Pediatric Thyroid Carcinoma Research Articles

Management of pediatric thyroid carcinoma

Management of pediatric thyroid carcinoma

Pediatric Papillary Thyroid Carcinoma: Outcomes After Surgery Without Adjuvant Radioactive Iodine.

Pediatric papillary thyroid carcinoma (PTC) is usually treated with total thyroidectomy followed by radioactive iodine (RAI). Recently, RAI is being used more selectively based on surgical pathology and postoperative dynamic risk stratification (DRS). To describe patients with pediatric PTC not initially treated with RAI and their disease outcomes. This was an ambispective study at a tertiary cancer center of patients < 19 years diagnosed from 1/1/1990 to 12/31/2021 with stage I PTC who intentionally were not treated with RAI within a year of diagnosis. We assessed clinical characteristics, management, and disease outcomes using DRS. Of 490 PTC patients, we identified 93 eligible patients (median age at diagnosis 16y; 87% female), including 46 (49%) with cervical lymph node metastases. Initial management included: total thyroidectomy ± neck dissection (n=69, 75%), lobectomy ± neck dissection (n=20, 21%), or a Sistrunk procedure for ectopic PTC (n=4, 4%). After a median follow-up of 5.5 years (range 1-26), most patients (85/93; 91%) remained disease-free with no further therapy. Persistent (n=5) or recurrent (n=3) disease was found in 9% of the entire cohort. Four patients ultimately received RAI, of which only one clearly benefited, and additional surgery was performed or planned in four patients, two of whom had an excellent response at last follow-up. Selected pediatric PTC patients, even those with lymph node metastases, may not require therapeutic 131I and can avoid the unnecessary risks of RAI while still benefitting from the excellent long-term outcomes that are well-described for this disease.

Recurrence Analysis of Pediatric Thyroid Carcinoma in Dr. Cipto Mangunkusumo General Hospital and its associated factors

Introduction. Thyroid cancer is the most common endocrine malignancy in children aged 0-14 years, frequently occurring in adolescents and young adults. The global incidence rate stands at 0.54 cases per 100,000 individuals. Pediatric patients exhibit higher recurrence rates compared to adults, although their mortality rates are low. Research exclusively for the pediatric population is still sparse in Indonesia. Thus, This study aimed to provide essential data on recurrence and mortality rates of pediatric thyroid cancer, along with an analysis of contributing factors. Method. This study is a retrospective cohort focusing on survival analysis. The research sample was drawn from the medical records registry of Cipto Mangunkusumo General Hospital (CMGH) from 2014 to 2020. Inclusion criteria encompassed all pediatric patients (0-18 years) diagnosed with thyroid cancer since January 1, 2014, and with confirmed thyroid cancer pathology results. Bivariate and multivariate analyses and recurrence event analyses proceeded. Results were considered significant at p &lt;0.05. Results. Out of 35 pediatric subjects with thyroid cancer, complete data for analysis were available for 29 patients. The overall median age was 16 (5-18) years. No reported mortality was observed among the subjects. The median overall survival was 60.0 months (32.5-60.0). The overall recurrence rate was 25.0%. None of the factors studied age, sex, histopathology, therapy, and types of surgery affect recurrency (p &gt;0.05). Conclusion. The recurrence rate among pediatric patients with thyroid cancer at CMGH is 25.0%, with a median disease-free survival of 96.0 months. No significant factors contributing to the increased risk of recurrence.

The significance and prognostic value of multifocal papillary thyroid carcinoma in children and adolescents

