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Compared to adults, thyroid nodules are much less common in children and adolescents. However, childhood thyroid nodules are more likely to be malignant. In some cases, pediatric thyroid cancer may have spread to nearby lymph nodes and distant sites (lung) at diagnosis. It is also more likely to recur. Despite the greater tendency for spread in children compared to adults, differentiated thyroid cancer has an excellent outcome, with greater than 95% survival rate.
Most thyroid cancers in children are differentiated thyroid cancers (DTC), which arise from follicular cells in the thyroid gland. There are two types of differentiated thyroid cancers: papillary and follicular. Approximately 90% of pediatric thyroid cancers are papillary thyroid cancer.
Papillary thyroid cancer can often present with more than one nodule and involve both lobes of the thyroid (bilateral). In many cases, it has spread outside the thyroid to regional (cervical) lymph nodes at diagnosis. Follicular thyroid cancer is usually localized in the neck but is more likely to spread to lungs and bones.
Differentiated thyroid carcinomas are iodine-avid. This means that they take up iodine. This feature is important for screening and treatment with radioactive iodine.
Differentiated thyroid cancer occurs most often in older children and teens. Adolescents are 10 times more likely to develop thyroid cancer compared to younger children. These cancers are more common in females than males. Certain genetic factors may increase risk, and the tendency to develop thyroid and other cancers may be passed down in families. Children with differentiated thyroid cancer often have gene rearrangements of the RET gene.
|Familial Adenomatous Polyposis (FAP)
|PTEN Hamartoma Tumor Syndrome
Patients treated with radiation as a medical therapy are at increased risk of developing thyroid cancer. Environmental exposure to radiation such as radioactivity from nuclear disasters also increases risk. Higher doses of radiation and younger age during exposure are associated with higher risk.
The main sign of thyroid cancer is a nodule, or lump, in the thyroid gland. Sometimes, lymph nodes in the neck will appear swollen. In rare cases, symptoms might include problems breathing, difficulty or pain swallowing, and hoarseness.
However, often thyroid cancer does not cause any symptoms and may be found as part of a routine exam.
The stage, or extent of disease, depends on whether the cancer has spread. Stage of thyroid cancer is based on the characteristics of the thyroid nodules as well as spread of disease to lymph nodes and other parts of the body (lungs).
Patients who have no spread of disease outside the neck are considered Stage I.
Patients with distant spread of disease outside the neck are considered Stage II.
Post-operative staging is used to classify patients into risk groups. For all pediatric thyroid cancer patients, the risk of death is very low. However, some patients may be at higher risk for continued disease or spread of disease (metastases) after surgery.
|Risk||Spread of disease|
|Low Risk||Cancer is only in the thyroid gland with little or no spread to lymph nodes|
|Intermediate Risk||Some spread of cancer to nearby lymph nodes|
|High Risk||Significant spread of cancer to nearby lymph nodes, invading tissues outside the thyroid, or distant spread (to lungs)|
Thyroid cancer has a >95% survival rate in pediatric patients. Recurrence is more likely in children under 10 years of age and patients who have regional lymph node involvement at diagnosis. However, even with risk of recurrence, chance of survival is very good.
Factors that influence prognosis include:
Care by a multidisciplinary pediatric team is important to manage assessment, treatment, and long-term monitoring of patients with pediatric thyroid cancer. Care decisions are focused on balancing risk of continued disease and harm due to treatment side effects. Because of the risk of recurrence and other considerations (e.g., hormone function, genetic predisposition), ongoing follow-up is needed for all patients.
Patients treated for thyroid cancer need lifelong monitoring and follow-up care by an interdisciplinary medical team. Specific recommendations for frequency and types of tests differ according to patient needs and thyroid cancer type and stage.
In differentiated thyroid cancer, thyroglobulin levels can serve as tumor markers to assist in monitoring. Additional considerations include support for adherence to medications including thyroid hormone replacement therapy and TSH suppression.
Key aspects of long-term care:
Recurrence of thyroid cancer can occur many years after treatment. Ongoing monitoring can help with early detection of late recurrences.
Patients can benefit from psychosocial support during treatment and survivorship. Care team members representing Psychology, Child Life, Social Work, and other disciplines can assist with coping and adherence to treatments that may affect quality of life. Possible issues include adjustment to daily medication use, body image concerns due to surgical scars, and other adjustment needs.
Patients may also need physical therapy after surgery to assist with neck mobility and range of motion.
Adjusting to a thyroid cancer diagnosis can be challenging for families. Although the prognosis is generally very good, the disease requires lifelong management through medication and monitoring. Support needs may increase during life transitions such as adolescence and young adulthood as patients gain independence. Adjustment challenges may also be greater for patients who experience thyroid cancer a second cancer.
Patients receiving radioactive iodine therapy should be followed for potential long-term and late effects of treatment. Special considerations include:
For general health and disease prevention, all cancer survivors should adopt healthy lifestyle and eating habits, as well as continue to have regular physical checkups and screenings by a primary physician. Survivors should discuss their medical histories with their health care providers including all cancer therapies.
Reviewed: June 2018