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    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

    Characteristic Children and Adolescents (%) Adults (%)
    a Adapted from Yamashita et al.[59]
    Histologic subtype:
    Papillary 67-98 85-90
    Follicular 4-23 <10
    Medullary 2-8 3
    Poorly differentiated <0.1 2-7
    Gene rearrangements:
    RET/PTC 38-87 0-35
    NTRK 1 5-11 5-13
    AKAP9-BRAF 11 1
    PAX8-PPARG Unknown 0-50
    Point mutations:
    BRAF 0-6 0-43
    RASfamily 0-16 25-69
    GNAS 0 11
    TP53 0-23 0-20
    Other:
    Multicentric 30-50 40-56
    Lymph node involvement 30-90 5-55
    Extrathyroid extension 24-51 16-46
    Vascular invasion <31 14-37
    Distant metastases 10-20 5-10

    Clinical presentation

    Patients with thyroid cancer usually present with a thyroid mass with or without cervical adenopathy.[60,61,62,63] 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%).[58] Likewise, when compared with 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.[64] In well-differentiated thyroid cancer, male gender, large tumor size, and distant metastases have been found to have prognostic significance for early mortality; however, even patients in the highest risk group who had distant metastases had excellent survival at 90%.[65]

    Diagnostic evaluation

    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.[66,67,68,69,70] 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.
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