IntroductionThe prognostic value of multifocality in paediatric papillary thyroid carcinoma (PTC) patients remains a subject of debate. This study aimed to explore the clinical significance and prognostic value of multifocality in children and adolescents with PTC.MethodsThis study retrospectively analysed the clinicopathological characteristics and postoperative follow-up data of 338 PTC patients aged ≤ 20 years from May 2012 to July 2022. The clinical and pathological characteristics of 205 patients with unifocal lesions and 133 patients with multifocal lesions were compared. A logistic regression model evaluated the relationship between multifocal lesions and disease recurrence/persistence in children and adolescents with PTC. Based on the median follow-up time of children with multifocal PTC, 114 patients with multifocal PTC older than 20 years were added, and the clinicopathological characteristics were compared between the 133. paediatric/adolescent patients and 114 adult patients with multifocal PTC.ResultsAmong the paediatric and adolescent patients, over a median follow-up time of 49 months, 133 had multifocal disease and 205 had unifocal disease. Multifocal PTC patients exhibited stronger invasiveness in the form of extrathyroidal extension, tumour diameter, lymph node metastasis, and distant metastasis. Multifocality (OR 2.68; p = 0.017), lateral lymph node metastasis (OR 2.85; p = 0.036), and distant metastasis (OR 4.28; p = 0.010) were identified as independent predictive factors for the recurrence/persistence of disease. Comparing the paediatric/adolescent vs. adult multifocal patients, the former demonstrated greater tumour invasiveness. Lateral lymph node metastasis (OR 6.36; P = 0.012) and distant metastasis (OR 3.70; P = 0.027) were independent predictive factors for recurrence/persistence of disease in multifocal patients, while age was not (OR 0.95; P = 0.455).ConclusionTumour multifocality independently predicts persistent/recurrent disease in paediatric and adolescent PTC patients.

Application value of multi-gene mutation detection in the clinical management of pediatric papillary thyroid carcinoma: a preliminary exploration.

Thyroid cancer rarely occurs in children and adolescents. Molecular markers such as BRAF, RAS, and RET/PTC have been widely used in adult PTC. It is currently unclear whether these molecular markers have equivalent potential for application in pediatric patients. This study aims to explore the potential utility of a multi-gene conjoint analysis based on next-generation targeted sequencing for pediatric papillary thyroid carcinoma (PTC). The patients diagnosed with PTC (aged 18 years or younger) in the pediatrics department of Lishui District Hospital of Traditional Chinese Medicine were retrospectively screened. A targeted enrichment and sequencing analysis of 116 genes associated with thyroid cancer was performed on paraffin-embedded tumor tissues and paired paracancerous tissue of fifteen children (average age 14.60) and nine adults (average age 49.33) PTC patients. Demographic information, clinical indicators, ultrasonic imaging information and pathological data were collected. The Kendall correlation test was used to establish a correlation between molecular variations and clinical characteristics in pediatric patients. A sample of 15 pediatric PTCs revealed a detection rate of 73.33% (11/15) for driver gene mutations BRAF V600E and RET fusion. Compared to adult PTCs, the genetic mutation landscape of pediatric PTCs was more complex. Six mutant genes overlap between the two groups, and an additional seventeen unique mutant genes were identified only in pediatric PTCs. There was only one unique mutant gene in adult PTCs. The tumor diameter of pediatric PTCs tended to be less than 4cm (p<0.001), and the number of lymph node metastases was more than five (p<0.001). Mutations in specific genes unique to pediatric PTCs may contribute to the onset and progression of the disease by adversely affecting hormone synthesis, secretion, and action mechanisms, as well as the functioning of thyroid hormone signaling pathways. But, additional experiments are required to validate this hypothesis. BRAF V600E mutation and RET fusion are involved in the occurrence and development of adolescent PTC. For pediatric thyroid nodules that cannot be determined as benign or malignant by fine needle aspiration biopsy, multiple gene combination testing can provide a reference for personalized diagnosis and treatment by clinical physicians.

An individualized protein-based prognostic model to stratify pediatric patients with papillary thyroid carcinoma

Pediatric papillary thyroid carcinomas (PPTCs) exhibit high inter-tumor heterogeneity and currently lack widely adopted recurrence risk stratification criteria. Hence, we propose a machine learning-based objective method to individually predict their recurrence risk. We retrospectively collect and evaluate the clinical factors and proteomes of 83 pediatric benign (PB), 85 pediatric malignant (PM) and 66 adult malignant (AM) nodules, and quantify 10,426 proteins by mass spectrometry. We find 243 and 121 significantly dysregulated proteins from PM vs. PB and PM vs. AM, respectively. Function and pathway analyses show the enhanced activation of the inflammatory and immune system in PM patients compared with the others. Nineteen proteins are selected to predict recurrence using a machine learning model with an accuracy of 88.24%. Our study generates a protein-based personalized prognostic prediction model that can stratify PPTC patients into high- or low-recurrence risk groups, providing a reference for clinical decision-making and individualized treatment.

Pediatric Medullary Thyroid Carcinoma: Clinical Presentations and Long-Term Outcomes in 144 Patients Over 6 Decades.

