Search Results (19)

Background: Thyroid disorders are significant endocrine conditions in pediatric populations, sometimes requiring surgical intervention. While surgical outcomes are well-documented, the broader impact on quality of life (QoL) remains insufficiently synthesized. This systematic review aimed to evaluate the effects of thyroid surgery on QoL in pediatric patients, focusing on physical, emotional, and social dimensions. Methods: Following the PRISMA guidelines, we searched PubMed, EMBASE, and SCOPUS from inception to January 2025. Studies reporting health-related QoL outcomes in pediatric patients undergoing thyroid surgery were included. Quality assessment was conducted using the Newcastle–Ottawa Scale. Data synthesis focused on emotional and psychological outcomes, social functioning, physical health, and surgical-approach-specific effects. Results: Five studies (ranging from 37 to 92 participants) met the inclusion criteria. Unilateral thyroidectomy was associated with better QoL outcomes compared to bilateral procedures, particularly in emotional and physical domains. Post-surgical anxiety significantly improved. However, male survivors reported higher levels of depression and reduced motivation. Employment status emerged as a significant factor influencing physical functioning scores. Thyroid cancer patients demonstrated better social functioning than peers with other cancers, yet they lagged behind healthy controls. Long-term follow-up highlighted ongoing challenges in physical functioning and fatigue. Conclusions: Thyroid surgery impacts multiple dimensions of QoL in pediatric patients, with variations depending on surgical approach and patient characteristics. These findings underscore the need for comprehensive post-operative care, including routine QoL assessments and tailored psychological support. Future research should aim to standardize assessment timing and develop targeted interventions for high-risk groups. Full article

The prevalence of goiter, thyroid nodules, and thyroid cancers in the pediatric population has increased. In some rare cases, local conditions such as juvenile desmoid-type fibromatosis (JDTF) can mimic specific thyroid pathology, complicating the diagnostic process. A 17-year-old obese adolescent girl was admitted to the Endocrinology Department with progressive swelling on the left side of the neck, persisting for approximately one year, recently accompanied by dysphonia and inspiratory dyspnea, and ultimately diagnosed as a unilateral nodular goiter associated with compressive phenomena. Despite her euthyroid status, the thyroid ultrasound identified a suspected, large, non-homogeneous, hypoechogenic nodule with calcifications in the left thyroid lobe (TI-RADS score of 4), confirmed by a cervical-region MRI. The biopsy specimens obtained through fine-needle aspiration were classified as Bethesda III (“atypia of undetermined significance” or “follicular lesion of undetermined significance”). Left thyroid lobe removal was performed by a specialized surgeon in thyroid pathology, with histopathological analysis revealing a diagnosis of JDTF in the thyroid gland. Post-surgery, the patient showed favorable progress without any relapse. Pediatric endocrinologists face challenges in diagnosing and managing thyroid nodules in children due to their higher malignancy potential. Familiarity with similar conditions, such as JDTF, is crucial in accurate diagnosis and appropriate pediatric management. Full article

Background/Objectives: Pediatric populations with well-differentiated thyroid cancer typically have favorable prognoses. However, the role of radioactive iodine (RAI) ablation in these patients remains uncertain. This investigation evaluates the national trends, therapeutic practices, and the impact of RAI on clinical outcomes. Methods: Patients aged 21 years or younger with differentiated thyroid cancer, identified from the SEER database between 2000 and 2019, were analyzed. We compared the treatment approaches and survival outcomes of patients who underwent RAI ablation with those who did not. Results: This retrospective cohort study encompassed 5318 pediatric patients, with 55.9% (n = 2973) who underwent RAI ablation. RAI utilization declined from 65% to 38.4% in 2019. Compared with those who did not undergo RAI, RAI patients presented with a larger tumor size (mean size: 27.7 vs. 20.4 mm), a higher T3/T4 stage (35.8% vs. 15.3%), nodal metastases (60.7% vs. 28.8%), and distant metastases (2.7% vs. 0.9%) (all p < 0.001). Despite this, RAI was not an independent predictor of recurrence, second malignancy, or mortality. The analysis showed no significant differences in long-term survival between the RAI and non-RAI groups (p > 0.05), with African American patients having an increased risk of mortality (HR = 3.81; p = 0.038). Cancer-directed surgery emerged as a protective factor (HR = 0.08; p = 0.018), while RAI treatment did not significantly affect mortality risk (p = 0.09). Conclusions: Excellent pediatric thyroid cancer outcomes were achieved regardless of RAI use. Further research should clarify appropriate RAI indications while addressing racial outcome inequities. Full article

