May 27, 2015
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ATA releases first guidelines for pediatric thyroid nodules, cancer

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An American Thyroid Association task force recently released guidelines for treating children with thyroid nodules and thyroid cancer that were published in Thyroid.

“These guidelines are important because the previous adult guidelines sort of suggested that the care of pediatric patients should be similar to adults,” Andrew J. Bauer, MD, of The Children’s Hospital of Philadelphia, told Endocrine Today. “The problem is that even though the disease has the same name, the molecular biology and the clinical behavior of the disease are different. The goal of the guidelines is to try to maintain that excellent outcome but to also raise awareness about these differences and to create recommendations that help people make the right decisions to reduce short- and long-term complications.”

Andrew Bauer

Andrew J. Bauer

Compiled by the Task Force on Pediatric Thyroid Cancer, the guidelines are intended for children aged 18 years or younger, but the transition to adult care can extend up to 21 years. Until the transition is completed, clinicians may continue to manage the child.

“These ATA guidelines fill an important gap and, for the first time, provide structured recommendations for the management of thyroid nodules and thyroid cancer in children,” Peter A. Kopp, MD, editor-in-chief of Thyroid and associate professor of medicine at Northwestern University Feinberg School of Medicine, said in a press release. “The guidelines specifically address the important differences in the biological behavior and management of these entities in children, and provide a much needed overview of the currently existing evidence.”

Robert C. Smallridge, MD, president of the ATA, said children are more likely to have malignancies, whereas adults are not, and the histopathology, molecular profile and clinical behaviors of differentiated thyroid cancer exhibit substantial differences.

“These first ATA guidelines highlight these distinguishing features for the clinician faced with caring for a patient with this uncommon thyroid tumor,” Smallridge said. “The task force is to be commended for their comprehensive review and balanced recommendations.”

The guidelines cover an array of topics, including the use of ultrasound and fine-needle aspiration (FNA) for analysis of cells to evaluate and manage benign nodules, assessing, treating and monitoring pediatric patients with differentiated thyroid cancer (DTC), preoperative staging, surgical management and radioactive iodine therapy.

Endocrine Today compiled some of the key recommendations put forward by the task force. To see a complete list of the recommendations, please visit: http://online.liebertpub.com/doi/pdfplus/10.1089/thy.2014.0460.

Diagnostics

The task force recommends FNA biopsy for all pediatric patients presenting with suspicious thyroid nodules.

“If you look at the risk of malignancy for pediatrics compared with adults, for the nodule, at face value, the difference is that about 5% to 10% of the nodules in adults are likely to be malignant, and in pediatrics patients it’s estimated to be about 20% to 25%,” Bauer said. “People say, ‘Well, if there’s a 25% risk, what’s the sense of doing a biopsy? We should take the patient to the operating room.’ But the problem with that is there’s still a 75% chance it’s going to be benign.”

Bauer said proper evaluation of the ultrasound before deciding whether surgery is appropriate, along with the FNA, would help identify those patients who require surgery.

“The biopsy is really important,” he said. “Providers shouldn’t just assume that a nodule with a 25% risk of malignancy should go to surgery. No other patient with cancer would be taken to surgery without a definitive diagnosis. It shouldn’t happen in thyroid cancer, especially when the odds of being benign are so high and in the evaluation of a cancer that has such a low disease-specific mortality.”

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Preoperative staging

To optimize surgical outcomes and medical therapy, preoperative evaluation of a child with newly diagnosed pituitary thyroid cancer (PTC) is needed. The task force recommends comprehensive neck ultrasound as well as FNA of suspicious lateral neck lymph nodes, and CT or MRI with contrast for large or fixed thyroid masses, vocal cord paralysis and bulky metastatic lymphadenopathy.

Total or near-total thyroidectomy is recommended for the majority of children with PTC because the condition has been associated with an increased incidence of bilateral and multifocal disease. Bilateral resection also is recommended over lobectomy because it is more likely to decrease the incidence of persistent and recurrent disease.

“We frequently see patients who have had a nodule found but the lateral neck was never checked to see if lateral neck lymph node metastasis was present,” Bauer said. “So the patient goes to the operating room, they have their thyroid cancer removed, receive radioactive iodine therapy and then it’s discovered that there is lateral neck metastasis. Then they have to go back for a second surgery.”

Bauer said complete preoperative staging by ultrasound could help avoid this situation.

Importance of appropriate care

To minimize the risks of surgery, the task force recommends that thyroid surgery be performed at a hospital with pediatric specialty care and should also be performed by a surgeon who performs at least 30 thyroid surgeries annually.

“This is a huge limitation in pediatrics,” Bauer said. “Finding a high-volume thyroid surgeon who operates on the pediatric population is essential. It doesn’t matter what the surgeon’s training was, it matters that they are experienced and regularly perform thyroid surgery.”

This is particularly important for children with bulky, macroscopic cervical disease, pathologic lymph nodes greater than 1 cm in size, where more extensive neck dissection is required. The goal is to have complete resection of disease but to also reduce the risk of lifelong surgical complications.

The task force also recommends that pediatric patients should receive care by a multi-disciplinary team who are experienced in the evaluation and management of children and adolescents with thyroid nodules and thyroid cancer, helping to ensure treatment is optimized, that overly aggressive treatment is reduced, and that inappropriate long-term follow-up and additional treatment is individualized.           

Risk classification

In an effort to help guide the appropriate post-surgical management of pediatric patients with DTC, the guidelines present an initial ATA pediatric risk classification. “These risk levels are not risk of mortality, these are risks of persistent disease,” Bauer said. “Based on what was found during the initial surgery, which is assumed to have been a complete surgery because you have accurate preoperative staging and the right surgeon performing the procedure.”

He added that the risk levels are intended to say what postoperative staging should occur and to help define which patients would or would not benefit from radioactive iodine treatment.

Beyond the initial treatment, the age of the patients, size and invasive behavior of the tumor, presence of regional or distant metastasis, prior treatment history, trend in thyroglobulin and/or anti-thyroglobulin levels and iodine activity should all be considered when decided to observe or pursure additional treatment, according to the task force. – by Amber Cox

Disclosure: Bauer reports no relevant financial disclosures. Francis receives research support from Grifols, the Juvenile Diabetes Research Foundation and Novo Nordisk, and is an adviser to ThyCa. Please see the full study for a list of all other authors’ relevant financial disclosures.