Sporadic medullary thyroid carcinoma (sMTC) rarely occurs in childhood and no studies have specifically focused on this entity. To describe the clinical presentations and long-term outcomes of a large cohort of children and young adults with sMTC compared with hereditary MTC (hMTC). Retrospective study of 144 patients diagnosed with MTC between 1961 and 2019 at an age ≤ 21 years and evaluated at a tertiary referral center. In contrast to hMTC (n = 124/144, 86%), patients with sMTC (n = 20/144, 14%) are older (P < .0001), have larger tumors (P < .0001), a higher initial stage grouping (P = .001) and have more structural disease (P = .0045) and distant metastases (DM) (P = .00084) at last follow-up, but are not more likely to die from MTC (P = .42). Among 77 patients diagnosed clinically, not by family history (20/20 sMTC and 57/124 hMTC), there was no difference in the initial stage (P = .27), presence of DM at diagnosis (P = 1.0), disease status at last follow-up (P = .13), overall survival (P = .57), or disease-specific survival (P = .87). Of the 12 sMTC tumors that underwent somatic testing, 11 (91%) had an identifiable alteration: 10 RET gene alterations and 1 ALK fusion. sMTC is primarily a RET-driven disease that represents 14% of childhood-onset MTC in this cohort. Pediatric sMTC patients are older, present with clinical disease at a more advanced TNM classification, and have more persistent disease at last follow-up compared with hMTC, but these differences disappear when comparing those presenting clinically. Somatic molecular testing should be considered in sMTC patients who would benefit from systemic therapy.

Case Series of Surgical Treatment in Pediatric Thyroid Carcinoma in a Single Institution.

The prevalence of thyroid carcinoma (TC) among pediatric and adolescent populations is infrequent, yet its global occurrence is escalating. Insufficient information pertaining to TC in the pediatric populace of Indonesia presents issues in the identification and management of patients with this condition. The objective of this study is to provide a detailed account of a collection of pediatric TC cases that were recorded at a tertiary care center throughout an extended interval of eight years. The present study conducted a retrospective data analysis of ten patients who received a diagnosis of TC during the period spanning from 2014 to 2022. The present study focused on analyzing the clinical manifestation, diagnostic measures, treatment modalities, and immediate postoperative consequences of TC. All patients diagnosed with thyroid cancer exhibited the presence of neck lump. Nine patients underwent total thyroidectomy, while one patient underwent isthmolobectomy. The histopathologic evaluation confirmed the diagnosis of thyroid cancer in every individual. There were a significant number of patients, up to 50%, who encountered complications after undergoing surgery. Two patients reported the hoarseness of voice, whereas two other patients experienced the signs of hypocalcemia. Only one patient exhibited both these symptoms simultaneously. The implementation of total thyroidectomy as a surgical intervention in pediatric patients poses a notable challenge. Postoperative monitoring for potential complications following surgery for total thyroidectomy is imperative. The current study provides evidence that the utilization of total thyroidectomy in combination with lymph node dissection as indicated in patients with lymph node metastases in pediatric patients with thyroid carcinoma leads to a significant reduction in the probability of recurrence of lymph node enlargement. During the performance of surgical procedures, meticulous observation plays a key role in mitigating the risk of postoperative complications, such as hypoparathyroidism, hypocalcemia, and injury to the recurrent laryngeal nerve. Thus, it is imperative to conduct follow-up procedures for post-surgical interventions among pediatric patients diagnosed with TC.

THU681 Change In The Clinicopathological Characteristics And Outcomes Of Pediatric Thyroid Carcinoma In Korea: Focusing On Data From A Tertiary Hospital In Korea

