Children with Thyroid Nodules and Differentiated Thyroid Cancer

Publication Date: July 10, 2015
Last Updated: November 20, 2024

Objective

Objective

The purpose of this patient summary is to provide guidance for the evaluation, treatment and follow-up of thyroid nodules and differentiated thyroid cancer (DTC) in children.

Overview and Background

Overview and Background

  • Differentiated thyroid cancer (DTC) is a type of thyroid cancer where the cancer cells look similar to normal thyroid cells under a microscope.
  • Differentiated Thyroid Cancer includes both papillary thyroid cancer and follicular thyroid cancer. So papillary and follicular thyroid cancers are collectively referred to as differentiated thyroid cancers (DTC).
    • Papillary thyroid cancer is the most common type, making up about 70% to 80% of all thyroid cancers. Papillary thyroid cancer can occur at any age. It tends to grow slowly and often spreads to lymph nodes in the neck. Papillary cancer has a generally excellent outlook, even if there is spread to the lymph nodes.
    • Follicular thyroid cancer makes up about 10% to 15% of all thyroid cancers in the United States. Follicular cancer can spread through the blood to distant organs, particularly the lungs and bones.
  • Thyroid cancer is usually very treatable and is often cured with surgery and, if indicated, radioactive iodine. Even when thyroid cancer is more advanced, effective treatment is available for the most common forms of thyroid cancer. While the diagnosis of cancer is terrifying, the prognosis for most patients with papillary and follicular thyroid cancer is excellent.
  • Compared with adults, thyroid neoplasms in the pediatric population exhibit differences in pathophysiology, clinical presentation, and long-term outcomes.
  • Tumors can either be benign or malignant. The main difference between benign and malignant tumors is that benign tumors are not cancerous, while malignant tumors are.
  • All children with differentiated thyroid cancer should be cared for by teams of physicians experienced in the management of thyroid cancer in children. This will facilitate help determine optimal therapy, and will reduce the possibility that treatment and follow up will be either overly aggressive or ineffective.

Additional Terms to Know

  • Thyroid stimulating hormone (TSH)
    • A hormone that signals the thyroid gland to produce and release hormones that regulate the body's metabolism.
  • Thyroglobulin
    • A protein produced by the thyroid gland that helps produce hormones that control metabolism and growth. It is also a key market to monitor for thyroid cancer.
  • Thyroglobulin antibodies (TgAb)
    • TgAb, or thyroglobulin antibodies, are proteins produced by the immune system that attack the protein thyroglobulin.
  • Fine-needle aspiration (FNA)
    • A minimally invasive procedure that uses a thin needle to extract cells, tissue, or fluid from a suspicious area of the body. It's also known as a fine-needle biopsy.
  • Total thyroidectomy
    • A surgical procedure to remove the entire thyroid gland.
  • Thyroid lobectomy
    • A thyroid lobectomy is used to remove one of your two thyroid lobes, leaving the other intact.
  • Isthmusectomy
    • A surgical procedure to remove the isthmus, the tissue that connects the two lobes of the thyroid gland.
  • Thyroid scintigraphy
    • Also known as a thyroid scan, this is a nuclear medicine imaging test that provides information about the structure and function of the thyroid gland.
  • Central neck dissection
    • A surgical procedure that removes lymph nodes from the central neck to treat thyroid cancer
  • SPECT-CT
    • A SPECT/CT scan is a nuclear medicine imaging test that combines a SPECT (single-photon emission computed tomography) scan with a CT (computed tomography) scan to create detailed images of the body's anatomy and physiology.
  • Radioactive iodine (RAI) therapy
    • A treatment that uses a radioactive form of iodine to treat thyroid conditions.
  • 131-I therapy
    • A type of radioactive iodine therapy often used for thyroid cancer.
  • 18FDG-PET/CT
    • Fludeoxyglucose F18 (FDG) is a positron-emitting radiotracer used with positron emission tomography (PET) to diagnose and monitor various conditions, including thyroid cancer.

