Anaplastic Thyroid Cancer Patient Guideline

Publication Date: March 20, 2021
Last Updated: April 13, 2022



  • The thyroid is a butterfly-shaped endocrine gland located in the lower front of your neck.
  • The thyroid gland is responsible for sending out thyroid hormones to the rest of your body.
  • Thyroid hormone helps the body use energy, stay warm and keep the brain, heart, muscles, and other organs working normally.
  • Thyroid cancer is a malignant tumor of the thyroid gland.
  • Thyroid cancer is a common type of cancer, but anaplastic thyroid cancer, or ATC, is rare and only makes up 1% or less of all thyroid cancers. ATC affects one to two people per one million per year in the US.
  • There are four types of thyroid cancers, of which ATC is the least common. ATC grows and spreads more rapidly than any other type.
  • ATC is also known as undifferentiated thyroid cancer because the cells do not in any way resemble normal thyroid cells. In contrast, the cells in other thyroid cancers are abnormal but retain some healthy thyroid cell features (they are called differentiated thyroid cells).
  • There are no known causes for ATC, however it usually occurs in individuals aged 60 or older.
  • About half of all patients with ATC have had or currently have another type of thyroid cancer.
  • While overall survival statistics are discouraging – with an average survival rate of 6 months and approximately 1 in 5 alive after 12 months – it is important to note that there are long-term survivors and new effective treatment options.



  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.




  • 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!


  • 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.

Image from Cancer Research UK



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.


  • 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.



Having trouble viewing table?
Drug Genetic alterations
dabrafenib + trametinib BRAF V600E
larotrectinib NTRK
entrectinib NTRK
pralsetinib RET
selpercatinib RET
crizotinib ALK
ceritinib ALK
alectinib ALK
pembrolizumab High PD-L1 and/or ≥10 mutations/Mb TMB



  • 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
  • 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

ThyCa: Thyroid Cancer Survivor’s Association

NCI Center for Cancer Research


ATA, American Thyroid Association; ATC, Anaplastic Thyroid Cancer; FNA, Fine Needle Aspiration; TSH, Thyroid Stimulating Hormone



Q: What causes ATC?
A: The cause of ATC is unknown, however, in some cases it can arise from other types of thyroid cancers. There are not any known associations between ATC and any lifestyle choices or behavioral factors. ATC occurs most often in people over age 60, and it rarely occurs in younger people.

Q: What are the symptoms of ATC?
A: ATC can present in several ways. Most often it presents as a lump or nodule in the neck. In some cases it may present as a neck mass with difficulty swallowing, difficulty breathing, or even hoarseness if one of the vocal cords is paralyzed by the tumor.

Q: How is ATC diagnosed?
A: Typically, a fine needle aspiration (FNA) or core biopsy (a biopsy obtained using a larger needle) is performed. Once the diagnosis is confirmed, a full assessment of your overall health is normally completed.

Q: Why wasn’t my ATC diagnosed sooner?
A: ATC is rare, extremely difficult to detect early on, and progresses rapidly. The time period between developing ATC and having clinical symptoms is very short. Many patients don’t show any signs or symptoms until the cancer has already spread to other parts of the body.

Q: What is my prognosis?
A: The prognosis for a person with ATC depends on several factors, including the patient’s age, the size of the tumor(s), and whether and where the disease has spread outside the thyroid gland at the time of diagnosis. While ATC is a very aggressive form of cancer, it is treatable in some cases. A small group of patients do quite well, and there are even long-term survivors. It is important to remember that each patient experience is unique.

Q: How is ATC treated?
A: Treatment typically consists of one or more combinations of surgery, radiation, chemotherapy and targeted therapy, along with supportive care. Unlike other thyroid cancers, ATC does not respond to radioactive iodine therapy or thyroid stimulating hormone suppression.

Q: What happens after my initial treatment?
A: Monitoring will be needed on an ongoing basis. This is to check whether the treatment was effective at slowing, halting or even reversing the cancer. Monitoring may include imaging, lab work and regular appointments with your healthcare team. It’s very important to keep these appointments, even if you believe the cancer is in remission.

Further details on this and other thyroid-related topics are available in the patient thyroid information section on the American Thyroid Association® website at

Source Citation

Bible KC, Kebebew E, Brierley J, Brito JP, Cabanillas ME, Clark TJ Jr, Di Cristofano A, Foote R, Giordano T, Kasperbauer J, Newbold K, Nikiforov YE, Randolph G, Rosenthal MS, Sawka AM, Shah M, Shaha A, Smallridge R, Wong-Clark CK. 2021 American Thyroid Association Guidelines for Management of Patients with Anaplastic Thyroid Cancer. Thyroid. 2021 Mar;31(3):337-386. doi: 10.1089/thy.2020.0944. Erratum in: Thyroid. 2021 Oct;31(10):1606-1607. PMID: 33728999; PMCID: PMC8349723.