Abstract Disclosure: J. Yoo: None. D. Leem: None. H. Ryu: None. H. Park: None. J. Kim: None. Y. Oh: None. T. Kim: None. S. Kim: None. J.H. Chung: None. Introduction: The incidence of thyroid carcinoma in adults in Korea increased until 2012, but has declined rapidly since then. However, the incidence of pediatric thyroid carcinoma increased until 2013, decreased slightly, and then increased again. Changes in the clinicopathological characteristics of pediatric thyroid carcinoma over time have not yet been elucidated. In this study, we aimed to evaluate the changes in the clinicopathological characteristics and outcomes of pediatric thyroid carcinoma from 1995 to 2022. Subjects and Methods: A total of 170 patients under the age of 19 years who were diagnosed with differentiated thyroid carcinoma at tertiary hospital in Seoul, Korea were enrolled. The patients were divided into four groups based on the year of initial surgery: 1995 – 2005 (n = 33), 2006 – 2010 (n = 46), 2011 – 2015 (n = 57), and 2016 – 2022 (n = 34). Results: The age at diagnosis, gender, primary tumor size, T stage, N stage, and M stage did not significantly differ among the four groups. Also, there was no significant difference over time in the route of detection (symptomatic vs. asymptomatic; P for trend = 0.519). The incidence of diffuse sclerosing variant of papillary thyroid carcinoma tended to increase over time (P=0.06). Surgical extent (P for trend = 0.001) and cumulative radioactive iodine (RAI) dose (P for trend &amp;lt; 0.001) significantly decreased over time. Nevertheless, the disease-free survival among the four groups did not change during the median follow-up 115.5 (62.3 – 155.3) months (log-rank P = 0.451). Conclusions: Although the overall incidence of pediatric thyroid carcinoma is gradually increasing in Korea, surgical extent and cumulative RAI dose significantly decreased and the clinicopathological characteristics and the disease-free survival did not change over time. Presentation: Thursday, June 15, 2023

RET fusion genes in pediatric and adult thyroid carcinomas: cohort characteristics and prognosis.

Thyroid cancer is associated with a broad range of different mutations, including RET (rearranged during transfection) fusion genes. The importance of characterizing RET fusion-positive tumors has recently increased due to the possibility of targeted treatment. The aim of this study was to identify RET fusion-positive thyroid tumors, correlate them with clinicopathological features, compare them with other mutated carcinomas, and evaluate long-term follow-up of patients. The cohort consisted of 1564 different thyroid tissue samples (including 1164 thyroid carcinoma samples) from pediatric and adult patients. Samples were analyzed for known driver mutations occurring in thyroid cancer. Negative samples were subjected to extensive RET fusion gene analyses using next-generation sequencing and real-time PCR. RET fusion genes were not detected in any low-risk neoplasm or benign thyroid tissue and were detected only in papillary thyroid carcinomas (PTCs), in 113/993 (11.4%) patients, three times more frequently in pediatric and adolescent patients (29.8%) than in adult patients (8.7%). A total of 20 types of RET fusions were identified. RET fusion-positive carcinomas were associated with aggressive tumor behavior, including high rates of lymph node (75.2%) and distant metastases (18.6%), significantly higher than in NTRK fusion, BRAF V600E and RAS-positive carcinomas. Local and distant metastases were also frequently found in patients with microcarcinomas positive for the RET fusions. 'True recurrences' occurred rarely (2.4%) and only in adult patients. The 2-, 5-, 10-year disease-specific survival rates were 99%, 96%, and 95%, respectively. RET fusion-positive carcinomas were associated with high invasiveness and metastatic activity, but probably due to intensive treatment with low patient mortality.

Single BRAFV600E mutation is not associated with aggressive biological behavior in adolescent and pediatric papillary thyroid carcinoma.

Papillary thyroid carcinoma is more likely to show aggressive biological behaviors in the majority of pediatric patients than in adult patients. The aim of this study was to investigate the mutation rate of the BRAFV600E gene in adolescents and children with papillary thyroid carcinoma and to analyze the association between BRAFV600E gene mutation and tumor-aggressive pathological factors. A total of 42 pediatric patients with papillary thyroid carcinoma who underwent thyroid surgery from 2017 to 2022 were studied retrospectively. Whether the BRAFV600E gene mutation in papillary thyroid carcinoma was related to aggressive biological behavior was analyzed. Among the 42 pediatric patients with papillary thyroid carcinoma, the median patient age was 15.71±2.51years (mean±SD) and the median tumor diameter was 24.95±12.29mm (mean±SD). Among all enrolled patients, the mutation rate of the BRAFV600E gene was 54.76% (23 of 42). There were 33 patients with classic papillary thyroid carcinoma, and 22 (66.67%) with classic subtypes were BRAFV600E positive. The BRAFV600E mutation was associated with lower distant metastasis (p=.013) and less Hashimoto's thyroiditis (p=.006). There was no significant difference in clinicopathological factors such as sex, age, tumor size, capsular invasion, multifocality, hypothyroidism, recurrence, lymph node metastasis, and extrathyroidal extension. The BRAFV600E mutation is not uncommon in pediatric papillary thyroid carcinoma but is not significantly associated with aggressive biological behavior. It is not possible to determine whether to adopt more active diagnosis and treatment measures on the basis of the mutation of a single BRAFV600E gene.