Thyroid Nodules in Children

Thyroid Nodules in Children

Diagnosis of Thyroid Nodules in Children

  • The evaluation and treatment of thyroid nodules in children should be the same as in adults with the exceptions that:
    • Ultrasound characteristics and clinical context should be used rather than size alone to identify nodules that warrant fine-needle aspiration.
    • All fine-needle aspiration in children should be performed under ultrasound-guidance.
    • Preoperative fine-needle aspiration of a hyperfunctioning nodule in a child is not warranted as long as the lesion is removed.
    • A diffusely infiltrative form of papillary thyroid cancer may occur in children and should be considered in a clinically suspicious gland.
    • Surgery (lobectomy + isthmusectomy) is favored over repeat fine-needle aspiration for most nodules with indeterminate cytology
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  • In the evaluation of thyroid nodules in children, a positive mutational test means that the tumors are likely malignant.
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  • For patients with autoimmune thyroiditis that have suspected nodules or asymmetry after a thyroid exam, evaluation by an experienced thyroid ultrasonographer should be pursued.
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Overview of the Initial Management, Treatment, and Follow Up of Pediatric Thyroid Nodules

  • Solitary or suspicious thyroid nodule detected by imaging or a physical examination.
    • If thyroid stimulating hormone is suppressed, consider nuclear thyroid scintigraphy.
      • If nuclear thyroid scintigraphy shows hypofunctioning, consider fine-needle aspiration under ultrasound guidance.
      • If nuclear thyroid scintigraphy shows hyperfunctioning, consider surgery.
    • If thyroid stimulating hormone is NOT suppressed, see “Fine needle aspiration under ultrasound guidance” section below
  • Fine needle aspiration under ultrasound guidance
    • If results are not clear, but also not suspicious, repeat the ultrasound and fine needle aspiration in 3-6 months.
      • If after that the nodule is stable and/or found to be benign, repeat the testing again in another 6-12 months.
      • If after that the nodule is growing and/or the fine needle aspiration findings are suspicious, consider surgery.
    • If results are not clear, but there are suspicious findings, consider surgery.
    • If the results show that it is malignant, move on to treatment of differentiated thyroid cancer cancer
    • If the results show that it is benign, repeat the ultrasound testing again in another 6-12 months.
      • If the nodule is stable, repeat the ultrasound testing again every 1-2 years.
      • If the nodule is growing or findings are suspicious, repeat the fine needle aspiration and/or consider surgery.
  • Surgery guidance
    • If the results following surgery show malignancy, identify whether the cancer is medullary, papillary, or follicular, and determine an appropriate treatment plan.
    • If the results following surgery show that the tumor(s) are benign, check the adequacy of thyroid hormone levels in 4 weeks and schedule a clinical follow up.
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Treatment of Benign Thyroid Nodules

  • Due to a lack of data, no recommendations can be made regarding the use of thyroid-hormone-suppressive therapy with levothyroxine (LT4) for children with benign thyroid nodules.
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  • Benign lesions should be followed by serial ultrasound and undergo repeat fine-needle aspiration if suspicious features develop, or the lesion continues to grow.
    • Lobectomy may be performed in patients with compressive symptoms, cosmetic concerns, or patient/parent preference and should be considered in all apparently benign solid thyroid nodules >4 centimeters, those lesions demonstrating significant growth, or in the presence of other clinical concerns for malignancy.
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  • For children with suppressed thyroid-stimulating hormone associated with a thyroid nodule, thyroid scintigraphy should be pursued.
    • Surgical resection, most commonly lobectomy, is the recommended approach for most autonomous nodules in children and adolescents.
    • An autonomous nodule, also known as an autonomously functioning thyroid nodule (AFTN), is a mass of hyperfunctioning tissue in the thyroid gland that produces thyroid hormone without the usual regulation of normal thyroid tissue.
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Diagnosis of Differentiated Thyroid Cancer