Background: DICER1, a cancer predisposition syndrome (CPS), seems to escape timely diagnosis in pediatric patients. Case report 1: A 16-year-old female patient was referred to the endocrinology ward due to a large goiter. Her medical history indicated normal sexual maturation, with menarche occurring at 13.5 years. Over the past 2.5 years, she had developed pronounced androgenic symptoms, including a deepened male voice; facial, back, and neckline acne; hirsutism; and menstrual irregularities leading to secondary amenorrhea. A thyroid ultrasound identified a multinodular goiter (MNG) with cystic–solid lesions containing calcifications. An abdominal ultrasound identified a 5.7 × 6.9 cm solid mass in the right adnexal region, displacing the uterus to the left. Histopathological examination confirmed a Sertoli–Leydig cell tumor. The patient was subjected to a total thyroidectomy. Histopathology revealed benign follicular cell-derived neoplasms. Thyroid follicular nodular disease (TFND) was diagnosed bilaterally. DNA analysis using NGS, confirmed via the Sanger method, revealed a pathogenic heterozygotic variant c.2953C>T [p.Gln985*] in exon 18 of the DICER1 gene. Case report 2: A 12-year-old male patient was admitted to the pediatric surgery unit due to a 33 mL goiter. A month prior to his admission, the patient discovered a palpable nodule in his neck, accompanied by hoarseness. An ultrasound revealed MNG. Molecular analysis revealed a pathogenic heterozygotic variant c.2782C>T [p.Gln928*] in exon 17 of the DICER1 gene. Subsequently, a total thyroidectomy was performed, and histopathological examination revealed TFND bilaterally. Conclusions: Recent advances in genetic evaluation and in histological approaches indicate that MNG/TFND, although rare in the pediatric population, when accompanied by characteristic ultrasound and histopathological features, and by additional features such as androgenization, may warrant assessment also of the DICER1 gene within CPS molecular panel screening. Full article

Pediatric Differentiated Thyroid Cancer (pedDTC) is a rare pediatric malignancy with an increasing incidence over time. To date, there is a paucity of literature specifically addressing pedDTC within the context of Middle Eastern ethnicity. This retrospective study aimed to assess the risk-stratifying factors for overall survival (OS) and event-free survival (EFS) in pediatric DTC patients from Iraq and Jordan. The medical records of 81 patients from two tertiary cancer institutes were retrieved. Kaplan–Meier analysis was employed to investigate OS and EFS, and the Cox proportional hazards model was employed to estimate hazard ratios. All patients underwent surgery and radioactive iodine therapy, with a median age of 14 and an interquartile range of 12–15. Lymph node involvement was observed in 55% of cases, while distant metastases were present in 13.5%. After a median follow-up period of 68 months, the 10-year survival rate was determined to be 94%, while the 10-year EFS rate was 58%. EFS was negatively impacted by cervical lymph node metastases and early age of diagnosis (p ≤ 0.01, each). Therefore, pediatrics with initial cervical lymph node metastases and those diagnosed before puberty tend to experience poorer EFS, which may justify the need for more aggressive management plans. Full article

The association between Hashimoto’s thyroiditis (HT) and pediatric thyroid cancer is controversial. Most studies examining this connection have been based on adults, and larger studies in children are lacking. We performed a retrospective study of all sequential pediatric patients who underwent a thyroidectomy for a neoplasm at our institution over a twenty-year period in order to explore the link between HT and pediatric thyroid cancer. A total of 153 patients, median age 16.5 (interquartile range [IQR] 14.2–18.3) years, underwent thyroid surgery for a neoplasm. Patients were mainly female (80%) and White (84%). Median follow-up was 58.6 (IQR 20.7–105.4) months. Thirty-five (23%) patients had HT. Patients who underwent thyroid surgery and had HT were more likely to harbor a malignant neoplasm (p = 0.05); specifically, papillary thyroid carcinoma (PTC, p = 0.02). There was a difference in the distribution of HT among the subtypes of PTC (p = 0.03). Despite this, there was no difference in terms of survival between patients with/without HT. In conclusion, children with a thyroid malignancy, specifically, PTC, are more likely to have HT. The association between HT and pediatric PTC appears to be subtype-specific but does not seem to affect patient survival. Full article

