion

Due to specific state laws and medical practice regulations in the following states, we are unable to provide a remote second opinion for residents in the states of California, Wyoming, North Dakota, Iowa, Louisiana, Tennessee, South Carolina, New York, and Maine. Residents in these states who wish to seek a medical opinion from the University of Michigan are encouraged to call 800-865-1125.

Everyone else, please call 734-647-8902 to request a remote second opinion.

Most adrenocortical cancers are found after they have been growing for years. Fewer than 30% of adrenocortical cancers are confined to the adrenal gland at the time of diagnosis.

The most common symptom reported by patients with adrenocortical cancer is pain in the back or side (called the flank).

Unfortunately, this type of pain is common and does not directly suggest a disease of the adrenal cortex. In adrenocortical cancer, these symptoms usually are due to pressure caused by the tumor as it compresses organs, nerves and other structures around the adrenal gland. Some patients describe feeling full with no appetite because of pressure on the stomach and other abdominal organs.

Adrenal cancers act in one of two ways:

They secrete hormones and are called functional tumors, or they do not and are called non-functional tumors. Some adrenal cancers, and even some benign adenomas, are functional and secrete excessive amounts of hormones. Approximately 60% of patients will experience symptoms because of these high levels of hormones in the blood. These symptoms include:

• Weight gain and fluid retention
• Early puberty in children
• High blood pressure (more common in adenomas and pheochromocytomas)
• Excess facial and body hair growth in women
• Excess breast tissue in men
• Easy bruising
• Muscle weakness
• Diabetes
• Osteoporosis
• Mood changes

Diagnosis and Staging

Most adrenal cancers have grown for a long time before they are diagnosed. They are often found after the patient seeks treatment for the symptoms caused by the cancer. However, some tumors are found when a physician orders imaging testing for another condition.

Patients with symptoms suspicious for adrenal cancer will undergo tests to determine the cause of these symptoms. The first step is a thorough medical history and physical exam to determine the extent of symptoms and their possible causes.

Further evaluation may include the following:

• Blood and urine tests will be done to evaluate levels of adrenal hormones. Remember that adenomas (benign tumors) may produce high levels of adrenal hormones. Therefore, high hormone levels do not always indicate an adrenal cancer. Some blood and urine tests may be done after you are given a steroid such as dexamethasone. The blood and urine tests after the medication will measure your bodies’ response to the steroid, indicating the presence of a hormone secreting tumor or other problem with the adrenal gland.
• Computed Tomography (CT)
  The CT scan can show small tumors as well as important blood vessels that the tumor might be growing into or around. A CT scan can look at surrounding organs for spread (metastasis) of the cancer into lymph nodes or other organs in the abdomen.
  
  A CT scan takes longer than a regular x-ray. You will lie still on a table while it is being done. Usually, you will need an IV placed for a dye injection just before the scan. You may also be asked to drink a contrast solution before the exam. This helps outline the intestine so it is not mistaken for tumors.
• Magnetic Resonance Imaging (MRI)
  The MRI scan uses magnets to make a picture of the inside of the body. MRIs produce very sharp, precise pictures of the area and can be helpful in distinguishing an adenoma from a cancer.
• Ultrasound of the abdomen:
  An ultrasound can identify a tumor or mass by using sound waves to make a picture of the inside of the abdomen. This is the fastest and cheapest test used; however it is the least accurate. It can be difficult to tell the difference between an adenoma and cancer with an ultrasound. An ultrasound may be used to guide the biopsy procedure as described above under CT scan.
• Positron emission tomography (PET)
  A PET scan involves the injection of radioactive sugar into a vein followed by a body scan that looks for areas taking up the sugar. Cancer cells take up sugar much faster than normal tissue, so cancerous areas can be located with this test. Research studies have shown the usefulness of PET scanning in identifying adrenal tumors. However, studies are still in progress to look at ways PET scanning can be used to tell the difference between adrenal cancers and benign adenomas and between primary adrenal cancers from metastatic tumors that started in other organs. One of these studies uses a substance called metomidate as the radioactive substance. Some studies suggest it can be helpful in distinguishing primary tumors from metastatic ones.
• Angiography
  The adrenal gland is situated near a number of important veins and arteries. Some studies may be necessary to evaluate the involvement of these blood vessels, such as whether the tumor is compressing or blocking them. These studies are referred to as selective angiography and adrenal venography.

Staging Adrenal Cancer

In general, patients with adrenocortical cancer are divided into 4 groups or stages of adrenal cancer.