Is Multifocality a Predictor of Poor Outcome in Childhood and Adolescent Papillary Thyroid Carcinoma?

Total thyroidectomy in pediatric papillary thyroid carcinoma (PTC) is recommended in national guidelines because of the high incidence of multifocal disease (MFD). To determine the incidence of MFD in childhood and adolescent vs adult PTC and whether MFD is a predictor for poorer outcomes in childhood and adolescent PTC. We conducted an institutional review board-approved review of patients with PTC undergoing surgery (1986-2021) at Memorial Sloan Kettering Cancer Center. Clinical and pathological characteristics in patients with unifocal disease (UFD) and MFD were compared using Pearson's χ2 test. Survival outcomes were analyzed using the Kaplan-Meier method and log-rank test. Multivariate analysis assessed the impact of MFD on outcome. MFD was less common in childhood and adolescent patients with PTC (45%; 127/283) than in adults (54%; 3023/5564; P = .002). Childhood and adolescent patients with UFD and MFD had similar tumor stage and PTC subtype at presentation, with no significant difference in histopathologic features. Median follow-up was 68 months. There was no significant difference in 5-year recurrence-free probability and overall survival was 100% in both groups. There was no significant difference in 5-year contralateral lobe PTC-free probability between patients with UFD and MFD treated with lobectomy. Multivariate analysis showed MFD was not a predictor for recurrence. MFD was less common in childhood and adolescent patients with PTC than adults and was not a predictor of poor outcome on multivariate analysis, with excellent long-term outcomes in all patients with PTC. MFD does not appear to warrant completion thyroidectomy in childhood and adolescent patients selected for lobectomy.

Thyroid Nodules and Follicular Cell-Derived Thyroid Carcinomas in Children.

Although pediatric thyroid tumors have many similarities to those occurring in adults, significant differences are also recognized. For example, although thyroid nodules in children are much less common than in adults, a higher percentage is malignant. Moreover, while pediatric papillary thyroid carcinoma (PTC) is associated with more advanced disease, death due to disease in children and adolescents is very rare, even when distant metastases are present. Some subtypes of thyroid carcinoma, like diffuse sclerosing variant, are especially common in children and adolescents. Moreover, certain histologic findings, such as a tall cell morphology or increased mitotic activity, may not carry the same prognostic significance in children as in adults. Recent studies exploring the molecular underpinnings of pediatric thyroid carcinoma indicate that while driver alterations of thyroid tumorigenesis in children and adults are essentially the same, they occur at very different frequencies, with translocation-associated tumors (most commonly harboring RET and NTRK fusions) comprising a sizable and distinct group of pediatric PTC. DICER1 mutations, an infrequent mutation in adult thyroid tumors, are relatively frequent in pediatric encapsulated follicular-patterned thyroid tumors (with or without invasion or nuclear features of PTC). Additionally, tumor predisposition syndromes (most notably DICER1 syndrome and PTEN hamartoma tumor syndromes such as Cowden syndrome) should be considered in children with thyroid tumors, especially follicular-patterned thyroid tumors and poorly differentiated thyroid carcinoma. This review will explore the current state of knowledge of thyroid nodules and carcinomas in children and adolescents.

Factors associated with resistant disease in pediatric differentiated thyroid carcinomas