Hungry bone syndrome (HBS), severe hypocalcemia following parathyroidectomy (PTX) due to rapid drop of PTH (parathormone) after a previous long term elevated concentration in primary (PHPT) or renal hyperparathyroidism (RHPT), impairs the outcome of underlying parathyroid disease. Objective: overview HBS following PTx according to a dual perspective: pre- and post-operative outcome in PHPT and RHPT. This is a case- and study-based narrative review. Inclusion criteria: key research words “hungry bone syndrome” and “parathyroidectomy”; PubMed access; in extenso articles; publication timeline from Inception to April 2023. Exclusion criteria: non-PTx-related HBS; hypoparathyroidism following PTx. We identified 120 original studies covering different levels of statistical evidence. We are not aware of a larger analysis on published cases concerning HBS (N = 14,349). PHPT: 14 studies (N = 1545 patients, maximum 425 participants per study), and 36 case reports (N = 37), a total of 1582 adults, aged between 20 and 72. Pediatric PHPT: 3 studies (N = 232, maximum of 182 participants per study), and 15 case reports (N = 19), a total of 251 patients, aged between 6 and 18. RHPT: 27 studies (N = 12,468 individuals, the largest cohort of 7171) and 25 case reports/series (N = 48), a total of 12,516 persons, aged between 23 and 74. HBS involves an early post-operatory (emergency) phase (EP) followed by a recovery phase (RP). EP is due to severe hypocalcemia with various clinical elements (<8.4 mg/dL) with non-low PTH (to be differentiated from hypoparathyroidism), starting with day 3 (1 to 7) with a 3-day duration (up to 30) requiring prompt intravenous calcium (Ca) intervention and vitamin D (VD) (mostly calcitriol) replacement. Hypophosphatemia and hypomagnesiemia may be found. RP: mildly/asymptomatic hypocalcemia controlled under oral Ca+VD for maximum 12 months (protracted HBS is up to 42 months). RHPT associates a higher risk of developing HBS as compared to PHPT. HBS prevalence varied from 15% to 25% up to 75–92% in RHPT, while in PHPT, mostly one out of five adults, respectively, one out of three children and teenagers might be affected (if any, depending on study). In PHPT, there were four clusters of HBS indicators. The first (mostly important) is represented by pre-operatory biochemistry and hormonal panel, especially, increased PTH and alkaline phosphatase (additional indicators were elevated blood urea nitrogen, and a high serum calcium). The second category is the clinical presentation: an older age for adults (yet, not all authors agree); particular skeleton involvement (level of case reports) such as brown tumors and osteitis fibrosa cystica; insufficient evidence for the patients with osteoporosis or those admitted for a parathyroid crisis. The third category involves parathyroid tumor features (increased weight and diameter; giant, atypical, carcinomas, some ectopic adenomas). The fourth category relates to the intra-operatory and early post-surgery management, meaning an associated thyroid surgery and, maybe, a prolonged PTx time (but this is still an open issue) increases the risk, as opposite to prompt recognition of HBS based on calcium (and PTH) assays and rapid intervention (specific interventional protocols are rather used in RHPT than in PHPT). Two important aspects are not clarified yet: the use of pre-operatory bisphosphonates and the role of 25-hydroxyitamin D assay as pointer of HBS. In RHPT, we mentioned three types of evidence. Firstly, risk factors for HBS with a solid level of statistical evidence: younger age at PTx, pre-operatory elevated bone alkaline phosphatase, and PTH, respectively, normal/low serum calcium. The second group includes active interventional (hospital-based) protocols that either reduce the rate or improve the severity of HBS, in addition to an adequate use of dialysis following PTx. The third category involves data with inconsistent evidence that might be the objective of future studies to a better understanding; for instance, longer pre-surgery dialysis duration, obesity, an elevated pre-operatory calcitonin, prior use of cinalcet, the co-presence of brown tumors, and osteitis fibrosa cystica as seen in PHPT. HBS remains a rare complication following PTx, yet extremely severe and with a certain level of predictability; thus, the importance of being adequately identified and managed. The pre-operatory spectrum of assessments is based on biochemistry and hormonal panel in addition to a specific (mostly severe) clinical presentation while the parathyroid tumor itself might provide useful insights as potential risk factors. Particularly in RHPT, prompt interventional protocols of electrolytes surveillance and replacement, despite not being yet a matter of a unified, HBS-specific guideline, prevent symptomatic hypocalcemia, reduce the hospitalization stay, and the re-admission rates. Full article

