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PURPOSE: High-grade gliomas in infants and very young children (less than 3 to 5 years old) pose significant challenges due to the limited scientific literature available and high risks associated with treatments. This study aims to investigate their characteristics, treatment, and outcomes. METHODS: A cohort study was conducted at Children's Cancer Hospital, Egypt. Cases included children aged < 5 years old with confirmed CNS high-grade glioma. Baseline clinical and radiological characteristics, besides potential prognostic factors were assessed. RESULTS: In total, 76 cases were identified, 7 of them were < 1 year old. Gross- or near-total resection (GTR/NTR) was achieved in 32.9% of all cases. Of the tested cases, H3K27M-alteration was present in 5 subjects only. The 3-year OS and EFS for all cases were 26.9% and 15.4%, respectively. Extent of resection was the most important prognostic factor, as those achieving GTR/NTR experienced more than double the survival compared to those who do not (p = 0.05). Age had a "bimodal" effect on EFS, with those aged 1 to 3 years old faring better than younger and older age groups. Subjects with midline tumors had worse survival compared to non-midline tumors (1-year EFS = 18.5% vs 35%, respectively, p = 0.02). CONCLUSION: This study in a large cohort of HGG in infants and very young children offers insights into the characteristics and treatment challenges. Extent of resection, age group, and tumor localization are important prognostic factors. Further research with larger sample size is warranted to refine treatment approaches and improve outcomes.
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Neoplasias Encefálicas , Glioma , Humanos , Lactente , Glioma/terapia , Glioma/cirurgia , Masculino , Feminino , Pré-Escolar , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/cirurgia , Estudos de Coortes , Resultado do Tratamento , Procedimentos Neurocirúrgicos/métodos , PrognósticoRESUMO
BACKGROUND: F18-FET PET has an established diagnostic role in adult brain gliomas. In this study we analyzed image derived static and dynamic parameters with available conventional MRI, histological, clinical and follow-up data in assessment of pediatric brain tumor patients at different stages of the disease. METHODS: Forty-four pediatric patients with median age 7 years, diagnosed with brain tumors and underwent forty-seven 18F-FET PET scans either initially (20 scans) or post-therapy (27 scans) were enrolled. Standardized analysis of summed FET PET images early from 10-20 min and late from 30-40 min post-injection were used for static (mean and maximum tumor to brain ratio [TBR] and biological tumor volume [BTV]) parameters evaluation as well as the time activity curve [TAC]. RESULTS: Nineteen out of 20 initially assessed patients had pathologically and/or clinico-radiologically proven neoplastic lesions and one patient had pathologically proven abscess. Receiver operator curve (ROC) marked early TBR max 2.95, early TBR mean 1.76, late TBR max 2.5 and late TBR mean 1.74 as discriminator points with diagnostic accuracy reaching 90% when TBR max was combined with dynamic parameters. Significant association was found between initial FET scans, early and late BTV and event free survival (EFS) (P value=0.042 and 0.005 respectively). In post-therapy assessment, the diagnostic accuracy of conventional MRI was 81.48% when used alone and 96.30% when combined with F18-FET PET scan findings. A cutoff point of 3.2 cm3 for late BTV, in post-therapy scans, was successfully marked as a predictor for therapy response (P value 0.042) and was significantly associated with EFS (P value 0.002). In FET-avid / MRI non-enhancing lesions, early TBR max was able to detect highly malignant processes (high-grade tumors in initial scans and residue/recurrence in post-therapy scans) with 80% sensitivity and 100% specificity when cutoff value of 2.25 was used (P value=0.024). In patients with FET-avid brainstem lesions, whether enhancing or non-enhancing in MRI scans, 81.8% were associated with high risk diagnoses and 68.2% of them were associated with poor therapy outcome. The degree of FET uptake matched tumor-grading, but did not show significant association with OS or EFS (P value>0.05). CONCLUSIONS: F18-FET PET seems to be an evolving pediatric neuro-imaging technique with valuable diagnostic and prognostic information at initial and post-therapy evaluation.