Diagnosis of Differentiated Thyroid Cancer

  • An annual physical exam is recommended in children at high risk for thyroid cancer.
    • Additional imaging should be pursued if palpable nodules, thyroid asymmetry and/or abnormal cervical lymphadenopathy are found on exam.
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  • Routine ultrasound screening in high-risk children with a history of radiation exposure to the thyroid can neither be recommended for nor against until more data becomes available.
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  • Patients at increased risk of developing familial differentiated thyroid cancer should be referred to centers of excellence so that appropriate evaluation, follow-up, genetic counseling and/or treatment can be undertaken prior to undertaking any (potentially unnecessary) aggressive treatment decisions.
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  • The TNM Classification System should be used to describe the extent of disease in pediatric patients with papillary thyroid cancer. Children with papillary thyroid cancer should be stratified into risk levels (Low-, Intermediate-, or High-Risk) based on clinical presentation, tumor size and evidence of regional invasion or spread.
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  • The TNM classification system is a standard way to describe the spread and amount of cancer in a patient's body. It's used to stage most types of cancer, especially solid tumors. The TNM system is based on three main factors:
    • T: The size and extent of the tumor, including whether it has spread to nearby tissue
    • N: The number and location of nearby lymph nodes that contain cancer
    • M: Whether the cancer has spread to other parts of the body, known as metastasis
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TNM Classification System for Differentiated Thyroid Carcinoma

  • Primary Tumor (T)
    • TX - Size not assessed, limited to the thyroid
    • T1
      • T1a - ≤1 cm, limited to the thyroid
      • T1b - >1 cm but ≤2 cm, limited to the thyroid
    • T2 - >2 cm but ≤4 cm, limited to the thyroid
    • T3 - >4 cm, limited to the thyroid, or any tumor with minimal extrathyroid extension
    • T4
      • T4a - Tumor extends beyond the thyroid capsule to invade subcutaneous soft tissues, larynx, trachea, esophagus, or recurrent laryngeal nerve
      • T4b - Tumor invades prevertebral fascia or encases carotid artery or mediastinal vessels
  • Lymph Nodes (N)
  • NX - Regional lymph nodes not assessed
  • N0 - No regional lymph node metastasis
  • N1
    • N1a - Metastasis to Level VI (pretracheal, paratracheal, and prelaryngeal/Delphian lymph nodes)
    • N1b - Metastasis to unilateral, bilateral, or contralateral cervical Levels I, II, III, IV, or V) or retropharyngeal or superior mediastinal lymph nodes (Level VII)
  • Distant Metastasis (M)
    • MX - Distant metastasis not assessed
    • M0 - No distant metastasis
    • M1 - Distant metastasis

American Thyroid Association (ATA) Pediatric Thyroid Cancer Risk Levels and Postoperative Management in Children with Papillary Thyroid Carcinoma

  • ATA Risk Level: Low-Risk
    • Definition: Disease grossly confined to the thyroid with N0/Nx disease or patients with incidental N1a disease (microscopic metastasis to a small number of central neck lymph nodes)
    • Initial Postoperative Staging: Tg5
    • TSH Goals: 0.5 – 1.0 mIU/L
    • Surveillance of Patients With No Evidence of Disease: ultrasound at 6 months postoperatively and then annually × 5 years; Tg5 on LT4 every 3-6 months for two years and then annually
  • ATA Risk Level: Intermediate-Risk
    • Definition: Extensive N1a or minimal N1b disease
    • Initial Postoperative Staging: TSH-Stimulated Tg5 and diagnostic I scan in most patients
    • TSH Goals: 0.1 – 0.5 mIU/L
    • Surveillance of Patients With No Evidence of Disease: ultrasound at 6 months postoperatively, every 6-12 months for 5 years, and then less frequently; Tg5 on LT4 every 3-6 months for 3 years and then annually; Consider TSH-stimulated Tg5 ± diagnostic I scan in 1-2 years in patients treated with 131-I
  • ATA Risk Level: High-Risk
    • Definition: Regionally extensive disease (extensive N1b) or locally invasive disease (T4 tumors), with or without distant metastasis
    • Initial Postoperative Staging: TSH-Stimulated Tg5 and diagnostic I scan in all patients
    • TSH Goals: < 0.1 mIU/L
    • Surveillance of Patients With No Evidence of Disease: ultrasound at 6 months postoperatively, every 6-12 months for 5 years, and then less frequently; Tg5 on LT4 every 3-6 months for 3 years and then annually; TSH-stimulated Tg5 ± diagnostic I scan in 1-2 years in patients treated with 131-I
  • For patients falling into the intermediate or high risk level categories, additional postoperative staging is warranted to better define which patients may or may not benefit from additional therapy.
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  • It remains unclear if younger children (less than 10-15 years of age) are at greater risk for more extensive disease or higher rates of recurrence. Other factors aside from age may interact to modify this risk.
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Treatment of Differentiated Thyroid Cancer