While some studies suggest that PTEN mutations correlate with a low-risk phenotype in pediatric thyroid nodules, the relationship between the mutation and malignancy in the adult populations is abstruse. This study investigated whether PTEN mutations result in thyroid malignancy, and whether these malignancies are aggressive. This multicenter study involved 316 patients who underwent preoperative molecular testing, and subsequent lobectomy or total thyroidectomy at two quaternary care hospitals. A four-year retrospective review was performed on the 16 charts of patients that opted for surgery following a positive PTEN mutation on molecular testing results from January 2018 to December 2021. Of the total 16 patients, 37.5% (n = 6) had malignant tumours, 18.75% (n = 3) had non-invasive follicular thyroid neoplasms with papillary-like nuclear features (NIFTPs), and 43.75% (n = 7) had benign disease. Aggressive features were detected in 33.33% of the malignant tumours. Malignant tumours were found to have a statistically significant higher allele frequency (AF). The aggressive nodules were all poorly differentiated thyroid carcinomas (PDTCs) with copy number alterations (CNAs) and the highest AFs. Full article

Horner’s syndrome (HS), caused by lesions of the 3-neuron oculosympathetic nerve pathway (ONP), includes the triad: blepharoptosis, miosis and anhidrosis (ipsilateral with ONP damage). Thyroid–related HS represents an unusual entity underling thyroid nodules/goiter/cancer–HS (T-HS), and post-thyroidectomy HS (Tx-HS). We aim to overview Tx-HS. This is a narrative review. We revised PubMed published, full-length, English papers from inception to November 2022. Additionally, we introduced data on post-thyroidectomy lymphocele/chylous leakage (Tx-L), and introduced a new pediatric case with both Tx-HS and Tx-L. Tx-HS: the level of statistical evidence varies from isolated case reports, studies analyzing the large panel of post-thyroidectomy complications reporting HS among the rarest side effects (as opposite to hypocalcemia), or different series of patients with HS due to various disorders, including T-HS/Tx-HS. Tx-HS is related to benign or malignant thyroid conditions, regardless the type of surgery. A pre-operatory rate of T-HS of 0.14%; a post-operatory rate of Tx-HS between 0.03% and 5% (mostly, 0.2%) are identified; a possible higher risk on endoscopic rather than open procedure is described. Incomplete HS forms, and pediatric onset are identified, too; the earliest identification is after 2 h since intervention. A progressive remission is expected in most cases within the first 2–6 months to one year. The management is mostly conservative; some used glucocorticoids and neurotrophic agents. One major pitfall is an additional contributor factor like a local compression due to post-operatory collections (hematoma, cysts, fistula, Tx-L) and their correction improves the outcome. The prognostic probably depends on the severity of cervical sympathetic chain (CSC) lesions: indirect, mild injury due to local compressive masses, intra-operatory damage of CSC like ischemia and stretching of CSC by the retractor associate HS recovery, while CSC section is irreversible. Other iatrogenic contributors to HS are: intra-operatory manipulation of parathyroid glands, thyroid microwave/radiofrequency ablation, and high-intensity focused ultrasound, and percutaneous ethanol injection into thyroid nodules. Tx-L, rarely reported (mostly <0.5%, except for a ratio of 8.3% in one study), correlates with extended surgery, especially lateral/central neck dissection, and the presence of congenitally—aberrant lymphatic duct; it is, also, described after endoscopic procedures and chest-breast approach; it starts within days after surgery. Typically low-fat diet (even fasting and parental nutrition) and tube drainage are useful (as part of conservative management); some used octreotide, local sealing solutions like hypertonic glucose, Viscum album extract, n-Butyl-2-cyanoacrylate. Re-intervention is required in severe cases due to the risk of lymphorrhoea and chylothorax. Early identification of Tx-HS and Tx-L improves the outcome. Some iatrogenic complications are inevitable and a multifactorial model of prediction is still required, also taking into consideration standardized operatory procedures, skillful intra-operatory manipulation, and close post-operatory follow-up of the patients, especially during modern era when thyroid surgery registered a massive progress allowing an early discharge of the patients. Full article

