Thyroglossal Duct Carcinoma in Children: Case Presentation and Review of the Literature

Abstract and Introduction

Carcinoma within the thyroglossal duct (TGDCa) is a very rare pediatric tumor. This report presents the case of a 15-year-old girl with TGDCa, reviews the previously published pediatric cases of this tumor, and provides diagnostic and therapeutic considerations in TGDCa in children. Twenty one cases of TGDCa have been reported, 12 of them in females. Mean age at presentation was approximately 13 years for females and approximately 12 years for males. The duration of a midcervical mass prior to the diagnosis of TGDCa varied from 3 weeks to 8 years. The size of the neck mass at presentation averaged 2 cm–4 cm. All masses were asymptomatic, and the diagnosis of TGDCa was incidental following surgery. All pediatric cases of TGDCa reported thus far were of the papillary type, except for 3 patients who presented with a mixed papillary–follicular carcinoma. Capsular invasion was detected in 10 (45%) patients. Local invasive disease was found in 5 (23%) patients, but all removed thyroid glands^[12] were free of carcinoma. One patient had lung metastases. Thyroidectomy with subsequent radioiodine ablation was the treatment of choice in the majority of the cases. The reported prognosis for TGDCa in children was favorable, with only one reported death.

Thyroglossal duct cysts(TGDC) are the second most common neck masses and most common congenital cervical abnormalities in children.^[1] Approximately 7% of the adult population has thyroglossal remnants,^[2] with an equal sex distribution.^[3]

As the thyroid gland descends from the foramen cecum to its location at the point below the thyroid cartilage, it leaves behind an epithelial trace known as the thyroglossal tract. The tract disappears during the 5th–10th gestational week.^[3] Incomplete atrophy of the thyroglossal tract, or retained epithelial cysts, creates the basis for the origin of TGDC.^[4] A thyroglossal remnant can be a cyst, a tract or duct, a fistula, or an ectopic thyroid within a cyst or duct.

Thyroglossal duct carcinoma (TGDCa) is a very rare event,with approximately 250 cases reported in the literature since its first description by Brentano in 1911.^[5] The incidence of primary carcinoma within the thyroglossal duct (TGD)varies in the literature, and ranges from 0.7%^[6] –1.6%.^[7] These figures pertain to patients of all ages, but in the pediatric population this cancer is even scarcer. Our literature search revealed 22 patients (including our own patient) with TGDCa among children worldwide.^[6–21] This report presents a case of TGDCa in a child, and reviews the literature on this rare condition.

Thyroid. 2004;14(9) © 2004 Mary Ann Liebert, Inc.

Cite this: Thyroglossal Duct Carcinoma in Children: Case Presentation and Review of the Literature - Medscape - Sep 01, 2004.