Thyroid Carcinoma In Children Research Articles

Population characteristics of thyroid carcinoma in children and adolescents living in conditions of iodine deficiency in the republic of uzbekistan

Population characteristics of thyroid carcinoma in children and adolescents living in conditions of iodine deficiency in the republic of uzbekistan

Risk factors for recurrence of differentiated thyroid carcinoma in children and adolescents: A retrospective cohort study.

This study aimed to provide a recent clinical evaluation of the outcome of treatment and the predictors of recurrence for Chinese children and adolescents with differentiated thyroid carcinoma (DTC). This is a retrospective cohort study at the Yunnan Cancer Hospital from May 2002 to August 2021. We analyzed several risk factors related to the recurrence of DTC in children and adolescents. The Chi-square test, Kaplan-Meier log-rank tests, and Cox regression analysis were used in the statistical analysis. A P-value <.05 was considered statistically significant. A total of 103 patients were enrolled, including 68 girls (66.0%) and 35 boys (34.0%) with a median age of 18 years (range: 7-20 years). All enrolled patients received standard treatment. Children (≤14years) tended to have multifocality and higher levels of thyroid imaging reporting and data system, higher pN stage, and higher American Thyroid Association (ATA) pediatric risk compared with adolescents (P < .05). The chief complaints and clinical treatment differed between children and adolescents. During a follow-up of 6 to 239 months (average 74.7 months, median 59 months), all patients survived, but recurrence occurred in 22 patients (22.4%). The disease-free survival rates at 1, 2, 5, and 10 years were 91.2%, 78.4%, 77.1%, and 77.1%, respectively. Univariate Cox regression and log-rank tests showed that positive preoperative thyroglobulin level, bilaterality, extrathyroidal extension, high pT/pN/pM stage, and high ATA pediatric risk were the risk factors for DTC recurrence in children and adolescents. Multivariate Cox regression found that extrathyroidal extension and ATA pediatric risk were independent risk factors for the recurrence of DTC in children and adolescents. Additionally, among the 38 cases with cN0 stage, one who had bilateral, and multifocal thyroid nodules experienced recurrence, while the remaining 37 cases with cN0 stage had no recurrence. In conclusion, compared with adolescents, children present with more highly malignant disease and are more prone to metastasis. The significant risk factors associated with the recurrence of DTC in children and adolescents were positive preoperative thyroglobulin level, bilaterality, high pT/pN/pM stage, extrathyroidal extension, and high ATA pediatric risk, with the latter 2 being independent risk factors. The surgical approach for cN0 patients should be personalized taking into account invasive features.

Relationship between sonographic features, clinicopathological characteristics and lymph node metastasis of papillary thyroid carcinoma in children

Objective: This study aimed to evaluate the relationship between sonographic features, clinicopathological characteristics and lymph node metastasis (LNM) of papillary thyroid carcinoma (PTC) in children ( 18 years). S ≤ ubject and method: A total of 46 pediatric patients who were histopathologically diagnosed with PTC from January 2015 to March 2022 were included in this study. The preoperative ultrasonography, histological, and LNM features after surgery were retrospectively analyzed. Result: PTC in children accounted for 0.6% of all PTC, with a higher rate of females (67.4%), aged 15-18 years was 71.7%. The tumors weremostly classic type (87.0%), tumor size &gt; 10mm (76.1%), with high rates of central and lateral LNM (69.6% and 45.7%, respectively). Central LNM was significantly related to extrathyroidal extension (ETE). A significant relationship was observed between lateral LNM and tumor size, multifocality, ETE, intrathyroidal spread, microcalcification, and abnormal lymph nodes on ultrasound. Conclusion: Central and lateral LNM in pediatric PTC were common (69.6% and 45.7%, respectively). Several sonographic and clinicopathologic features had a significant relationship with LNM, especially lateral LNM. Patients with risk factors should undergo both clinical and neck ultrasound examinations in order to find evidence of LNM.

