Pediatric Thyroid Cancer 

  • Author: Mark E Gerber, MD, FACS, FAAP; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Apr 5, 2011
 

Background

Although a review of the literature contains numerous reports on the subject of pediatric thyroid carcinoma, the low incidence and subsequent lack of prospective randomized trials make drawing absolute conclusions regarding the definitive workup, management, and treatment of this disease difficult.

A detailed understanding of how to perform a comprehensive evaluation of the pediatric thyroid nodule is necessary in order to establish the diagnosis of pediatric thyroid cancer. The incidence of head and neck malignancies, including those of the thyroid, has increased 25% during the past 30 years.[1] Although the incidence of thyroid nodules in children is rare before adolescence (1.5%), pediatric thyroid nodules have a 26.4% mean risk of cancer. Some authors have reported an incidence of as high as 36%.[2] Moreover, pediatric thyroid nodules are 4 times more likely to carry a diagnosis of thyroid cancer than adult nodules. Because pediatric thyroid nodules carry this increased risk of malignancy, physicians should perform an expeditious workup.[3, 4]

The recommended diagnostic protocol of thyroid nodules consists of the following steps:

  1. Child's history, including the prior existence and treatment of a benign thyroid disease
  2. Clinical examination
  3. Laboratory tests
  4. Thyroid ultrasonography
  5. Fine-needle aspiration biopsy (FNAB)

The beneficial role of scintigraphy is limited, and molecular marker analysis is currently more beneficial in a clinical research setting.[5, 6]

Most childhood thyroid nodules are asymptomatic and are detected by parents or by physicians during routine examination. Only about 50% of children with thyroid carcinoma present with nodular thyroid enlargement as the presenting symptom. Follicular adenoma is the most common cause of solitary thyroid nodules in the pediatric population; however, solitary nodules in children reportedly have a 20-73% incidence of malignancy.[7, 8, 9] A painless noninflammatory metastatic cervical mass is the presenting symptom in 40-60% of patients.[10] Malignant lesions are usually papillary and follicular carcinomas. Radiation exposure, which is still used either as therapy prior to bone marrow transplantation or as a treatment of Hodgkin disease, remains a major risk factor.[11]

The subsequent diagnostic workup is aimed at determining whether the lesion represents a malignancy. Collected data can be useful in preoperative planning if surgery is indicated. Pediatric and adult thyroid cancers have differing biological behaviors. Despite the fact that pediatric thyroid cancer usually presents at an advanced stage, it carries an excellent prognosis.

An image depicting thyroid cancer can be seen below.

A monomorphous cell population of Hürthle cells arA monomorphous cell population of Hürthle cells arranged in loosely cohesive clusters and single cells. The cells are polyhedral and have abundant granular cytoplasm with well-defined cell borders. The nuclei are enlarged and have a central prominent macronucleolus.
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Epidemiology

Frequency

United States

Thyroid cancer, the most common pediatric endocrine neoplasm, represents 1-1.5% of all pediatric malignancies and 5-5.7% of malignancies in the head and neck. Only 5% of all thyroid cancers occur in children and adolescents.[12] Thyroid nodules occur in 4-7% of the general adult population and in only 1-2% of the pediatric population. These numbers are estimated using a compilation of data from multiple reports.[11, 13, 14]

Paradoxically, despite the lower incidence of thyroid nodules in children, a pediatric thyroid nodule has a greater risk of containing or developing a malignancy. Whereas 5% of nodules in adults are malignant, in the pediatric population, the percentage of malignant nodules is 26.4%.[15] The incidence of malignancy in multinodular goiter is 1-7% and 10-25% in solitary nodules.[11] Pediatric thyroid cancer (3% prevalence) in adolescents is also associated with juvenile autoimmune thyroiditis.[16]

Papillary thyroid cancer is by far the common thyroid malignancy in children. Although papillary carcinoma is more aggressive in children than in adults, pediatric papillary cancer carries a much better prognosis that adult thyroid cancer.[17]

Medullary thyroid cancer (MTC), which constitutes 5% of pediatric thyroid malignancies, is usually associated with multiple endocrine neoplasia type 2 (MEN2) in the pediatric population. The inheritance pattern occurs either sporadically or as familial MTC without other associated endocrine abnormalities. MEN2 consists of MTC and pheochromocytoma and either hyperparathyroidism (2A) or mucosal neuromas (2B). MTC associated with MEN2B is more virulent and may occur and metastasize early in infancy.