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Neoplasias Encefálicas , Glioma , Adulto , Humanos , Criança , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Glioma/diagnóstico por imagem , Glioma/patologia , Encéfalo , Tomografia por Emissão de Pósitrons/métodos , Gradação de Tumores , Imageamento por Ressonância MagnéticaRESUMO
Background: Deep inspiration breath-hold (DIBH) has been established to decrease normal tissue radiation dose in breast cancer. Methods: Forty-nine patients had two CT scans during DIBH or free breathing. Chest-wall position, setup verification and breath-hold monitoring were performed. Cone-beam CT and a surface image system were used for verification. Results: A total of 1617 breath-holds were analyzed in 401 fractions. The mean time bit was 6.01 min. The mean breaths-holds per fraction was 4.96. The median for intra-breath hold variability was 3 mm. No patient stopped treatment for intolerance. Clinical target volume margins were calculated as 0.36, 0.36 and 0.32 for the three translational positions. The mean saved volume was 26.3%. Conclusion: Voluntary DIBH is feasible, tolerable and easy to apply for children with Hodgkin lymphoma involving the mediastinum.
Deep inspiration breath-hold (DIBH) is a radiotherapy maneuver to decrease the exposure of normal tissues during the radiation of the target organ. It was developed for the treatment of breast cancer, both intact breast and chest wall, after mastectomy. For mediastinal Hodgkin lymphoma, especially in children and adolescents, DIBH will benefit in decreasing the radiation dose to the lungs and heart in this category of patients who still have normal growing tissues. We treated 49 pediatric and adolescent patients with DIBH and precise radiotherapy (volumetric modulated arc therapy) to augment the benefit of lowering the dose to normal tissues. All patients were trained and coached to breath-hold for more than 20 s. No patient stopped treatment due to poor tolerance or discomfort. Only one breath-hold was required for CT simulation in all populations (100%). The mean number of breath-holds per treatment fraction was 5.1 ± 1.8. The mean treatment time was 6 ± 1.8 min. DIBH is feasible, tolerable and easy to apply for children and adolescents with Hodgkin lymphoma involving the mediastinum. A considerable dose volume could be saved, hence decreasing the rate of side effects.
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Suspensão da Respiração , Doença de Hodgkin , Inalação , Neoplasias do Mediastino , Radioterapia de Intensidade Modulada , Doença de Hodgkin/diagnóstico por imagem , Doença de Hodgkin/radioterapia , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/radioterapia , Tomografia Computadorizada de Feixe Cônico , Humanos , Masculino , Feminino , Criança , AdolescenteRESUMO
Aims: To assess the clinical, pathological and molecular characteristics (Sonic hedgehog and group 3/4 molecular subtypes expression) and treatment modalities for infantile medulloblastoma in correlation with outcomes. Materials & methods: A retrospective study of 86 medulloblastoma patients (≤3 years) was conducted. M0 patients <2.5 years received four cycles of chemotherapy followed by focal radiotherapy (FRT) and chemotherapy. Between 2007 and 2015, Metastatic patients <2.5 years received craniospinal irradiation (CSI) after the end of chemotherapy. After 2015, metastatic patients <2.5 years received CSI postinduction. Results: The hazard ratio for death was significantly higher in the FRT (HR = 2.8) group compared with the CSI group (hazard ratio = 1). Metastatic disease significantly affected the overall survival of the Sonic hedgehog group and the overall survival and event-free survival of group 3/4. Conclusion: Metastatic disease had a significant impact on outcomes. FRT is not effective in treating infantile medulloblastoma.
This study aimed to analyze the management of and prognostic factors affecting the outcomes of 86 young children (<3 years of age at presentation) diagnosed with medulloblastoma, an aggressive brain tumor that is commonly seen in this age group. All children had surgical operations aiming at resecting their tumors, followed by chemotherapy and irradiation. Study results showed that disease disseminated into the nervous system was associated with poorer outcomes compared with localized disease. Administration of local irradiation to the primary tumor site in the brain only, without exposing the spinal cord to radiotherapy, was associated with a higher risk of death.
