Anaplastic thyroid cancer (ATC) is the deadliest form of thyroid malignancy, an extremely aggressive disease for which effective treatment hinges crucially on rapid diagnosis and evaluation, and clear, frank communication with the patient, according to the American Thyroid Association’s independent Anaplastic Thyroid Cancer Guidelines Taskforce’s newly-published first comprehensive guidelines for diagnosing and managing anaplastic thyroid cancer. ATC “is a rare but highly lethal form of thyroid cancer,” the taskforce authors report. “Rapid evaluation and establishment of treatment goals are imperative for optimum patient management and require a multidisciplinary team approach.”

The evidence-based guidelines, detailing 65 recommendations based on a comprehensive review of the published research literature, were published in their entirety online, in the November issue of the journal Thyroid. They are “a remarkable and comprehensive document” that emphasizes the vital importance of quick action to diagnose, evaluate, and organize a multidisciplinary team-based patient management plan for ATC, says Professor of Medicine and Pathology Bryan R. Haugen, MD, President of the American Thyroid Association and the Mary Rossick Kern & Jerome H. Kern Chair in Endocrine Neoplasms Research at the University of Colorado’s School of Medicine in Denver, CO.

“The Guidelines demand to be read now, not learned during the emergency that is anaplastic thyroid cancer,” emphasized Charles H. Emerson, MD, Editor-in-Chief of Thyroid and Professor Emeritus of Medicine at the University of Massachusetts’s School of Medicine in Worcester, MA. “The Anaplastic Thyroid Cancer Guidelines are a unique contribution to the endocrine literature.”

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The guidelines “include the diagnosis, initial evaluation, establishment of treatment goals, approaches to locoregional disease (surgery, radiotherapy, systemic therapy, supportive care during active therapy), approaches to advanced/metastatic disease, palliative care options, surveillance and long-term monitoring, and ethical issues including end of life,” the authors report.

Patients diagnosed with Stage IVA/IVB resectable disease have the best prognosis, the taskforce concluded – “particularly if a multimodal approach” including surgery, radiation, and systemic therapy, is employed.

Some Stage IVB unresectable patients also “may respond to aggressive therapy,” they noted, but patients with Stage IVC ATC should be educated about clinical trial enrollment or hospice or palliative care and encouraged to choose one of these options in lieu of standard curative-intent treatment.

Thyroid cancers are rare, representing approximately 2.5% of cancers in the United States. Nationally, less than 2% of thyroid cancers are ATC cases, though that percentage varies from 1.3% to 9.8% geographically, the taskforce points out. ATC is particularly unforgiving, with a median survival time of 5 months and a 1-year survival rate of 20%, the guidelines point out.

Because it is so rare, it is also unfamiliar to many clinicians, potentially causing delays in treatment planning, and therefore, lethality.

“Patients who present with a rapidly expanding neck mass require rapid histopathologic confirmation of the diagnosis,” the taskforce wrote. “If ATC is diagnosed, the patient’s overall clinical status and TNM stage of the tumor should be determined.”

Tissue cytology and histopathology-based diagnosis is followed by clinical assessment, lab studies and imaging, and staging, the taskforce authors explain in the guidelines. Then – and quickly – the patient’s status should be clearly disclosed to him or her, along with the risks and benefits, and probable outcomes, of therapeutic options. Treatment goals (aggressive versus supportive care) should be established after a frank and clear discussion of prognosis. In developing a patient’s treatment plan, the taskforce authors emphasize the importance of asking patients – and incorporating their answers – about their “values and preferences.”  

Patients with metastatic ATC “only rarely” respond to traditional treatments, so if a patient wishes to undergo the rigors of aggressive treatment, she or he should be encouraged to participate in a clinical trial, the taskforce recommends.

Patient autonomy and values must be respected throughout the treatment planning and implementation processes, and patients “must have decision-making capacity to consent to or make particular medical decisions,” the taskforce notes. Psychiatric or clinical ethics consultations should be requested if concerns arise about the patient’s cognitive status or decision-making capacity.

Because of the poor survival rates associated with ATC, patients should be told about palliative care options early on, and “encouraged to draft an advance directive in which they name a surrogate decision maker and list code status and other end-of-life preferences.”

“Consider, in some cases, using ‘allow natural death’ (AND) over ‘do not resuscitate (DNR), which may be better understood by patients and families as an order that limits inappropriate aggressive care,” advises the taskforce.

Readers, we want to hear from you!

  • Will you incorporate these task force recommendations into your practice for diagnosing and managing your patients with anaplastic thyroid cancer?