Primary presentation and clinical course of pediatric and adolescent patients with differentiated thyroid carcinoma after radioiodine therapy.

Differentiated thyroid carcinoma (DTC) in childhood and during adolescence is extremely rare. Pediatric DTC commonly presents with advanced disease at diagnosis including a high prevalence of cervical lymph node metastases and pulmonary metastases. Studies in children with DTC are limited. Therefore, we aimed to evaluate the initial presentation, effectiveness of radioiodine therapy (RIT), and long-term outcome of prepubertal in comparison to pubertal/postpubertal patients. Eighty-five pediatric and young patients aged 6.4 to 21.9 years with histopathologically confirmed DTC were retrospectively included. They all underwent total thyroidectomy followed by RIT. Initial presentation and outcome of prepubertal and pubertal/postpubertal patients were compared 1 year after RIT, during follow-up, and at the last visit of follow-up. Prepubertal patients presented with significantly higher T and M stages. One year after RIT, 42/81 (52%) patients still presented with evidence of disease (ED). During follow-up of a median of 7.9 years, prepubertal patients were less often in complete remission (58% vs. 82% in pubertal patients). At the last visit of follow-up, 19/80 (24%) patients still had ED without statistical differences between the two groups (42% prepubertal vs. 18% pubertal/postpubertal, p-value 0.06). None of our patients died disease-related over the observed period. Prepubertal children with DTC presented with a more advanced tumor stage at the initial presentation. During follow-up, they present more often with ED. However, at the end of our study, we did not observe statistically relevant differences in patient outcomes between the prepubertal and pubertal/postpubertal groups.

Molecular features of thyroid cancer in children

Background. Malignant neoplasms of the thyroid gland are the most common endocrine malignant neoplasms in the children’s age group. Unlike adults, who most often have BRAF and RAS mutations, children have chromosomal displacements. The relevance is explained by the lack of comprehensive studies on thyroid cancer in children in the Russian-language literature.The aim of the study is — to summarize and systematize the literature data on the molecular features of thyroid cancer in children.Materials and methods. The authors conducted a search for publications in the PubMed electronic database using the following algorithm: Molecular AND Thyroid cancer AND pediatrics. We found 451 publications, and after reviewing the annotations, 111 studies describing the molecular features of thyroid cancer in the pediatric population were included in the review. Publications were excluded due to duplication and inconsistency with the subject of this study.Results. Instead of point mutations of BRAF and RAS, characteristic of adults, regardless of radiation status, chromosomal displacements are more common in children. Re-arrangement of RET/PTC genes is the most common, followed by BRAF fusions. Mutations of the TERT promoter, which are markers of the aggressive course of the disease in adults, are rare in children. DICER1 mutations appear to play a key role in pediatric cases of follicular and papillary thyroid cancer. Medullary thyroid cancer (MTC) in children requires the exclusion of multiple endocrine neoplasia syndrome (MEN type 2). Tumors originating from follicular cells other than MTC can rarely be familial. Literature data on the role of microRNA as a biomarker in thyroid carcinomas in children is currently limited.

Status in clinical and molecular characteristics of differentiated thyroid carcinoma in children and adolescents

The epidemiological, clinical, and molecular characteristics of differentiated thyroid carcinoma (DTC) in children and adolescents are different from those in adults. The incidence of DTC in children and adolescents is very low, with high rates of lymph node metastasis, extra-thyroidal extension and recurrence, but mortality is lower than that in adults. Younger children with DTC tend to show a higher rate of recurrence, more lymph node metastases, and more extra-thyroidal extension. Furthermore, studies on molecular characteristics suggest that the diversity of gene mutations causes the clinical manifestations of DTC in children and adolescents that are different from those in adults. The incidence of gene fusion is significantly higher than in adults, while the incidence of point mutations is lower than in adults, which may be closely related to clinicopathological characteristics such as high tumor aggressiveness and poor prognosis.