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Neoplasias Cerebelares , Meduloblastoma , Humanos , Meduloblastoma/diagnóstico , Meduloblastoma/genética , Meduloblastoma/terapia , Terapia Combinada , Prognóstico , Proteínas Hedgehog , Estudos Retrospectivos , Egito/epidemiologia , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/radioterapia , Irradiação CranianaRESUMO
During the COVID-19 pandemic, major challenges are facing pediatric cancer centers regarding access to cancer centers, continuity of the anti-cancer therapy, hospital admission, and infection protection precautions. Pediatric oncologists actively treating children with cancer from 29 cancer centers at 11 countries were asked to answer a survey from May 2020 to August 2020 either directly or through the internet. COVID-19 pandemic affected the access to pediatric cancer care in the form of difficulty in reaching the center in 22 (75.9%) centers and affection of patients' flow in 21 (72.4%) centers. Health care professionals (HCP) were infected with COVID-19 in 20 (69%) surveyed centers. Eighteen centers (62%) modified the treatment guidelines. Care of follow-up patients was provided in-hospital in 8(27.6%) centers, through telemedicine in 10 (34.5%) centers, and just delayed in 11 (38%) centers. Pediatric oncologists had different expectations about the future effects of COVID-19 on pediatric cancer care. Seventy-six percent of pediatric oncologists think the COVID-19 pandemic will increase the use of telemedicine. Fifty-five percent of pediatric oncologists think if the COVID-19 pandemic persists, we will need to change chemotherapy protocols to less myelosuppressive ones. Collaborative studies are required to prioritize pediatric cancer management during COVID-19 era.
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COVID-19 , Neoplasias , Telemedicina , Humanos , Criança , COVID-19/epidemiologia , COVID-19/prevenção & controle , Pandemias/prevenção & controle , Neoplasias/epidemiologia , Neoplasias/terapia , Inquéritos e QuestionáriosRESUMO
Global debt has grown to record levels. Government debt, corporate debt and household debt around the world rose to a high of 350% of world gross domestic product in 2022. The systemic risk that has built up during the extended period of low interest rates threatens to materialize now as rates rise worldwide. For countries in which external liabilities are high, debt service costs will increase, and refinancing may become prohibitively expensive. External liabilities and their term structures provide insight into which emerging and developing countries may be most vulnerable in the next months. Supplementary Information: The online version contains supplementary material available at 10.1007/s11293-023-09763-y.
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Background: This study explores the prognostic factors and outcomes of different treatment modalities in focal brain stem glioma (FBSG). Materials & methods: Pediatric FBSG patients diagnosed during 2010-2017 were retrospectively reviewed for clinical and therapeutic data. Results: A total of 71 cases were identified and the median age was 6.4 years. The 5-year overall- and progression-free survival were 74.5 and 70.6%, respectively. Radiotherapy was the main line of treatment (66.2%) and there were no survival differences between radiotherapy, chemotherapy and surveillance groups. Two independent poor prognostic factors were identified on multivariate analysis: age <8 years and cervicomedullary tumor site (p = 0.02 for both). Conclusion: Surveillance, radiotherapy and chemotherapy have comparable clinical outcomes in pediatric FBSG.