Purpose: Thyroid carcinoma is the most common carcinoma that originated from endocrine tissue in pediatric patients. Pediatric thyroid carcinomas are more prone to reveal lymph node or lung metastasis and recurrence. In this study, clinical, pathological and genetic features that may be related with resistant differentiated thyroid carcinoma in pediatric patients were investigated.&#x0D; Materials and Methodsː Thirty-nine pediatric patients who were 18 years old and below and were given 131I Radioactive Iodine (RAI) therapy were included to the study. 131I RAI therapy was planned in accordance with the American Thyroid Association (ATA) Guideline. Clinical and postoperative pathological features of patients were retrospectively determined and the patients who received single or multiple RAI therapy due to resistant disease compared statistically in terms of these features. Genetic analysis was also made in the patients who received multiple therapies. &#x0D; Resultsː Thirty-one patients were female and 8 patients were male. Thirty-four patients had papillary thyroid carcinoma, 4 had follicular thyroid carcinoma and 1 had mixed papillary-oncocytic carcinoma of patients. Eight patients received multiple I-131 RAI therapy cycles due to resistant disease. Any clinical, pathological feature and genetic mutation that would indicate resistant disease was not found in these patients. &#x0D; Conclusionː Although pediatric thyroid carcinoma is rarely seen, it has an increasing incidence in recent years. We were not able to reveal any feature related to resistant disease in our limited group. Clinical, pathological and genetic characteristics related to poor prognosis must be investigated with larger study groups.

Comments to the Section on Radioiodine Therapy for Differentiated Thyroid Cancer in Children of the European Thyroid Association Guidelines 2022

Children are not “miniature” adults! Most experts around the world recognize the differences between adult and pediatric thyroid cancer and emphasize the need for specific guidelines for the pediatric population. The American Thyroid Association (ATA) first developed separate Guidelines for the Treatment of Pediatric Thyroid Nodules and Carcinoma (hereafter, ATA Guidelines) in 2015 [1], which has not been revised to date. In September 2022, the European Thyroid Association (ETA) published its Guideline on the Management of Pediatric Thyroid Nodules and Differentiated Thyroid Carcinoma (hereafter, ETA Guidelines) [2]. The aim of our article, in anticipation of the revision of Russian clinical guidelines for the treatment of pediatric thyroid cancer, is to inform specialists in the field of radioiodine therapy (RIT) with the key recommendations of the ETA.

Development of a pediatric differentiated thyroid carcinoma registry within the EuRRECa project: rationale and protocol

Background Although differentiated thyroid carcinoma (DTC) is the most frequent endocrine pediatric cancer, it is rare in childhood and adolescence. While tumor persistence and recurrence are not uncommon, mortality remains extremely low. Complications of treatment are however reported in up to 48% of the survivors. Due to the rarity of the disease, current treatment guidelines are predominantly based on the results of small observational retrospective studies and extrapolations from results in adult patients. In order to develop more personalized treatment and follow-up strategies (aiming to reduce complication rates), there is an unmet need for uniform international prospective data collection and clinical trials. Methods and analysis The European pediatric thyroid carcinoma registry aims to collect clinical data for all patients ≤18 years of age with a confirmed diagnosis of DTC who have been diagnosed, assessed, or treated at a participating site. This registry will be a component of the wider European Registries for Rare Endocrine Conditions project which has close links to Endo-ERN, the European Reference Network for Rare Endocrine Conditions. A multidisciplinary expert working group was formed to develop a minimal dataset comprising information regarding demographic data, diagnosis, treatment, and outcome. We constructed an umbrella-type registry, with a detailed basic dataset. In the future, this may provide the opportunity for research teams to integrate clinical research questions. Ethics and dissemination Written informed consent will be obtained from all participants and/or their parents/guardians. Summaries and descriptive analyses of the registry will be disseminated via conference presentations and peer-reviewed publications.

Clinicopathologic and Prognostic Features of Pediatric Follicular Cell-Derived Thyroid Carcinomas: A Retrospective Study of 222 Patients

Clinical ThyroidologyVol. 35, No. 02 Pediatric ThyroidologyClinicopathologic and Prognostic Features of Pediatric Follicular Cell-Derived Thyroid Carcinomas: A Retrospective Study of 222 PatientsTheodora PappaTheodora Pappa Division of Endocrinology, Diabetes, and Hypertension, Department of Medicine, Brigham and Women's Hospital, Boston, MA, U.S.A. Head and Neck Oncology Cancer Treatment Center, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, U.S.A.Search for more papers by this authorPublished Online:8 Feb 2023https://doi.org/10.1089/ct.2023;35.50-53AboutSectionsView articleView Full TextPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail View articleFiguresReferencesRelatedDetails Volume 35Issue 02Feb 2023 Information© Copyright 2023, Mary Ann Liebert, Inc.To cite this article:Theodora Pappa.Clinicopathologic and Prognostic Features of Pediatric Follicular Cell-Derived Thyroid Carcinomas: A Retrospective Study of 222 Patients.Clinical Thyroidology.Feb 2023.50-53.http://doi.org/10.1089/ct.2023;35.50-53Published in Volume: 35 Issue 02: February 8, 2023PDF download