Treatment of Differentiated Thyroid Cancer

Surgery for Pediatric Differentiated Thyroid Cancer

  • Pediatric thyroid surgery should be performed in a hospital with the full spectrum of pediatric specialty care, to include, but not be limited to: endocrinology, radiology (ultrasound and anatomic imaging), nuclear medicine, anesthesia, a high volume thyroid surgeon, and intensive care.
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  • When possible, any pediatric thyroid surgery should be performed by a surgeon who performs at least 30 or more cervical endocrine procedures annually. This is associated with lower complications rates, decreased hospital stay and lower cost.
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  • A comprehensive ultrasound to interrogate all regions of the neck is required in order to optimize the surgical plan.
    • Fine needle aspiration of suspicious lateral neck lymph nodes is recommended.
    • In order to optimize surgical planning, anatomic imaging by MRI or CT with contrast should be considered in patients with large or fixed thyroid masses, vocal cord paralysis, or bulky metastatic lymphadenopathy.
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  • For most children with differentiated thyroid cancer, total thyroidectomy is recommended.
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  • Central neck dissection is recommended for children with malignant cytology and clinical evidence of gross extrathyroidal invasion and/or loco-regional metastasis on preoperative staging or intraoperative findings.
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  • For patients with papillary thyroid cancer and no clinical evidence of gross extrathyroidal invasion and/or loco-regional metastasis, prophylactic central neck dissection may be selectively considered based upon tumor focality and size and the experience of the surgeon.
    • For patients with unifocal disease, ipsilateral central neck dissection, with pursuit of contralateral Central neck dissection based on intraoperative findings, may help balance the risks and benefits.
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  • Compartment-oriented resection is the recommended approach for lymph node dissection. "Berry picking" and attempting to use palpation to determine if metastatic disease is present in a lymph node are NOT recommended.
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  • Cytological confirmation of metastatic disease to lymph nodes in the lateral neck is recommended prior to surgery. Routine prophylactic lateral neck dissection is NOT recommended. However, lateral neck dissection should be performed on patients with cytologic evidence of metastases to the lateral neck. Measurement of thyroglobulin in the fine needle aspiration washout can be considered if the cytological diagnosis is equivocal.
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  • The early incorporation of calcium and calcitriol in children at high-risk for hypocalcemia may decrease the risks of symptomatic hypocalcemia. Postoperative intact parathyroid hormone measurement may be used to help predict which patients would benefit from more intensive monitoring and treatment.
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  • Postoperative staging is usually performed within 12 weeks after surgery and allows for stratification of patients who may or may not benefit from further therapy, to include additional surgery or radioactive iodine 131-I therapy.
    • ATA Pediatric Low- Risk patients may be initially assessed and followed with a thyroid stimulating hormone-suppressed thyroglobulin alone.
    • In contrast, a thyroid stimulating hormone-stimulated thyroglobulin and a diagnostic whole body scan is typically recommended to assess for evidence of persistent disease in ATA Pediatric Intermediate- and High-Risk patients.
    • Additional imaging, to include neck ultrasound and/or hybrid imaging may be used conjunctively to more accurately define the anatomic location of radioactive iodine uptake noted on a diagnostic whole body scan. Whenever possible, 123-I should be used for the diagnostic whole body scan.
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Initial Postoperative Staging for ATA Pediatric Intermediate- and High-Risk Pediatric Thyroid Carcinoma