The 4 stages of adrenal cancer are:

• Stage 1: The cancer is smaller than 5 centimeters (2 inches) and has NOT spread outside of the adrenal gland.
• Stage 2: The cancer is larger than 5 centimeters and has NOT spread outside of the adrenal gland.
• Stage 3: The cancer has spread into the fat surrounding the adrenal gland or has spread to the lymph nodes or other organs near the adrenal gland.
• Stage 4: The cancer has spread to adjacent organs / other parts of the body.

Because adrenal cancer is so uncommon, there are several staging systems that may be used. This system of staging is the most recently developed and implemented by the World Health Organization (WHO).

If an adrenal nodule is found, the team wants as much information as possible to develop an individualized treatment plan. We will evaluate the nodule by obtaining a CT scan or MRI done specifically to look at the adrenal glands. Our team prefers to investigate adrenal abnormalities using CT first, and MRI if a different type of study is needed. Patients also have blood drawn and submit urine samples (drawn over 24 hours) to test for excess amounts of adrenal hormones. Other laboratory studies may be obtained depending on individual patient findings.

In some instances, patients are referred to one of U-M Health's genetic counselors for potential further genetic testing. For more information, please visit the Cancer Genetics Clinic.

Surgery

Surgery is the treatment of choice if possible. In many cases, patients present after tumor has spread to other organs in the body or the tumor has invaded structures that cannot be removed and the tumor is considered inoperable. If patients are able to undergo surgery, it is extremely important that your surgery is performed by a surgeon experienced in the treatment of adrenal cancer (most surgeons see one adrenal cancer patient in a lifetime), as the covering of the tumor must not be penetrated during surgery and a wide margin of normal tissue around the tumor should be included to ensure complete resection and minimize the chance for local recurrence. At U-M Health, our endocrine surgeons often operate on patients with advanced adrenal cancer, including patients who have been told by other health care teams that their tumor is unresectable. Appropriate preoperative evaluation and planning is of the utmost importance in adrenal cancer patients to assure optimal outcome. Adrenal cancer is a very rare cancer different from many other types of cancers. It must be carefully and completely removed. 

Based on experience and research done at the Rogel Cancer Center, we generally do not recommend removing adrenal cancer (or any mass/nodule suspected of potentially being malignant) using a laparoscopic approach as it often leads to early recurrence in the abdominal cavity due to microscopic tumor spillage. Often, the entire tumor is not resected when the procedure is performed laparoscopically, and the "margins" (edges of the tissue removed) are positive for tumor cells. An open approach (through a larger incision along the rib cage or along the midline of the abdomen) allows the surgeon to remove a rim of normal (benign) tissue around the tumor more easily to help decrease rates of local recurrence and spread within the abdomen and has been found to improve survival in patients with adrenal cancers which have not already spread outside the adrenal gland.

Unfortunately, even with pathologically documented complete resection of ACC, up to two-thirds of patients have recurrence of tumor at a locoregional or distant site, or both. Individual markers of tumor biology such as histologic grade (calculated by the number of cells seen to be multiplying within the tumor), length of time without evidence of measurable disease, and slow progression of tumor can be used as a guide when considering re-operation. If a recurrence is found where the original tumor previously was and there is no evidence of tumor elsewhere in the body (metastases), re-operation is associated with increased length of survival when compared to patients treated with mitotane and/or chemotherapy alone.

Treatment After Adrenal Cancer Surgery

After surgery, our team reviews the surgeon’s operative report and the pathology report. Important aspects of the pathology report include completeness of resection and margin status (positive or negative), capsular or vascular invasion, tumor grade (a measurement of how fast the tumor cells are multiplying), evidence of tumor within lymph nodes, or invasion of nearby structures. Decisions regarding further treatment are made. Treatment may include one or more of the following:

• external beam radiation therapy
• mitotane
• chemotherapy

If mitotane is started, labs to check mitotane levels, thyroid function and liver function will be checked frequently and replacement steroids will also be started. University of Michigan physicians are experienced in the administration of standard chemotherapy regimens for adrenal cancer patients and are also involved in finding new therapies. Often, physicians will discuss participation in ongoing studies for promising new medications to treat adrenal cancer.

Follow-up Care

Clinic visits, imaging studies, and blood work are generally obtained every three months to assess disease status and look for evidence of recurrence or disease progression. U-M Health physicians will work closely with a patient's primary care physician and other physicians (oncologists) near their home to ensure they are receiving optimal care. For those with persistent or recurrent disease, U-M Health physicians consider all treatment options and stay abreast of new clinical trials offered around the world.