PAPILLARY THYROID CARCINOMA IN PEDIATRIC PATIENT- A CASE REPORT

Thyroid carcinoma is the most common endocrine malignant diseases, accounting for 06% of all pediatrics cancer. Papillar y thyroid carcinoma which accounts f or approximately 90% of pediatric thyroid cancer. However, pediatric papillary thyroid carcinoma is still a rare disease. Due to lack of clinical trials, treatment options remain controversial. Pediatric thyroid cancers typically present as neck masses with no associated symptoms and thus come to medical attention at widely varying stages of disease progression. In contrast to adult papillary thyroid carcinoma, pediatric papillary thyroid carcinoma tends to be more aggressive at presentation with higher incidence of multifocality, lymph node metastases and extracapsular extension

Molecular Landscape of Pediatric Thyroid Cancer: A Review.

Thyroid carcinomas (TC) are rare in the pediatric population; however, they constitute the most common endocrine malignancy. Despite some similarities with adult carcinomas, they have distinct clinical behavior and responses to therapy due to their unique pathology and molecular characteristics. The age cut-off used for defining the pediatric age group has been variable across different studies, and the universally accepted recommendations influence accurate interpretation of the available data. Moreover, factors such as radiation exposure and germline mutations have greater impact in children than in adults. Papillary TC is the most common and the most evaluated pediatric TC. Others, including follicular, poorly differentiated and medullary carcinomas, are rarer and have limited available literature. Most studies are from the West. Asian studies are primarily from Japan, with few from China, India, Saudi Arabia and Republic of Korea. This review provides a comprehensive account of the well-established and novel biomarkers in the field, including point mutations, fusions, miRNA, and thyroid differentiation genes. Familial and syndromic associations are also discussed. Current management guidelines for pediatric patients are largely derived from those for adults. An awareness of the molecular landscape is essential to acknowledge the uniqueness of these tumors and establish specific diagnostic and therapeutic guidelines.

Prepubertal Children with Papillary Thyroid Carcinoma Present with More Invasive Disease Than Adolescents and Young Adults.

Introduction: Pediatric papillary thyroid carcinomas (PTCs) are more invasive than adult PTCs. No large, contemporary cohort study has been conducted to determine whether younger children are at higher risk for advanced disease at presentation compared to adolescents. We aimed to describe pediatric PTC and contextualize its characteristics with a young adult comparison cohort. Methods: The National Cancer Database was interrogated for pediatric and young adult PTCs diagnosed between 2004 and 2017. Clinical variables were compared between prepubertal (≤10 years old), adolescent (11-18 years old), and young adult (19-39 years old) groups. Multivariable logistic regression modeling for independent predictors of metastases was conducted. A subanalysis of microcarcinomas (size ≤10 mm) was performed. Results: A total of 4860 pediatric (prepubertal n = 274, adolescents n = 4586) and 101,159 young adult patients were included. Prepubertal patients presented with more extensive burden of disease, including significantly larger primary tumors, higher prevalence of nodal and distant metastases, and increased frequency of features such as lymphovascular invasion, and extrathyroidal extension (ETE). Prepubertal age was an independent predictor of positive regional nodes (adjusted odds ratio [AOR] = 1.36 [95% confidence interval {CI} 1.01-1.84], p = 0.04) and distant metastatic disease (AOR = 3.12 [CI 1.96-4.96], p < 0.001). However, there was no difference in survival between groups (p = 0.32). Prepubertal age independently predicted lymph node metastases for microcarcinomas (AOR = 2.19 [CI 1.10-4.36], p = 0.03). Prepubertal (n = 41) versus adolescent (n = 937) patient age was associated with gross ETE (p = 0.004), even with primary tumors ≤1 cm in size. Conclusions: Patients aged <11 years old present with more advanced disease than adolescents, with a higher likelihood of nodal and distant metastatic disease at time of diagnosis, although survival is high. Prepubertal children undergo more extensive treatment, likely reflective of more invasive disease at the outset, even in the setting of a subcentimeter primary tumor.