  • Diagnostic whole body scan and thyroid stimulated thyroglobulin
    • Findings show that the cancer has spread, but no cervical uptake outside of thyroid bed
      • I therapy and post-treatment scan + surveillance
    • Findings show cervical uptake outside of the thyroid bed
      • Imaging to identify possible resectable disease and surgical consult
        • If no or minimal residual disease not amenable to surgery
          • I therapy and post-treatment scan & levothyroxine (LT4) suppression + surveillance
        • If significant residual disease amenable to surgery
          • Perform surgery
  • Findings show no or only minimal thyroid bed uptake
    • If stimulated thyroglobulin is less than 2 ng/mL
      • Not indicated + surveillance
    • If stimulated thyroglobulin is greater than 10 ng/mL
      • I therapy and post-treatment scan + surveillance
    • If stimulated thyroglobulin is between 2-10 ng/mL
      • I therapy and post-treatment scan & levothyroxine (LT4) suppression + surveillance

131-I Treatment

  • 131-I is indicated for treatment of iodine-avid persistent locoregional or nodal disease that cannot be resected as well as known or presumed iodine-avid distant metastases.
    • For patients with persistent disease following 131-I administration, the decision to pursue additional 131-I therapy should be individualized according to clinical data and previous response. The potential risks and benefits must be weighed on an individual basis.
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  • In order to facilitate 131-I uptake by residual iodine-avid cancer, the thyroid stimulating hormone should be above 30 mIU/L. This can be achieved in almost all children by withdrawing levothyroxine (LT4) for ≥14 days. In selected patients who cannot mount an adequate thyroid stimulating hormone response or cannot tolerate profound hypothyroidism, recombinant human thyroid stimulating hormone (rhTSH) may be considered. Low iodine diets have not been specifically evaluated in children but may enhance the effective radiation activity of 131-I and are recommended.
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  • Adequate hydration should be ensured in all children receiving therapeutic 131-I to facilitate clearance of the radioisotope, and additional supportive care with antiemetic medications and stool softeners/laxatives should be considered.
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  • The routine use of lithium and amifostine can NOT be recommended for differentiated thyroid cancer in children.
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  • Due to the differences in body size and iodine clearance in children compared with adults, it is recommended that all activities of 131-I should be calculated by experts with experience in dosing children.
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  • A posttreatment whole body scan is recommended for all children 4-7 days after 131-I therapy. The addition of SPECT-CT may help to distinguish the anatomic location of focal uptake.
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  • There are clear benefits and risks, both acute and chronic, following administration of 131-I during childhood. Families should be provided full disclosure of the risks and benefits of 131-I and their opinion must be considered in the final decision.
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Surveillance and Follow Up for Pediatric Differentiated Thyroid Cancer