International

After the Chernobyl nuclear power plant disaster, individuals living in Russia, Ukraine, and Belarus were exposed to significant levels of radioactive iodines, primarily 131I. This radioactivity, which is concentrated in the thyroid gland, has resulted in a substantial increase in pediatric thyroid cancer rates among this cohort of children.[18, 19]

Mortality/Morbidity

Pediatric thyroid malignancies are usually a well-differentiated papillary subtype or the papillary-follicular subtype, but all histologic types have been observed. Children commonly present with advanced disease. At presentation, 70% of patients have extensive regional nodal involvement, and 10-20% of patients have distant metastasis.[20] The lungs are the most common sites of metastasis.

Pediatric patients seem to have higher local and distant recurrence rates than adults, but they tend to respond rapidly to therapy. The prognosis for children is excellent, with mortality rates of less than 10%.[21] Benign tumors such as follicular adenomas should be considered at risk for tumor progression toward follicular thyroid carcinoma, and they must be surgically addressed.[15]

Sex

Thyroid carcinoma is 2-3 times more common in females.[22]

The gender distribution of thyroid carcinoma differs between adults and children. Thyroid cancer is 4 times as common in women as in men. This difference is not seen in individuals younger than 15 years; the girl-boy ratio is as low as 1.5:1. However, in individuals aged 15–20 years, the female-to-male ratio is 3:1.[23] This implies that female sex hormones, especially during puberty, play a significant yet still undefined role in the increased incidence of thyroid cancer in females.[12]

Age

Age is a major determinant of both the incidence and recurrence of pediatric thyroid carcinoma. Pediatric thyroid carcinoma occurs more frequently in adolescents, although it has been reported in the neonatal period.[24] In children younger than 10 years, identified thyroid lesions are more likely to be malignant.[25] Children younger than 10 years are also more likely to have recurrent cancer.[21]

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Contributor Information and Disclosures
Author

Mark E Gerber, MD, FACS, FAAP  Clinical Assistant Professor of Otolaryngology, University of Chicago, Pritzker School of Medicine; Section Head, Pediatric Otolaryngology-Head and Neck Surgery, NorthShore University HealthSystem

Mark E Gerber, MD, FACS, FAAP is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American Cleft Palate/Craniofacial Association, American College of Surgeons, American Rhinologic Society, American Society of Pediatric Otolaryngology, and Society for Ear, Nose and Throat Advances in Children

Disclosure: Nothing to disclose.

Coauthor(s)

Brian Kip Reilly, MD  Assistant Professor of Otolaryngology and Pediatrics, Department of Otolaryngology, Children's National Medical Center, George Washington University School of Medicine

Brian Kip Reilly, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Russell A Faust, MD, PhD  Consulting Staff, Department of Otolaryngology, Columbus Children's Hospital

Russell A Faust, MD, PhD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Legal Medicine, American Laryngological Rhinological and Otological Society, American Rhinologic Society, American Society for Head and Neck Surgery, and American Society of Law, Medicine & Ethics

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Nader Sadeghi, MD, FRCS(C)  Professor of Surgery, Director of Head and Neck Surgery, George Washington University School of Medicine and Health Sciences

Nader Sadeghi, MD, FRCS(C) is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society, Federation of Medical Specialists in Quebec, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Christopher L Slack, MD  Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders

Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA  Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society

Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation Unrestricted gift Unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo Consulting; Medvoy Ownership interest Management position; Cerescan Imaging Honoraria Consulting; GYRUS ACMI Honoraria Consulting

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A monomorphous cell population of Hürthle cells arranged in loosely cohesive clusters and single cells. The cells are polyhedral and have abundant granular cytoplasm with well-defined cell borders. The nuclei are enlarged and have a central prominent macronucleolus.
 
 
 
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