Anaplastic Thyroid Cancer Patient Guideline

1. Rapid and definitive diagnosis
   The sooner ATC can be caught and treated, the better your chances are for long-term survival.
2. Coordinate early care with a multidisciplinary healthcare team
   This should include endocrinologists, surgeons, radiation oncologists, medical oncologists and providers specializing in supportive care.
3. Identify the extent of the disease, including size of tumor(s), location(s) and any genetic mutations
   This is important, because it will help determine the best course of action for treating your ATC.
4. Start counseling to establish goals of care
   Counseling and setting realistic goals is an important step in your path to finding the best management option. Counseling works best when it also includes your family, friends and any other loved ones. This will include a listing of risks and benefits for each decision that needs to be made during your care.
5. Evaluate options for surgery
   If you are a candidate for surgery, your healthcare team may be able to remove the tumor(s). This is your best chance for long-term survival.
6. Decisive decision making on all available treatment option(s)
   Because ATC is so aggressive, many patients will require radiation, chemotherapy and/or targeted therapy. These therapies may also come with side effects, so you and your healthcare team will discuss which option(s) are best for your unique situation.
7. Keep an open dialogue about end of life preparations
   While ATC is curable, it is an extremely aggressive disease, and it can spread quickly. You shouldn’t postpone end of life discussions with friends, family and loved ones. You should decide who should make medical decisions on your behalf if you are too ill to do so.

Symptoms

• ATC can start as a neck mass, or bump in the throat area.
• The tumor growing on the thyroid can make your voice hoarse by invading the nerve that controls your vocal cords.
• The tumor can also make it difficult to breathe by blocking your windpipe.
• Difficulty swallowing, frequent coughing and coughing up blood, and loud breathing are other potential symptoms.
• Sometimes people can have ATC for a while and not notice it because the tumor remains small.

Evaluation and Diagnosis

• Evaluating a patient with ATC usually consists of one or more of the following:
  • Laboratory tests (e.g. blood tests)
  • Imaging (e.g. PET/CT scans or MRIs)
  • Examinations/Procedures (e.g. laryngoscopy)
  • Biopsies (e.g. ultrasound with fine needle aspiration, or FNA)
• Diagnosis of ATC would be based on biopsy (or other tissue assessment – i.e. core biopsy, excisional biopsy, surgical pathology)
• In addition to confirming a diagnosis of ATC, your healthcare providers will assess the tumor to look for genetic mutations that can help determine the best treatment option(s).
• It is important to complete all tests and assessments as quickly as possible. Do not delay!

Staging

• All patients with Anaplastic Thyroid Cancer are diagnosed as Stage IV due to the aggressive nature of this tumor.
• There are three sub-stages:
  • Stage IVA: Anaplastic thyroid cancer is present only in the thyroid
  • Stage IVB: Anaplastic thyroid cancer is present in the thyroid and in the neck, but not in other parts of the body
  • Stage IVC: Anaplastic thyroid cancer is present in the thyroid as well as other parts of the body, such as the bones, lungs or brain
• On average, when patients are diagnosed with ATC, about 10% have Stage IVA, 40% have stage IVB, and 50% have Stage IVC.
• The sub-staging is important, as it will help you and your healthcare team decide on the best treatment options for you.

Overview and Informed Consent

• Anaplastic thyroid cancer is difficult to treat because it can grow and spread rapidly in the neck and to other parts of the body.
• Because ATC grows and spreads quickly, it is urgent that you come up with a treatment plan as soon as possible. The sooner a treatment plan is started, the better the outcome.
• Informed consent is an important part of the treatment process. It is critical that you understand, appreciate, rationalize and express your decisions with your healthcare team, as well as your family and loved ones.
• When making decisions about treatment you should weigh up your own priorities and decide what treatment feels right for you based on multiple factors including:
  • What treatment options are available to you to consider?
  • How much benefit is expected from the treatment? e.g., cure or slowing spread
  • What is the potential for side effects? How common and how severe?
  • How is your health otherwise? Do you have any health condition which will affect how you respond or react to the treatment?
• You need to decide if an aggressive treatment plan to try to cure or slow disease progression is right for you.
• Every patient and scenario is different, and there is no right or wrong answer – only what is right for you.

Surgery

• The only opportunity to completely cure ATC is with the successful removal of all tumor(s) during surgery. For this reason, if you are a candidate, surgery is usually the best initial course of action.
• Not all patients are candidates for surgery, such as those with Stage IVC disease or those with other conditions that may make surgery too risky.
• Surgery will involve removing the thyroid. This surgery is called a thyroidectomy.
• Nearby lymph nodes may also be removed during surgery if they are known or suspected to contain cancer.
• If surgery is not an option, do not lose hope. Some patients start with other treatment options, and receive surgery later on once the tumors are reduced in size.

Radiation Therapy

• If you are not a candidate for surgery, if your surgery failed to remove all of the tumors, and/or if your tumors do not contain genetic mutations, radiation therapy is often the next step.
• Radiation therapy may be offered by itself, or alongside chemotherapy.
• External beam radiation directs precisely focused X-rays to areas that need to be treated – often the tumor itself or cancer that has spread to bones or other organs. The procedure aims to kill or slow the cancer without injuring the healthy nearby muscle and tissue.
• The goal of both radiation therapy and systemic therapy is to halt or reverse tumor growth to extend overall survival time, and in the scenario for those with Stage IVB, allow for surgery later to remove the tumor(s).

Systemic Therapy

• Systemic therapy includes both chemotherapy and targeted therapy
• For those wishing to undertake an aggressive form of treatment, chemotherapy is often begun immediately while awaiting results of genetic mutation tests for any tumors, and may also be used alongside radiation therapy.
• New medications called targeted therapies are available when genetic mutations are present. The most common genetic mutations, for which drugs are available, are BRAF V600E, NTRK, ALK and RET.
• Systemic therapy can often come with side effects, and during treatment you may need additional supportive care and medications for nausea and vomiting, pain, bone density loss, and more. This is why it’s important to weigh the pros and cons when deciding on treatment options, especially those that are most aggressive.

• In most cases, supportive care is also given to manage symptoms of the cancer and side effects of the treatment.
• Supportive care may include: clearing the airway via tracheostomy and stenting, placing a feeding tube, giving pain medication, nutritional support and additional approaches to make you as comfortable as possible.
• It’s important to remember that supportive or palliative care is given in addition to treating the cancer and does not necessarily mean the cancer treatment will not be effective.
• Some patients may also qualify for ongoing clinical trials. Clinical trials are strongly recommended when available. To see available trials in your area visit Thyroid.org/clinical-trials
• Advances are being made every day in the treatment of advanced thyroid cancers including undifferentiated and anaplastic thyroid cancers.
• The treatment is challenging but it is important to not give up hope when first diagnosed.

ATA Alliance for Thyroid Patient Education
Thyroid.org

ThyCa: Thyroid Cancer Survivor’s Association
ThyCa.org

NCI Center for Cancer Research
Cancer.gov

Abbreviations