J Clin Endocrinol Metab
September 2025
Context: Pediatric differentiated thyroid carcinoma (DTC) often presents with advanced disease but generally has excellent long-term survival. However, recurrence or failure to achieve remission remains relatively frequent, underscoring the need for improved early risk stratification.
Objective: To develop and evaluate an interpretable machine learning model for predicting recurrence or non-remission in pediatric DTC using routine clinical and biochemical variables.
Background: Pediatric differentiated thyroid carcinoma (pedDTC) is rare but increasingly prevalent, requiring multidisciplinary care to ensure optimal outcomes. In 2021, the pediatric national reference program of the German Malignant Endocrine Tumor (MET) registry was established to standardize the management of pedDTC, with a particular focus on radioactive iodine (RAI) use and minimizing treatment variability.
Methods: This study evaluated the program's first 3.
Objective: To evaluate clinical characteristics and survival outcomes of paediatric differentiated thyroid carcinoma (paedDTC) in patients with and without autoimmune thyroiditis (AIT).
Design: Retrospective cohort analysis of paedDTC patients with and without AIT, prospectively enrolled in the German Malignant Endocrine Tumour Registry between 1997 and 2024.
Methods: We analysed data from paediatric patients (<18 years) with histopathologically confirmed DTC, stratified by AIT status.
Objectives: To explore the clinical spectrum and symptom duration in pediatric adrenocortical tumors (pACTs), with a focus on identifying cases that may reflect gradual tumor progression.
Methods: We retrospectively analyzed data from 110 pediatric patients with pACTs enrolled in the German Pediatric Oncology Hematology-Malignant Endocrine Tumor (GPOH-MET) studies (1997-2022). Endocrine symptom duration, histopathological classification, and clinical outcomes were assessed.
Pancreatic neuroendocrine tumors (panNETs) are rare pediatric malignancies with age-specific clinical and biological features. Data on their presentation, management, and outcomes remain limited. This retrospective study analyzed 28 pediatric panNET cases from the German Malignant Endocrine Tumor (MET) Registry enrolled between 1997 and 2024.
View Article and Find Full Text PDFPediatric adrenocortical carcinoma (pACC) is an exceptionally rare and aggressive malignancy, accounting for only 0.2-0.3% of childhood cancers.
View Article and Find Full Text PDFEur Thyroid J
February 2025
Introduction: This case report aims to discuss the development of fatal lung fibrosis in a young boy following treatment of metastasized differentiated thyroid carcinoma (DTC).
Case Presentation: A 3.6-year-old boy was diagnosed in year 1995 with papillary thyroid carcinoma with extensive metastases.
Among childhood cancer survivors, the cumulative incidence rate of differentiated thyroid carcinomas (DTCs) is estimated to be 8-11%. Although the association of DTC with prior radiotherapy is well-studied, the association with chemotherapy remains less understood. Most studies focused on young adults, leaving a knowledge gap on subsequent DTC occurring in childhood and adolescence.
View Article and Find Full Text PDFEarly identification of patients at risk with von Hippel-Lindau (VHL) syndrome-related pheochromocytoma and paraganglioma (PPGL) is crucial to prevent morbidity. We investigated the current surveillance recommendations in VHL-related PPGL in children and adolescents. German Pediatric Oncology and Hematology-Malignant Endocrine Tumor registry (GPOH-MET) and Freiburg-VHL registry (1996-2022).
View Article and Find Full Text PDFBackground: Paediatric appendiceal neuroendocrine tumours (appNET) are very rare tumours, mostly detected incidentally by histopathological evaluation after appendectomy. Treatment recommendations are based on adult data considering high-risk NET as defined by European Neuroendocrine Tumour Society (ENETS) guidelines for completion right-sided hemicolectomy (RHC). Recent data suggest that less aggressive therapy may be justified.
View Article and Find Full Text PDFJ Clin Endocrinol Metab
July 2024
Context: Adrenocortical carcinomas are very rare malignancies in childhood associated with poor outcome in advanced disease. Most adrenocortical tumors (ACT) are functional, causing signs and symptoms of adrenal hormone excess. In most studies, endocrine manifestations were reported 4 to 6 months prior to diagnosis.
View Article and Find Full Text PDFBackground: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management.
Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included.
Objectives: Bronchial carcinoid tumors (BC) are exceptionally rare in childhood, with an incidence of <0.2/1,000,000 per year. Typical low-grade BCs are distinguished from atypical, intermediate-grade BCs.
View Article and Find Full Text PDFBackground: Adrenocortical tumors (ACTs) encompassing the adrenocortical adenoma (ACA), carcinoma (ACC), and tumors of undetermined malignant potential (ACx) are rare endocrine neoplasms with a poor prognosis. We report on pediatric ACT patients registered with the Malignant Endocrine Tumor studies and explore the EXPeRT recommendations for management. Patients: Data from the ACT patients (<18 years) were analyzed.
View Article and Find Full Text PDFOrthopadie (Heidelb)
October 2022
Osteonecrosis occurs as an acute and long-term serious side effect in children, adolescents, and adults with acute lymphoblastic leukemia. It is associated with severe pain and reduced mobility, ultimately leading to joint destruction and significant long-term morbidity. The cumulative incidence ranges from 11 to 20% in adolescents and young adults.
View Article and Find Full Text PDFEndocr Relat Cancer
September 2022
Adjuvant treatment with mitotane and chemotherapy is recommended for paediatric advanced and metastatic adrenocortical carcinoma (ACC). Yet, questions on the indication, dosage, and length of therapy are unanswered. Data from the German Paediatric Oncology Haematology-Malignant Endocrine Tumour studies were analysed retrospectively for patients receiving mitotane during first- and/or second-line therapy.
View Article and Find Full Text PDFIn children and adolescents, neuroblastoma (NBL), pheochromocytoma (PCC), and adrenocortical tumors (ACT) can arise from the adrenal gland. It may be difficult to distinguish between these three entities including associated extra-adrenal tumors (paraganglioma, PGL). Precise discrimination, however, is of crucial importance for management.
View Article and Find Full Text PDFOsteonecrosis is a serious complication of antileukemic therapy associated with severe pain and reduced mobility, ultimately leading to joint destruction and significant long-term morbidity. The 5-year cumulative incidence of osteonecrosis ranges from 11% to 20% in adolescents and young adults to 3% to 8% in patients aged 30 years and older. Most symptomatic patients have multiple joints affected, which in turn poses a risk factor for developing severe osteonecrosis.
View Article and Find Full Text PDFEur J Cancer Care (Engl)
September 2020
J Adolesc Young Adult Oncol
February 2020
Biol Blood Marrow Transplant
February 2020
Allogeneic hematopoietic stem cell transplantation (HSCT) is widely performed in children and adolescents with hematologic diseases, including very high-risk leukemia. With increasing success and survival rates, the long-term sequelae of HSCT have become important. Here, we provide guidance to the prevention and treatment of the most common bone morbidities-osteoporosis and osteonecrosis-emerging in the context of HSCT in children and adolescents.
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