Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer

Abstract

Background: Previous guidelines for the management of thyroid nodules and cancers were geared toward adults. Compared with thyroid neoplasms in adults, however, those in the pediatric population exhibit differences in pathophysiology, clinical presentation, and long-term outcomes. Furthermore, therapy that may be recommended for an adult may not be appropriate for a child who is at low risk for death but at higher risk for long-term harm from overly aggressive treatment. For these reasons, unique guidelines for children and adolescents with thyroid tumors are needed.

Methods: A task force commissioned by the American Thyroid Association (ATA) developed a series of clinically relevant questions pertaining to the management of children with thyroid nodules and differentiated thyroid cancer (DTC). Using an extensive literature search, primarily focused on studies that included subjects ≤18 years of age, the task force identified and reviewed relevant articles through April 2014. Recommendations were made based upon scientific evidence and expert opinion and were graded using a modified schema from the United States Preventive Services Task Force.

Results: These inaugural guidelines provide recommendations for the evaluation and management of thyroid nodules in children and adolescents, including the role and interpretation of ultrasound, fine-needle aspiration cytology, and the management of benign nodules. Recommendations for the evaluation, treatment, and follow-up of children and adolescents with DTC are outlined and include preoperative staging, surgical management, postoperative staging, the role of radioactive iodine therapy, and goals for thyrotropin suppression. Management algorithms are proposed and separate recommendations for papillary and follicular thyroid cancers are provided.

Conclusions: In response to our charge as an independent task force appointed by the ATA, we developed recommendations based on scientific evidence and expert opinion for the management of thyroid nodules and DTC in children and adolescents. In our opinion, these represent the current optimal care for children and adolescents with these conditions.

FIG. 1.

Initial evaluation, treatment, and follow-up of the pediatric thyroid nodule. ¹Assumes a solid or partially cystic nodule ≥1 cm or a nodule with concerning ultrasonographic features in a patient without personal risk factors for thyroid malignancy (see Sections B3 and B4). ²A suppressed TSH indicates a value below the lower limits of normal. ³Refer to PTC management guidelines (Section C1) or MTC management guidelines. ⁴Surgery can always be considered based upon suspicious ultrasound findings, concerning clinical presentation, nodule size >4 cm, compressive symptoms, and/or patient/family preference. ⁵Surgery implies lobectomy plus isthmusectomy in most cases. Surgery may be deferred in patients with an autonomous nodule and subclinical hyperthyroidism, but FNA should be considered if the nodule has features suspicious for PTC. (See Section B10.) Consider intraoperative frozen section for indeterminate and suspicious lesions. Can consider total thyroidectomy for nodules suspicious for malignancy on FNA. ⁶Consider completion thyroidectomy ± RAI versus observation ± TSH suppression based upon final pathology (see Section E1).

FIG. 2.

Initial postoperative staging for American Thyroid Association pediatric intermediate- and high-risk pediatric thyroid carcinoma. ¹Assumes a negative TgAb (see Section D2) and a TSH >30 mIU/L; in TgAb-positive patients, consideration can be given (except in patients with T4 tumors or clinical M1 disease) to deferred evaluation to allow time for TgAb clearance (“delayed” staging). ²Imaging includes neck ultrasonography ± SPECT/CT at the time of the diagnostic thyroid scan. ³Consider ¹³¹I in patients with thyroid bed uptake and T4 tumors or known residual microscopic cervical disease. ⁴While there are no prospective studies in patients ≤18 years of age, the use of ¹³¹I remnant ablation may not decrease the risk for persistent or recurrent disease. Consider surveillance rather than ¹³¹I with further therapy determined by surveillance data. ⁵Repeat postoperative staging 3–6 months after surgery. ⁶See Table 6 and Figures 3 and 4.

FIG. 3.

Management of the pediatric patient with known or suspected residual/recurrent disease (no known distant metastases). This algorithm is intended to be used in children who are known or suspected to have residual or recurrent disease based upon the suppressed Tg level and knowledge of previous disease extent 6–12 months after all primary therapies have been completed. ¹Assumes a negative TgAb (see Section D2); in TgAb-positive patients, the presence of TgAb alone cannot be interpreted as a sign of disease unless the titer is clearly rising. ²Imaging includes SPECT/CT at the time of the diagnostic thyroid scan and/or contrast-enhanced CT/MRI neck. ³Repeat ¹³¹I therapy in patients previously treated with high-dose ¹³¹I should generally be undertaken only if iodine-avid disease is suspected and a response to previous ¹³¹I therapy was observed (see Sections D7 and D8).

FIG. 4.

Management of the pediatric patient with known distant metastases. ¹Assumes a negative TgAb (see Section D2); in TgAb-positive patients, the presence of TgAb alone cannot be interpreted as a sign of disease unless the titer is clearly rising; a declining TgAb titer would suggest continued response to treatment. ²Tg can transiently rise after ¹³¹I therapy and should not be misinterpreted as evidence for progression. ³Repeat ¹³¹I therapy in patients previously treated with high-dose ¹³¹I should be undertaken only if iodine-avid disease is suspected and if there was a previous response to therapy (see Section D7 and D8).