Publications by authors named "Marina Kunstreich"

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.

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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.

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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.

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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.

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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.

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Pediatric adrenocortical carcinoma (pACC) is an exceptionally rare and aggressive malignancy, accounting for only 0.2-0.3% of childhood cancers.

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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.

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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.

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Early 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).

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Article Synopsis
  • The study analyzes the rising incidence of differentiated thyroid carcinoma (DTC) among children and adolescents in Germany over 25 years, using data from two significant registries.
  • A total of 469 DTC cases were identified, predominantly papillary thyroid cancer, with the average age-specific incidence rate rising especially among females and adolescents aged 15-17.
  • The findings show an overall increase in pediatric DTC, highlighting improved detection methods while raising concerns about potential underlying health factors contributing to this trend.
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Background: 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.

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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.

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Background: 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.

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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.

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Background: 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.

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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.

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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.

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In 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.

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Osteonecrosis 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.

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Article Synopsis
  • A significant majority of adolescent cancer patients received education about infertility risk (80.2%) and fertility preservation counseling (73.2%), but only half felt well-informed to make decisions.
  • Lack of recall of fertility counseling and being female were linked to lower rates of cryopreservation uptake, while older age correlated with higher utilization.
  • Although fertility counseling was fairly accessible and impacted decisions on cryopreservation, there is a need for better information to help patients make informed choices about their fertility options.
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  • A study involving adolescent cancer patients and their parents across multiple centers in Europe examined their wishes and concerns about fertility three months post-diagnosis.
  • Both patients (86.1%) and parents (96.3%) expressed a strong desire for biological parenthood, with female patients and parents showing higher fears of cancer recurrence.
  • Understanding concerns about fertility and providing tailored counseling on fertility preservation are crucial for supporting patients and parents through the cancer treatment process.
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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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Article Synopsis
  • Infertility is a significant concern for adolescents after cancer treatment, yet many are not informed about this risk during their care.
  • An intervention study compared standard patient education with additional fertility information to see if it impacted the use of cryopreservation among young cancer patients.
  • Although the addition of fertility resources did not significantly change cryopreservation rates, it did make patients more aware of their individual infertility risks, leading to higher usage among those at greater risk.
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Article Synopsis
  • The study focused on improving knowledge and empowerment about fertility preservation among adolescent cancer patients and their parents through specially designed educational materials.
  • Although the educational intervention increased knowledge levels, the differences observed were not statistically significant, but empowerment levels improved for both patients and parents.
  • Future initiatives will aim to create simpler and digital resources to better reach younger and less educated individuals regarding fertility preservation.
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