Figure 1. Low-power view of surgical specimen revealing tumor with papillary projections into cystic spaces and scattered calcification (hematoxylin-eosin, original magnification ×60).
Figure 2. Papillary projections surfaced by epithelial cells with nuclear folding and optically clear "Orphan Annie" nuclei (hematoxylin-eosin, original magnification ×400).
Figure 3. An epithelial cell with an intranuclear pseudoinclusion is present in the center of the field (hematoxylin-eosin, original magnification ×500).
Figure 4. Normal thyroid tissue adjacent to tumor (hematoxylin-eosin, original magnification ×250).
The thyroid gland develops from the floor of the pharynx between pharyngeal arches 1 and 2. It is anchored to the pharynx by a stalk known as the thyroglossal duct (TGD). As the thyroid gland descends to its normal position, it remains connected to the tongue by the TGD, which may pass around or through the hyoid bone. After embryogenesis the TGD usually disappears, but remnants may give rise to a TGD cyst or a fistula.
Thyroglossal duct cysts are the most common midline neck mass found in children. There is no sex predilection and they are typically diagnosed by age 10 years. Because of the likelihood of infection or rupture, surgical excision is the treatment of choice. The central portion of the hyoid bone as well as an en bloc resection of the soft tissue surrounding the cyst (Sistrunk procedure) is recommended to prevent recurrence.2 Microscopic examination reveals thyroid tissue remnants adjacent to the cyst in less than a third of the cases.
Carcinoma arising in a TGD is rare, with only approximately 200 reported cases and an estimated incidence of less than 1%. Most cases have occurred in adults and the overwhelming majority are papillary carcinoma. While the carcinoma may evolve from ductal epithelium, approximately 90% of cases originate from thyroid remnants.3 There seem to be no predisposing factors. Typically, neither clinical history nor physical examination leads to the preoperative diagnosis.
Thyroid papillary carcinoma has characteristic features that were readily found in this case. In addition to the numerous papillary protrusions, psammomatous calcifications are frequently scattered throughout the tumor. The cells surfacing the papillae exhibit nuclear folds and often have optically clear or "Orphan Annie" nuclei. Nuclear pseudoinclusions are also found, which are actually cytoplasmic invaginations.
While carcinoma of the thyroid gland is not common in children, when it does occur it is usually papillary. Papillary carcinoma is a very slow-growing neoplasm with an excellent cure rate. When it occurs in the thyroid, the treatment of choice is subtotal thyroidectomy. However, the best treatment of TGD papillary carcinoma is unclear. This is because between 11% and 33% of the cases reported have also had a second focus of tumor in the thyroid gland.3- 4 These second foci are often not detectable by either physical examination or nucleotide scans. Weiss and Orlich3 believed that because papillary thyroid carcinoma has a prolonged course, in the absence of objective suspicion of a thyroid mass, only long-term follow-up is warranted. In contrast, Heshmati et al4 recommended thyroidectomy on all patients with TGD carcinoma. However, the youngest patient in their series was aged 17 years and, therefore, they allow for a different approach in younger children. Given the excellent cure rate for papillary thyroid carcinoma, further treatment for this child should be limited to regular physical examinations and periodic imaging.
Accepted for publication February 19, 1999.
Reprints: John J. Buchino, MD, Kosair Children's Hospital, PO Box 35070, Louisville, KY 40207.
Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature
Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal
Thank you for submitting a comment on this article. It will be reviewed by JAMA Pediatrics editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more
Subscribe for full-text access to content from 1998 forward and a host of useful features
Activate your current subscription (AMA members and current subscribers)
Purchase Online Access to this article for 24 hours
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 4
Customize your page view by dragging & repositioning the boxes below.
and access these and other features:
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.