Analysis of clinicopathological characteristics and prognosis of papillary thyroid carcinoma in children and adolescents at different ages

Objectives: To examine the age cut-off point for poor clinicopathological characteristics and prognosis of differentiated thyroid carcinoma (DTC) in children and adolescents. Methods: The clinicopathological features of 74 patients with DTC aged 18 years and younger who underwent surgery in the Department of Thyroid Surgery, the First Hospital of China Medical University from January 2011 to December 2020 were retrospectively analyzed. There were 20 males and 54 females, aged (M(IQR)) 16 (4) years (range: 8 to 18 years). Firstly, the cut-off point of age affecting prognosis was determined according to the receiver operator characteristic curve, the patients included in the study were grouped according to the age cut-off, and then the univariate and multivariate analysis for prognostic factors were performed using the Cox risk proportional regression model. The Kaplan-Meier survival curve was used to verify the factors affecting the prognosis and analyzed the possible mechanisms by bioinformatics. Results: The cut-off value of age was 15 years. Age ≤15 years was the only prognostic factor for recurrence (HR=4.427, 95%CI: 1.236 to 15.859, P=0.022). The number of metastatic cervical lymph nodes was higher in patients aged ≤15 years, and the 10-year recurrence-free survival rate was much lower than in patients aged >15 years (50.4% vs. 84.1%, P=0.018). Gene enrichment analysis and differential gene identification showed that differential genes between ≤15 years old group and>15 years old group were closely related to the transforming growth factor-β signaling pathway and some metabolism-related signaling pathways. Conclusions: Age ≤15 years is the only prognostic factor for the prognosis of DTC in children and adolescents. Age ≤15 years had more cervical lymph node metastases and a worse prognosis. For younger DTC patients, more active treatment and a stricter postoperative management strategy should be adopted.

Ultrasonographic, clinical, and pathological features of papillary thyroid carcinoma in children and adolescents with or without Hashimoto's thyroiditis.

To compare the ultrasonographic, clinical, and pathological features of children and adolescents with papillary thyroid carcinoma (PTC) with and without Hashimoto's thyroiditis (HT). A total of 52 children and adolescent patients surgically diagnosed with PTC between 2017 and 2022 were included; 14 children and adolescent patients with PTC were diagnosed with HT via pathological examination. The preoperative ultrasonographic, postoperative histological, and molecular and clinical characteristics were retrospectively analyzed. The prevalence rate of PTC in patients with HT was 27%. Papillary thyroid microcarcinomas were found in 11 of 38 patients without HT, but none in patients with HT (p = 0.023). Extrathyroidal extension, capsular invasion, and lymph node metastases were more frequent in patients with PTC and HT than in patients with PTC alone (p < 0.05 for both). The ultrasonographic features of nodule composition, echogenicity, shape, margin, Thyroid Imaging Reporting and Data System categories, and total points were similar. The patterns of echogenic foci were more prominent in the nodules of patients with HT than in those of patients without HT (p = 0.016). The frequency of papillary thyroid microcarcinomas in patients with PTC and HT was less, whereas that of extrathyroidal extension, capsular invasion, and lymph node metastasis was significantly higher in patients with PTC and HT than in those with PTC alone. The patterns of echogenic foci on ultrasonography may represent a risk for PTC.

Inaccurate Examination of Neck Lump as Regional Metastasis of Papillary Thyroid Carcinoma in Children: A Case Report