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Neoplasias do Tronco Encefálico/diagnóstico , Neoplasias do Tronco Encefálico/terapia , Glioma/diagnóstico , Glioma/terapia , Adolescente , Neoplasias do Tronco Encefálico/mortalidade , Criança , Pré-Escolar , Terapia Combinada/efeitos adversos , Terapia Combinada/métodos , Gerenciamento Clínico , Feminino , Glioma/mortalidade , Humanos , Lactente , Masculino , Gradação de Tumores , Estadiamento de Neoplasias , Prognóstico , Modelos de Riscos Proporcionais , Avaliação de Sintomas , Resultado do TratamentoRESUMO
BACKGROUND: Wilms tumor (WT) with an inferior Vena cava (IVC) malignant thrombus comprises 4-10% of all WT cases. METHODS: This retrospective analysis included 51 pediatric patients presenting at Children Cancer Hospital Egypt-57357 from July 2007 to December 2016 with the diagnosis of WT with malignant IVC thrombus. RESULTS: Median age at presentation = 4.4 years and 28 cases (55%) were females. Twenty-five patients (49%) were metastatic and 4 patients (7.8%) had bilateral disease. Forty-seven cases (92.2%) had favorable histology with no evidence of anaplasia. Level of thrombus extension at presentation was classified as infra-hepatic, retro-hepatic, supra-hepatic and intra-cardiac in 33, 9, 6 and 3 patients, respectively. Fifty patients started neoadjuvant chemotherapy (CTH) with 16 patients showing complete resolution of thrombus after 6 weeks of CTH. None of the patients developed thrombus progression after neoadjuvant CTH; one patient had stationary intra-cardiac thrombus, while remaining patients showed partial regression of their thrombus and had nephrectomy with en-bloc thrombectomy. The mean cranio-caudal dimension of IVC thrombi at initial presentation was 6.5 cm, and 3.6 cm post 6th week of CTH. The 5-year OS and EFS were 75.9% and 71.1%, respectively. There was no significant correlation of initial levels of thrombus extension with survival. CONCLUSION: Neoadjuvant chemotherapy followed by radical nephrectomy with en-bloc thrombectomy and radiotherapy seems a successful approach for management of patients with WT and IVC tumor thrombus. Measurement of the cranio-caudal dimension of thrombus and its response to treatment should be considered in the surgical planning.
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Neoplasias Renais/terapia , Trombose Venosa/patologia , Tumor de Wilms/terapia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Masculino , Terapia Neoadjuvante , Nefrectomia/métodos , Estudos Retrospectivos , Trombectomia/métodos , Trombose/patologia , Resultado do Tratamento , Veia Cava Inferior/patologia , Tumor de Wilms/patologia , Tumor de Wilms/cirurgiaRESUMO
Association of trace metal concentrations in water is problematic; however, its information is scarce and sometimes contradicted. This work presents variations in dissolved major constituents and trace element concentrations along the quaternary aquifers located in middle Upper Egypt (Minia and Assiut governorates). A total of 205 groundwater samples from these aquifers were collected. Auxiliary parameters (pH, alkalinity, and conductivity), major cations (Ca2+, Mg2+, Na+, and K+), dominant anions (HCO3-, SO42-, Cl-, and NO3-), and trace element (B, Fe, Cu, Mn, Ni, Pb, Cd, and Cr) concentrations were measured in all samples. Univariate (correlation coefficient and scatter matrix) analysis was employed combined with multivariate (principal coordinates analysis) analysis to identify the chemical characteristics of groundwater that are responsible for generating most of the variability within the dataset. Also, hierarchical cluster analysis was applied to classify the geochemical origin of the groundwater constituents. The results indicate that the groundwater pollution is mainly due to water-rock interactions, including aquifer matrix dissolution, redox reaction of trace metals, input from wastewater, and agricultural fertilizers.
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Água Subterrânea , Oligoelementos , Poluentes Químicos da Água , Egito , Monitoramento AmbientalRESUMO
PURPOSE: Our aim is to present our experience in the management of pediatric meningiomas in the largest referral center in Egypt for pediatric tumors focusing on variables including clinical picture, anatomic location, histopathology, treatment strategies, and outcome together with their possible correlation to prognosis. METHODS: We retrospectively reviewed the medical records of 39 pediatric patients who were treated for CNS meningiomas in Children's Cancer Hospital-Egypt (CCHE-57357) 2007-2017. RESULTS: The prevalence of pediatric meningioma was 1.42%. Four cases had type 2 neurofibromatosis (NFII). The mean age was 8.19 years. The presence of NFII was associated with challenging multiple lesions, older age of presentation and poorer prognosis and functional outcome. Convexity was the commonest location. Gross total resection (GTR) was achieved in 28 cases, subtotal resection (STR) in 8 cases, and biopsy was decided in 3 patients. Histopathological examination revealed WHO grade I in 16 patients and higher grades in 23 patients (59%). The 5-year overall survival (OS) rate was 87.8% while the 5-years event-free survival (EFS) rate was 85.6%. Tumor location, histopathology, and clinical presentation were not statistically correlated to prognosis. CONCLUSIONS: Pediatric CNS meningiomas are uncommon pediatric tumors but of an aggressive clinical and pathological behaviors as compared to adult meningiomas. The presence of NFII is associated with a poorer prognosis and functional outcomes. Although being challenging, the maximum and safe surgical excision should be exercised even in recurrent cases in order to achieve the best outcome. Adjuvant radiotherapy provides good tumor control for inoperable residual atypical or anaplastic meningiomas.
