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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

CharacteristicChildren and Adolescents (%)Adults (%)
a Adapted from Yamashita et al.[57]
Histologic subtype:  
Papillary67–9885–90
Follicular4–23<10
Medullary2–83
Poorly differentiated<0.12–7
 
Gene rearrangements:  
RET/PTC38–870–35
NTRK 15–115–13
AKAP9-BRAF111
PAX8-PPARGUnknown0–50
 
Point mutations:  
BRAF0–60–43
RASfamily0–1625–69
GNAS011
TP530–230–20
 
Other:  
Multicentric30–5040–56
Lymph node involvement30–905–55
Extrathyroid extension24–5116–46
Vascular invasion<3114–37
Distant metastases10–205–10

Clinical presentation

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%).[56] 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.[62]

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.[63,64,65,66,67] Open biopsy or resection may be preferable for young children as well.

Table 2. Thyroid Carcinomas in Children

HistologyAssociated Chromosomal AbnormalityPresentationDiagnosisTreatment
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 familialThyroid 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 carcinomaMEN2Aggressive. 50% with metastases at presentation.In familial MEN2,RETtesting.Aggressive surgical intervention. Prophylactic thyroidectomy is indicated in familial cases.
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