Background: Malignancy of the thyroid in children was a rare finding and the most pathological finding was papillary thyroid carcinoma. Thyroid carcinoma in children can present with neck lumps or enlarged neck lymph nodes. However, enlarged neck lymph nodes can be a mark of disruption immune system or lymph nodes’ malignancy or metastases from another organ. Case: An eight-year-old girl came to surgical oncology policlinic Prof. Dr. I.G.N.G. Ngoerah general hospital with a tumor on her right neck below the ear lobe and a scar from surgery on her right neck. Previously, she was done surgery on the right neck lump without cytomorphological examination at a regional hospital and it was a papillary thyroid carcinoma. She was referred to Prof. Dr. I.G.N.G. Ngoerah general hospital. She had done total thyroidectomy and right radical neck dissection with sternocleidomastoid muscle removal. The pathological examination was papillary thyroid carcinoma with four metastasis lymph nodes from nine lymph nodes. She had done radioactive iodine and take levothyroxine daily for hormonal suppression. After radioactive iodine therapy, she did not have any malignancy in the surgical field. Conclusion: In children, enlarged neck lymph nodes must be considered as a metastasis lesion of the thyroid. Although thyroid carcinoma is rare in children, we can do fine needle aspiration biopsy to evaluate the origin of enlargement. It can reduce the increase in cancer staging and the risk of metastasis.

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.

Clinicopathologic Characteristics of Differentiated Thyroid Carcinoma in Children and Adolescents

Clinicopathologic Characteristics of Differentiated Thyroid Carcinoma in Children and Adolescents

Personalized immunohistochemical particulars of thyroid cancer in children

The idea of the paper from the state scientific program under the name „Modern personalized surgery in the diagnosis and complex treatment of tumors in children”. In the first stage, the executors conducted statistical research on the incidence of thyroid cancer in children. During 40 years from 18549 cases of nodular thyroid pathology, 1078 cases were detected at children: 105 - (9.7%) with cancer, adenomas 534 (49.5%), toxic nodular goiter 213 (19.7%) , struma Hashimoto 226 (21%),: ratio girl : boys 6: 1. The most affected age is 9-16 years. The highest morbidity is in children aged from 12 to 15 years.The morphological structure of thyroid cancer in children (105 patients) was as follows: first - papillary cancer - 68.6%, the second follicular cancer 23.8%, the third medullary cancer - 5.7% and the fourth anaplastic cancer - 1.9%.The priority goal of the authors was personalized medicine, which involves selecting an appropriate treatment for the patient, depending on the volume of the primary tumor, the morphological type and the biological evolution of the tumor. In the diagnostic plan for the assessment of tumor aggression, the authors will propose immunohistochemical research in assessing the angioinvasive aggression of thyroid carcinomas in children. It is proposed to investigate the expression of thyroiglobulin, keratin and vimentin, peroxidase. Peroxidase expression was studied in 22 children. Thyroglobulin expression was studied in 31 children. And the expression of keratin and vimentin was studied in 16 children [2, 4-6].

Relationship Between Thyroid Autoantibodies and Recurrence of Papillary Thyroid Carcinoma in Children and Adolescents.

BackgroundNumerous studies reported connection between papillary thyroid carcinoma (PTC) and thyroid autoantibody in adults, but few of them have investigated whether there is a similar link in children and adolescents. The purpose of this research was to explore the relationship between clinicopathological features, prognosis and preoperative thyroid peroxidase antibody (TPOAb) as well as thyroglobulin antibody (TgAb) status in children and adolescents with PTC.MethodsThis study retrospectively reviewed 179 patients of PTC who underwent a thyroidectomy from January 2000 to June 2021 at Tianjin Medical University Cancer Hospital. We compared preoperative TgAb and TPOAb status with the clinicopathological features and prognosis of children and adolescents with PTC in different age groups.ResultsPatients with positive preoperative TPOAb and TgAb had lower recurrence rate in the younger group (P = 0.006, 0.047, respectively). Patients with positive TPOAb preoperatively had normal level of preoperative Tg and less cervical LNM than patients with negative TPOAb in children and adolescents (P < 0.05). Positive TPOAb preoperatively of PTC patients had a longer median DFS (113.4 months) than negative TPOAb (64.9 months) (P = 0.009, log-rank). Univariate analyses showed age, maximal tumor size, T stage, multifocality, lateral LNM and N staging were predictors for cancer recurrence in children and adolescents (P<0.05). Cox regression analysis found younger age (HR 0.224, P < 0.001), lateral LNM (HR 0.137, P = 0.010), N stage (HR 30.356, P < 0.001) were independent risk factors for recurrence.ConclusionsOur study found that presence of preoperative TPOAb and TgAb could serve as novel prognostic factors for predicting recurrence of PTC in children.