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Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/terapia , Meningioma/patologia , Meningioma/terapia , Criança , Pré-Escolar , Egito , Feminino , Humanos , Masculino , Neoplasias Meníngeas/genética , Meningioma/genética , Neurofibromatose 2/complicações , Procedimentos Neurocirúrgicos/métodos , Radioterapia Adjuvante/métodosRESUMO
Pediatric radiotherapy is a critical part of pediatric oncology protocols and the quality of the radiotherapy may determine the future quality of life for long-term survivors. Multidisciplinary team decision making provides the basis for high-quality care. However, delivery of high-quality radiotherapy is dependent on resources. This article provides guidelines for delivery of good quality radiation therapy in resource-limited countries based on rational procurement and maintenance planning, protocol development, three-dimensional planning, quality assurance, and adequate staff numbers and training.
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Países em Desenvolvimento , Neoplasias/radioterapia , Radioterapia (Especialidade) , Radioterapia/métodos , Criança , Humanos , Radioterapia (Especialidade)/métodos , Radioterapia (Especialidade)/normas , Sociedades MédicasRESUMO
A secondary analysis of 2 randomized Radiation Therapy Oncology Group trials demonstrated that age ≥70 years was a favorable prognostic factor among men treated with external beam radiation therapy (EBRT). In contrast, several series based on men undergoing radical prostatectomy (RP) suggested that older age was an unfavorable prognostic factor. Our study was initiated to determine whether these observations reflect a true but paradoxical underlying age-related treatment-dependent biological phenomenon. We conducted a systematic review (PubMed, January 1, 1999-January 30, 2023) evaluating the effect of age on cancer-specific outcomes after definitive local treatment with either RP or EBRT. Our main objective was to assess possible interactions between age (using a cutoff of 70 +/- 5 years) and treatment type, with regard to adverse cancer-specific outcomes (eg, pathology, biochemical failure, distant metastasis, or prostate cancer-specific survival). Forty-five studies were selected for inclusion in this systematic review, including 30 and 15 studies with patients treated with RP and EBRT, respectively. Among patients treated with RP, 10 (50%) of these studies suggested that older age was associated with worse outcome(s) after RP. None suggested that age was a favorable prognostic factor after RP. Among the EBRT-based studies, 8 (53%) suggested that older age was associated with better outcomes, with an additional 3 studies (21%) trending to support a better outcome. None of these studies involving EBRT suggested that older age was an adverse prognostic factor. This systematic review suggests that age using a categorical cutoff of 70 +/- 5 years may be an adverse prognostic factor for men undergoing RP but a favorable prognostic factor for men treated with EBRT. Further research is needed to validate these findings.
