Pediatric Papillary Thyroid Cancer: Current Management Challenges

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the ONA take:

Although rare, differentiated thyroid cancer, which manifests almost exclusively as papillary thyroid cancer (PTC), represents between 1% and 2% of all pediatric malignancies and its incidence is rising. Management of pediatric PTC is mostly based on findings from studies of PTC in adults as there have been limited studies that have evaluated treatments in children with PTC. As a result, many challenges with respect to the treatment of pediatric patients with PTC persist.

For example, studies on the prevention of salivary gland damage in pediatric patients receiving iodine-131 (I-131) are completely lacking despite it being one of the most frequent long-term complications of I-131 therapy. A double-blind study in adult patients showed that administration of amifostine maintained salivary gland function during I-131 therapy; however, its safety and efficacy in pediatric patients has not been established.

Furthermore, data on the late effects of I-131 therapy, including development of second primary malignancies, following total thyroidectomy with lymph node dissection in pediatric patients with advanced disease remain scarce and may be currently underestimated. Because these late effects, like xerostomia, lacrimal gland dysfunction, and transient male/female gonadal dysfunction, can severely impact quality of life, additional research is crucial to determining the optimal cumulative dose and frequency of radiation exposure in this pediatric population.

OncoTargets and Therapy journal
OncoTargets and Therapy journal

Abstract: Although with a standardized incidence of 0.54 cases per 100,000 persons, differentiated thyroid cancer (DTC) is a rare disease in children and adolescents, it nonetheless concerns ~1.4% of all pediatric malignancies. Furthermore, its incidence is rising. Due to the rarity and long survival of pediatric DTC patients, in most areas of treatment little evidence exists. Treatment of pediatric DTC is therefore littered with controversies, many questions therefore remain open regarding the optimal management of pediatric papillary thyroid cancer (PTC), and many challenges remain unsolved. In the present review, we aim to provide an overview of these challenging areas of patient and disease management in pediatric PTC patients. Data on diagnosis, surgery, radionuclide, and endocrine therapy are discussed, and the controversies therein are highlighted.


Keywords: pediatric cancer, papillary thyroid cancer, survival, I-131 therapy, follow-up, treatment 

INTRODUCTION

Although with a standardized incidence of 0.54 cases per 100,000 persons,1 differentiated thyroid cancer (DTC) is a rare disease in children and adolescents; it nonetheless concerns ~1.4% of all pediatric malignancies. Furthermore, its incidence is rising.2 In children and adolescents, DTC almost exclusively manifests in the form of papillary thyroid cancer (PTC).

Pediatric PTC shows no relationship with gender in prepubertal children. However, with increasing age, the incidence of PTC also increases, especially in young female adolescents.2,3 In the case of DTC diagnosis, these patients more or less exclusively present with a well-differentiated PTC.

Compared to adults, children more often present with lymph node and/or disseminated (so-called miliary) lung metastases. The latter usually only becomes apparent after radioactive iodine (RAI), as they are usually not visible when morphologic imaging techniques such as X-ray or computed tomography (CT) are used. Fortunately, in these young patients, PTC is almost guaranteed to show an avid uptake of RAI. As a result, prognosis in the pediatric PTC population is excellent when appropriate treatment is administered.  

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