Predictive Factors for Recurrence of Papillary Thyroid Carcinoma in Children and Adolescents.

BackgroundThe incidence of papillary thyroid carcinoma (PTC) in children and adolescents has increased, but the data on long-term outcomes are limited. There are few literatures on the clinicopathological characteristics and prognosis of PTC in children and adolescents in China. Therefore, it is necessary to identify clinicopathological features to precisely predict clinical prognosis and to help choose the optimal method and perform the best therapeutic regimen.MethodsThis study was a retrospective analysis of patients undergoing thyroidectomy at Tianjin Medical University Cancer Institute and Hospital. We analyzed the factors related to the clinicopathological features and prognosis of PTC in children and adolescents.ResultsA total of 95 juvenile PTC patients who underwent thyroidectomy were enrolled. Our research found that patients with younger age (<14 years) were predominantly multifocal and have positive preoperative thyroglobulin (Tg) and higher recurrence rate, and their number of lymph node metastases (LNMs) was more than that of the older group (14–18 years). Maximal tumor size >2 cm, T stage, and multifocality were the risk factors for LNM and the number of LNM (p < 0.05). Multivariate analysis displayed the number of central LNM as the independent risk factor for lateral LNM, and multifocality was the independent risk factor for the number of central and lateral LNM. Younger age at diagnosis, positive preoperative thyroid-stimulating hormone (TSH), maximal tumor size >2 cm, lateral LNM, number of LNM, N staging, and American Thyroid Association (ATA) pediatric risk were related to poor prognosis in PTC patients (p < 0.05). Cox regression analysis found that younger age at diagnosis and positive preoperative TSH were independent risk factors for recurrence of PTC in children and adolescents.ConclusionsOur study showed that the clinicopathological characteristics of younger age compared with older age were as follows: highly aggressive, prone to metastases, and higher recurrence rate. In our opinion, patients with characteristics such as younger age at diagnosis, positive preoperative TSH, maximal tumor size >2 cm, lateral LNM, and number of LNM >5 may be considered for prophylactic or therapeutic dissection of additional metastatic LNs by high-volume surgeons to prevent and reduce the recurrence rate of patients during long-term follow-up.

Risk factors for lateral lymph node metastasis of papillary thyroid carcinoma in children

IntroductionLateral cervical lymph node metastases (LNM) for pediatric patients with papillary thyroid cancer (PTC) is a poor prognostic factor. We aimed to identify risk factors for lateral LNM. MethodsThis retrospective study had included 48 pediatric patients with papillary thyroid cancer underwent total thyroidectomy and central cervical lymphadenectomy at K hospital from 2016 to 2020. ResultsThe number of patients in each T stage was as follows: 24 (50.0%) in stage 1, 9 (18.7%) in Stage 2, 8 (16.7%) in Stage 3, and 7 (14.6%) in Stage 4. Most of the patients had LNM with N1a and N1b rates of 83.3% and 62.5%, respectively. Lung metastases were observed at presentation in three patients (6.3%). Univariate analysis revealed that age (p = 0.021), male (p = 0.011), tumor size > 10 mm (p = 0.002), multifocality (p < 0.001), extrathyroidal extension (p = 0.001) and central LNM (p < 0.001) were factors that increase the risk of metastasis to lateral LNM. ConclusionApproximately 62.5% of pediatric patients with PTC exhibited lateral LNM at the time of diagnosis. Our study confirmed that multifocality, maximum tumor diameter, extrathyroidal extension and central LNM were independent risk factors for lateral LNM in pediatric PTC. Level of EvidenceLevel IV.