Intraoperative neuromonitoring (IONM) of the recurrent laryngeal nerve (RLN) has been shown in adults to minimize nerve palsy after thyroid surgery, but only few studies on its efficacy in a pediatric population have been reported. We conducted a retrospective study on patients operated for thyroid lesions from 2016 to 2022. The analyzed population was divided in two groups: patients treated from 2016 to 2020, when the identification of the RLN was performed without IONM (Group A); and patients treated since 2021, when IONM was implemented in every surgical procedure on the thyroid (Group B). Intraoperative Neurophysiological Monitoring was performed by using corticobulbar motor-evoked potentials and continuous electromyography. Twentyfive children underwent thyroid resection, 19 (76%) of which due to thyroid carcinoma. Each patient’s recurrent nerve was identified; IONM was used in 13 patients. In Group A, one temporary nerve palsy was identified postoperatively (8.3%), while in group B one nerve dysfunction occurred (7.7%). No statistically significant difference was found between the two groups in terms of post-operative RLN palsy. No surgical complication due to the use of IONM was reported. In children and teenagers, intraoperative neuromonitoring of the recurrent laryngeal nerve is a safe and accurate method, minimizing the risk of nerve damage. Full article

Prophylactic and early thyroidectomy in RET germline mutation carriers allows the removal of the thyroid before medullary thyroid carcinoma (MTC) develops, or while it is still confined to the gland. This study was aimed to assess the clinicopathological features in RET carriers according to the age at surgery and the long-term outcomes after prophylactic and early thyroidectomy. A retrospective analysis of 63 operated asymptomatic RET carriers diagnosed after familial genetic screening was performed. Twenty-one RET carriers were operated at pediatric (<18 yrs) and 42 at adult (≥18 yrs) age. Serum preoperative calcitonin levels were significantly lower in pediatric compared to adult patients (p = 0.04); moreover, adult RET carriers had a greater frequency of microMTC at pathology (p = 0.009). Permanent postoperative morbidity occurred in 9.5% of patients, without differences between the two groups. Biochemical postoperative cure was achieved in all patients. At a median follow-up of 14 years, all C-cell hyperplasia patients are disease-free; conversely, biochemical, and structural recurrence of disease occurred in three adults and one pediatric patient with microMTC. The independent predictive factors of MTC were the age at surgery, the preoperative calcitonin level and the RET mutational risk profile (p < 0.02). In conclusion, prophylactic and early thyroidectomy are safe and effective procedures in achieving definitive cure in most RET carriers. However, since recurrences may occur at long-term in case of microMTC, thyroidectomy should be possibly performed earlier to prevent microMTC development. Full article

Introduction: Brain tumors are the second most frequent type of all pediatric malignancies. Depending on their localization, patients with brain tumors may present neurological or ophthalmological symptoms, but also weight anomalies and endocrine disorders ranging from growth hormone deficiency, anomalies of puberty, diabetes insipidus to panhypopituitarism. Immediately at diagnosis, all patients with brain tumors require a complete assessment of the hypothalamic–pituitary function in order to address eventual endocrine disorders. Moreover, children and adolescents undergoing brain surgery must receive peri- and postoperative hydrocortisone stress therapy. Post-operative disorders of water homeostasis are frequent, ranging from transient diabetes insipidus, as well as syndrome of inappropriate antidiuretic hormone secretion to persistent diabetes insipidus. Late endocrine disorders may result from surgery near or within the hypothalamic–pituitary region. Pituitary deficits are frequent after radiotherapy, especially growth hormone deficiency. Thyroid nodules or secondary thyroid cancers may arise years after radiotherapy. Gonadal dysfunction is frequent after chemotherapy especially with alkylating agents. Conclusion: Early detection and treatment of specific endocrine disorders at diagnosis, perioperatively, and during long-term follow-up result in improved general and metabolic health and quality of life. Full article

