Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Head and Neck Cancers
Table 1. Characteristics of Thyroid Carcinoma in Children and Adolescents Versus Adultsa continued...
Patients with thyroid cancer usually present with a thyroid mass with or without cervical adenopathy.[58,59,60,61] Younger age is associated with a more aggressive clinical presentation in differentiated thyroid carcinoma. Compared with adults, children have a higher proportion of nodal involvement (40%–90% vs. 20%–50%) and lung metastases (20%–30% vs. 2%). Likewise, when compared to pubertal adolescents, prepubertal children have a more aggressive presentation with a greater degree of extrathyroid extension, lymph node involvement, and lung metastases. However, outcome is similar in the prepubertal and adolescent groups.
Initial evaluation of a child or adolescent with a thyroid nodule should include the following:
- Ultrasound of the thyroid.
- Serum thyroid-stimulating hormone (TSH) level.
- Serum thyroglobulin level.
Tests of thyroid function are usually normal, but thyroglobulin can be elevated.
Fine-needle aspiration as an initial diagnostic approach is sensitive and useful. However, in doubtful cases, open biopsy or resection should be considered.[63,64,65,66,67] Open biopsy or resection may be preferable for young children as well.
Table 2. Thyroid Carcinomas in Children
|Histology||Associated Chromosomal Abnormality||Presentation||Diagnosis||Treatment|
|EGF = epidermal growth factor; MEN2 = multiple endocrine neoplasia type 2; TSH = thyroid-stimulating hormone.|
|Papillary thyroid carcinoma (differentiated with generally a benign course)||RET/PTCmore common in children.BRAFV600E mutations seen in adults are rare in children.||Thyroid mass. Prepubertal children more often with nodal and lung metastases.||Ultrasound, TSH, thyroglobulin. Fine needle or open biopsy.||Total or near-total thyroidectomy; I-131; thyroid hormone. In metastatic or recurrent disease, tyrosine kinase or EGF receptor inhibitors may be of benefit.|
|Follicular thyroid carcinoma (differentiated with generally benign course)||Sporadic or familial||Thyroid mass. Prepubertal children more often with nodal and lung metastases.||Ultrasound, TSH, thyroglobulin. Fine needle or open biopsy.||Total or near-total thyroidectomy; I-131; thyroid hormone. In metastatic or recurrent disease, tyrosine kinase or EGF receptor inhibitors may be of benefit.|
|Medullary thyroid carcinoma||MEN2||Aggressive. 50% with metastases at presentation.||In familial MEN2,RETtesting.||Aggressive surgical intervention. Prophylactic thyroidectomy is indicated in familial cases.|
Treatment of papillary and follicular thyroid carcinoma
The management of differentiated thyroid cancer in children has been reviewed in detail. Also, the American Thyroid Association Taskforce  has developed guidelines for management of thyroid nodules and differentiated thyroid cancer in older adolescents and adults; however, it is not yet known how to apply these guidelines to thyroid nodules in children.
Surgery performed by an experienced thyroid surgeon is the treatment required for all thyroid neoplasms.[53,56] For patients with papillary or follicular carcinoma, total or near-total thyroidectomy plus cervical lymph node dissection is the recommended surgical approach.[53,58,69] This aggressive approach is indicated for several reasons:
- Up to 40% of children with differentiated thyroid carcinoma have multifocal disease and a higher recurrence risk if less than a total thyroidectomy is performed.
- Many children have disseminated disease and require radioactive iodine therapy.
- Sensitive assays for serum thyroglobulin are used as a marker for active disease and are most useful after total thyroidectomy.[46,51,53]