  • Children with differentiated thyroid cancer may experience adverse psychosocial effects and be nonadherent with levothyroxine (LT4) therapy. Attention to these possibilities and supportive counseling as required are important adjuncts in the long-term follow up of children with differentiated thyroid cancer.
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  • Recurrence of differentiated thyroid cancer in children has been reported as long as 40 years after initial therapy. For that reason, children with differentiated thyroid cancer should be followed for life, even those with no evidence for disease.
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  • Thyroglobulin serves as a sensitive tumor marker in the evaluation, treatment, and long-term follow up of differentiated thyroid cancer in children.
    • Thyroglobulin antibody levels should be simultaneously measured in all samples since the presence of thyroglobulin antibodies will render the thyroglobulin result uninterpretable. Thyroglobulin and thyroglobulin antibody levels should be measured using the same laboratory and assay technique.
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  • An undetectable thyroid stimulating hormone-stimulated thyroglobulin (with negative thyroglobulin antibodies) identifies patients in remission with a very high probability to remain completely free of disease during follow-up and in whom the intensity of disease surveillance and the magnitude of thyroid stimulating hormone suppression should be relaxed.
    • Monitoring the thyroid stimulating hormone-suppressed thyroglobulin level on levothyroxine (LT4) treatment is the recommended approach to long-term follow-up, with the trend of this value being the most reliable indicator of disease activity.
    • Repeat thyroid stimulating hormone-stimulated thyroglobulin levels are not necessary if the thyroid stimulating hormone-suppressed thyroglobulin is detectable or if a previous thyroid stimulating hormone-stimulated thyroglobulin was undetectable.
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  • Detection of a low-level thyroid stimulating hormone-stimulated thyroglobulin<10 ng/ml) in a patient who has undergone surgery and therapeutic 131-I may indicate persistent disease. However, this value may decline over time without additional therapy. Continued follow up with serial thyroid stimulating hormone-suppressed thyroglobulin and thyroglobulin antibody levels as well as radiologic imaging (neck ultrasound) are indicated in this situation.
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  • Increasing or frankly elevated levels of thyroid stimulating hormone-stimulated thyroglobulin (>10 ng/ml) warrant further evaluation to localize disease and inform the decision as to whether additional surgery and/or 131-I therapy would be beneficial or whether one should pursue continued observation.
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  • The thyroglobulin level cannot be interpreted in children with positive thyroglobulin antibodies. In this setting, the thyroglobulin antibody trend should be followed using the same assay. If the thyroglobulin antibody trend is clearly rising, then further evaluation is warranted.
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  • Neck ultrasound is recommended in the follow-up of children with papillary thyroid cancer.
    • Neck ultrasound should be performed at least 6 months after initial surgery and then at 6-12 month intervals for ATA Pediatric Intermediate- and High-Risk patients and at annual intervals for ATA Pediatric Low-Risk patients.
    • Follow-up beyond 5 years should be individualized based on recurrence risk.
    • During the follow-up of children with papillary thyroid cancer who are suspected to have residual disease, a diagnostic whole body scan can be used to inform the decision of whether or not to use 131-I and the activity of 131-I to be administered. A final diagnostic whole body scan can be considered to confirm the absence of iodine-avid disease in children who were previously treated with 131-I and who have no evidence of disease 1-2 years after initial therapy.
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  • A diagnostic whole body scan should be performed in children with ATA Pediatric High-Risk disease who were previously treated with 131-I or known to have iodine-avid metastatic disease based upon a previous post treatment scan.
    • The diagnostic whole body scan should be obtained after at least 12 months of clinical follow-up, and deferred even longer in those children who continue to demonstrate a clinical response to previous treatment.
    • Once a negative diagnostic whole body scan is obtained, there is no benefit from serial diagnostic whole body scan to survey for disease recurrence as long as the patient otherwise remains without clinical evidence of disease.
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  • For the child with a detectable thyroid stimulating hormone-suppressed thyroglobulin but a negative cervical ultrasound and diagnostic whole body scan, contrast-enhanced cross-sectional imaging of the neck and chest should be considered once iodine excess has been eliminated as a cause of a false negative diagnostic whole body scan.
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  • The use of 18FDG-PET/CT can NOT be routinely recommended in the care of children who have persistent evidence of differentiated thyroid cancer on follow-up.
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  • Empiric 131-I therapy and a post treatment scan are NOT recommended to localize disease in the child with differentiated thyroid cancer and a negative diagnostic whole body scan unless there is evidence for clinical progression (e.g. a rising thyroglobulin level) and a documented clinical response to previous 131-I therapy.
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Thyroid Stimulating Hormone Suppression Therapy

  • Thyroid stimulating hormone suppression in children with differentiated thyroid cancer should be determined by ATA Pediatric Risk level and current disease status.
    • In children with known or suspected persistent disease, thyroid stimulating hormone suppression should be maintained.
    • In children with no evidence of disease, the thyroid stimulating hormone can be normalized to the low/normal range after an appropriate period of surveillance.
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Persistent/Recurrent Cervical Disease