This retrospective study aimed to describe, firstly, characteristics and outcomes of the intraoperative neural monitoring technology in the pediatric population, and secondarily the recurrent laryngeal nerve complication rate. Thirty-seven patients (age <18 years) operated on from 2015 to 2021 by conventional open thyroid surgery were included. Twenty-four (64.9%) total thyroidectomies and 13 (35.1%) lobectomies were performed. Seven central and six lateral lymph node dissections completed 13 bilateral procedures. Histology showed malignancy in 45.9% of the cases. The differences between the electromyographic profiles of endotracheal tubes or electrodes for continuous monitoring were not statistically significant. In our series of young patients, both adhesive (even in 4- or 5-year-olds) and embedded endotracheal tubes were used, while in patients 3 years old or younger, the use of a more invasive detection method with transcartilage placement recording electrodes was required. Overall, out of 61 total at-risk nerves, 5 (8.2%) recurrent laryngeal nerves were injured with consequent intraoperative loss of the signal; however, all these lesions were transient, restoring their normal functionality within 4 months from surgical procedure. To our knowledge, this is the first study of intraoperative neural monitoring management in a cohort of Italian pediatric patients. Full article

Pediatric thyroid cancer is rare, but increasing in annual incidence. Differentiated thyroid cancer in pediatric patients is treated surgically. Pediatric thyroidectomies are performed by general surgeons, otolaryngologists, general pediatric surgeons, and pediatric otolaryngologists. In a comprehensive literature review, we discuss the evidence supporting the importance of surgeon subspecialty and surgeon volume on outcomes for pediatric thyroid cancer patients. Pediatric general surgeons and pediatric otolaryngologists perform most pediatric thyroidectomies. Certain subpopulations specifically benefit from a combined approach of a pediatric surgeon and a high-volume thyroid surgeon. The correlation between high-volume surgeons and lower complication rates in adult thyroid surgery applies to the pediatric population; however, the definition of high-volume for pediatric thyroidectomies requires further investigation. The development of dedicated pediatric thyroid malignancy centers and multidisciplinary or dual-surgeon approaches are advantageous. Full article

(1) Background: Pediatric thyroidectomy is characterized by considerable space constraints, thinner nerves, a large thymus, and enlarged neck nodes, compromising surgical exposure. Given these challenges, risk-reduction surgery is of paramount importance in children, and even more so in pediatric thyroid oncology. (2) Methods: Children aged ≤18 years who underwent thyroidectomy with or without central node dissection for suspected or proven thyroid cancer were evaluated regarding suitability of intermittent vs. continuous intraoperative neuromonitoring (IONM) for prevention of postoperative vocal cord palsy. (3) Results: There were 258 children for analysis, 170 girls and 88 boys, with 486 recurrent laryngeal nerves at risk (NAR). Altogether, loss of signal occurred in 2.9% (14 NAR), resulting in six early postoperative vocal cord palsies, one of which became permanent. Loss of signal (3.5 vs. 0%), early (1.5 vs. 0%), and permanent (0.3 vs. 0%) postoperative vocal cord palsies occurred exclusively with intermittent IONM. With continuous nerve stimulation, sensitivity, specificity, positive and negative predictive values, and accuracy reached 100% for prediction of early and permanent postoperative vocal cord palsy. With intermittent nerve stimulation, sensitivity, specificity, positive and negative predictive values, and accuracy were consistently lower for prediction of early and permanent postoperative vocal cord palsy, ranging from 78.6% to 99.8%, and much lower (54.2–57.9%) for sensitivity. (4) Conclusions: Within the limitations of the study, continuous IONM, which is feasible in children ≥3 years, was superior to intermittent IONM in preventing early and permanent postoperative vocal cord palsy. Full article