Childhood thyroid carcinoma — single-centre experience

Papillary thyroid carcinoma (PTC) is the most common histological form of thyroid cancer in children and is characterized by a very good prognosis. However lymph node and lung metastases in PTC occur more frequently in pediatric patients than in adults. Ultrasound together with fine needle aspiration biopsy (FNAB) are the gold standard in the diagnosis of thyroid nodules in children. A new valuable diagnostic method became also elastography. The gold treatment standard remains thyroidectomy. In young patients with lymph node and/or distant metastases, there is abundant evidence to show that disease-free survival is improved by radioactive iodine (RAI) therapy. We present three patients diagnosed with PTC in Department of Paediatrics, Endocrinology, Diabetology with Cardiology Division, Medical University of Bialystok, Poland treated with the cooperation of the Department of Nuclear Medicine and Endocrine Oncology at M. Sklodowska-Curie National Research Institute in Gliwice, Poland.

Clinicopathological characteristics and prognostic analysis of medullary thyroid carcinoma in children and adolescents

Clinicopathological characteristics and prognostic analysis of medullary thyroid carcinoma in children and adolescents

Medullary thyroid carcinoma in children: current state of the art and future perspectives.

Medullary thyroid carcinoma (MTC) is a distinct type of malignant thyroid tumor in cell origin, biological behavior, and natural history. It accounts for 1.6% of all thyroid cancers and presents either sporadically or as a hereditary disease, the latter occurring as a part of multiple endocrine neoplasia (MEN) 2A and MEN2B syndromes or as a familial MTC disease with no other manifestations. The gene responsible for the hereditary form is the rearranged during transfection (RET) gene, a proto-oncogene located to human chromosome 10. Most pediatric MTC cases have been discovered after genetic testing investigations, leading to the concept of prophylactic surgery in presymptomatic patients. Therefore, the genetic status of the child, along with serum calcitonin levels and ultrasonographic findings, determine the appropriate age for prophylactic surgical intervention. Nevertheless, a diagnosis at an early stage of MTC warrants total thyroidectomy and central lymph node dissection with the addition of lateral/contralateral lymph node dissection depending on the tumor size, ultrasonographic evidence of neck disease, or calcitonin levels. Conversely, locally advanced/unresectable or metastatic MTC is primarily treated with multikinase inhibitors, while more specific RET inhibitors are being tested in clinical trials with promising results.

Calcitonin and complementary biomarkers in the diagnosis of hereditary medullary thyroid carcinoma in children and adolescents.

Medullary thyroid carcinoma (MTC) is a rare malignancy that is effectively curable by surgery. Unlike in adults, hereditary MTC has a predominant role in children. A fast and safe diagnosis is important to assure the good prognosis for the patients. A major cornerstone is the assessment of biomarkers, but the interpretation must respect their pre-, post- and analytical features. Especially calcitonin (Ctn) is a challenging biomarker in daily laboratory diagnostics. However, Ctn is of particular relevance for the diagnostic in MTC. The American Thyroid Association recommends thyroidectomy if the upper reference range of Ctn is exceeded. Interestingly, age-dependent reference ranges for children and adolescents have become available only recently for Ctn assays. With this review, we aim to highlight the importance of a timely diagnosis of MTC in children and adolescents. Recent developments in pediatric biochemical diagnostics of MTC were summarized. This includes guidance on interpretation of RET, Ctn, procalcitonin, carcinoembryonic antigen, carbohydrate antigen 19-9, and chromogranin A. Currently, Ctn is the most investigated biomarker in the diagnosis of MTC in children and adolescents. Other biomarkers as PCT suggest complementary evidence about pediatric MTC but their interpretation based largely on adult's data. A successful treatment of MTC requires, besides results of biomarkers, information about medical history, RET gene analysis and recent guideline knowledge. More research is required to validate complementary biomarkers of Ctn in children. Additionally, the effect of different confounder on pediatric Ctn levels has to be further clarified.