  • The decision to treat or to observe structurally identifiable cervical disease should be individualized and include considerations of age, initial ATA Pediatric Risk classification, the presence of distant metastases, and prior treatment history (including complications from previous therapy), in addition to the size, extent, anatomic location and iodine avidity of the disease.
    • Children with macroscopic cervical disease (>1 centimeters in size) should be assessed by a high-volume thyroid surgeon to determine the feasibility of additional surgery.
    • Iodine-avid cervical disease could be treated with surgery or 131-I depending on individual patient risks and the presence or absence of distant metastases. Surgery would be favored for disease localized to the neck, especially if located in a lymph node compartment not previously operated upon.
    • If repeat surgery is performed, postoperative re-staging can be utilized to determine whether additional 131-I treatment is warranted, especially in the patient who has not received previous therapeutic 131-I.
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Pulmonary Metastases

  • Children with radioactive iodine-avid pulmonary metastases visualized with a diagnostic whole body scan are good candidates for 131-I therapy.
    • After a therapeutic activity of 131-I, the thyroid stimulating hormone-suppressed thyroglobulin level and imaging studies should be monitored until the full clinical and biochemical (thyroglobulin) response is reached.
    • If the full clinical and biochemical (thyroglobulin) response suggests persistent disease or if there is documented disease progression more than12 months after 131-I therapy, further evaluation with a diagnostic whole body scan and a thyroid stimulating hormone-stimulated thyroglobulin is indicated.
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  • Re-treatment of radioactive iodine-avid pulmonary metastases should be considered in children who have demonstrated progression of disease and a previous response to 131-I, with each treatment carefully individualized based on the child’s unique clinical course, side-effect profile, risk tolerance, and cumulative administered 131-I activity. Treatment with 131-I should be performed by experts with experience in managing children with pulmonary metastases.
    • Re-treatment of pulmonary metastases with 131-I is NOT recommended in children who do not have uptake on a diagnostic whole body scan and who have not demonstrated a previous response to 131-I.
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  • Pulmonary function testing should be considered in all children with diffuse pulmonary metastases, especially if multiple 131-I treatments are planned.
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  • Children with incidental papillary thyroid cancer should be managed similarly to other children with ATA Pediatric Low-Risk disease.
    • Neck ultrasound is recommended to detect contralateral disease or disease in the regional lymph nodes.
    • Completion thyroidectomy is not required in those children who had less than a total thyroidectomy unless there is ultrasound evidence and cytologic confirmation of contralateral thyroid disease or malignant lymphadenopathy.
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  • Most children with asymptomatic and non-progressive 131-I-refractory disease can be safely monitored while continuing thyroid stimulating hormone suppression.
    • Systemic treatment for advanced thyroid cancer in children remains unstudied and at this time should be considered the purview of specialized centers for the treatment of children with thyroid cancer. Consultation with experts in this area should be invited prior to initiation of treatment. In exceptional cases where systemic treatment is contemplated, clinical trials are preferred. If unavailable, the use of oral kinase inhibitors may be considered.
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Follicular Thyroid Carcinoma in Children

  • Follicular thyroid carcinoma is a rare malignancy in children. Because of the lack of data, strong recommendations regarding therapy cannot be made and further studies are required to better understand treatment options and long-term outcomes.
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  • Patients with clear evidence of vascular invasion (>3 involved blood vessels), known spread of the cancer, and/or tumor size >4 centimeters should be treated with total thyroidectomy and staged postoperatively with radioactive iodine therapy.
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  • Minimally-invasive follicular thyroid carcinoma <4 centimeters in size and with no or minimal vascular invasion (≤3 involved blood vessels) should be treated on a case-by-case basis. However, lobectomy alone rather than total thyroidectomy may be sufficient.
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  • In all children diagnosed with follicular thyroid carcinoma , consideration should be given to genetic counseling and genetic testing for germline mutations. This is particularly important for children with macrocephaly and/or for those with a family history suggestive of the PTEN hamartoma tumor syndrome (a group of inherited disorders that cause a variety of benign and malignant tumors, as well as other abnormalities).
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Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.