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Neoplasias da Próstata , Masculino , Humanos , Idoso , Neoplasias da Próstata/radioterapia , Neoplasias da Próstata/patologia , Próstata/patologia , Prostatectomia , Terapia Combinada , Glândulas Seminais , Resultado do TratamentoRESUMO
BACKGROUND: Volumetric Modulated Arc Therapy (VMAT) is an option for the delivery of Radiotherapy treatment technique for pediatric nasopharyngeal cancer, VMAT is the most common treatment technique for pediatric nasopharyngeal cancer. The use of a combination of both biological and physical parameters in VMAT planning optimization may produce better target coverage and sparing of critical organs. This work was to compare Biological Cost Functions (BCFs) and Physical Cost Functions (PCFs) in the VMAT of pediatric nasopharyngeal cancer patients. METHOD: VMAT plans for 20 nasopharyngeal pediatric cancer patients were created using Monaco 5.11® treatment planning system (TPS). Three VMAT plans were retrospectively generated for each patient using BCFs, PCFs and mixed plan with a total dose of 61.2 Gy in 34 fractions to planning target volume (PTV). All plans were adjusted to deliver 95% of the prescribed dose to 95% of the PTV. The calculated plans were qualitatively and quantitatively evaluated using the dose-volume histogram (DVH). RESULTS: The coverage of the target and the maximum dose for the three plans were nearly the same, and better sparing was achieved in the serial organs (spinal cord and brain stem) with PCFs. On the contrary, more dose spring was observed using the BCFs in the organs at risk (OARs) that were not involved in the dose optimization, such as the optic nerve maximum dose, with a significant p-value (0.035 and 0.0001) respectively. Using the PCFs, both parotids received a lower mean dose, but not for the oral cavity, which had a lower mean dose using BCFs (p=<0.0001). The same values of tumor control probability (TCP) were found for both cost functions in PTVs and normal tissue complications probability (NTCP) (99%). The values reported were as follows: spinal cord = 0.5%, brain stem = 19.1%, and brain = 90.7% for BCFs, compared to spinal cord = 0.3%, brain stem = 14.9%, and brain = 90.7% for PCFs. The delivery time was found to be less in BCFs (p=0.005). CONCLUSION: The BCFs are superior to the PCFs in conformity index and time of radiation delivery. However, PCFs were better at dose sparing for the serial organs and achieving a sharper falloff dose around the involved volumes. A patient-specific clinical compromise is recommended to gain the best plan that meets the clinical goals.
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Neoplasias Nasofaríngeas , Radioterapia de Intensidade Modulada , Humanos , Criança , Neoplasias Nasofaríngeas/radioterapia , Estudos Retrospectivos , Encéfalo , Órgãos em RiscoRESUMO
Remdesivir can precipitate fatal acute necrotizing pancreatitis especially in patients who previously suffer from hypertriglyceridemia.
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BACKGROUND/PURPOSE: Treatment of spine and sacral chordoma generally involves surgical resection, usually in conjunction with radiation therapy.In certain locations, resection may result in significant neurological dysfunction, so definitive radiation has been used as an alternative to surgery. The purpose of this study is to report the results of high-dose, proton-based definitive radiotherapy for unresected spinal and sacral chordomas. MATERIALS/METHODS: Retrospective review of 67 patients with newly diagnosed, unresected spinal chordomas treated with high-dose definitive, proton-based radiotherapy at our center from 1975 to 2019. RESULTS: Reasons for radiotherapy alone included medical inoperability and/or concern for neurological dysfunction based on spine level or patient choice. Tumor locations included cervical (n = 10), thoracic (n = 1), lumbar (n = 4) spine, and sacrum (n = 52). Median maximal tumor diameter was 7.4 cm (range 1.8-25 cm). Median total dose was 77.4 Gy (RBE) (range 73.8-85.9 Gy RBE). Analysis with median follow-up of 56.2 months (range, 4-171.7 months) showed overall survival of 83.5 % (95%CI: 69.4-91.5%) and 65.9% (95%CI: 47.3-79.3%), disease-free survival of 64% (95%CI: 49.3-75.4) and 44.1% (95%CI: 27.8-59.2%), local control of 81.8% (95%CI: 67.6-90.2%) and 63.6% (95%CI: 44.7-77.5%), and distant control of 77.4% (95%CI: 63.6-86.5%) and 72.5% (95%CI: 55.7-83.8%) at 5 and 8 years respectively. The most common late side effect was insufficiency fracture. CONCLUSION: These results continue to support the use of high-dose definitive radiotherapy for patients with medically inoperable or otherwise unresected mobile spine or sacrococcygeal chordomas. There is a trend towards better disease-free survival with doses > 78 Gy (RBE).
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Cordoma , Terapia com Prótons , Neoplasias da Coluna Vertebral , Cordoma/radioterapia , Humanos , Terapia com Prótons/efeitos adversos , Prótons , Estudos Retrospectivos , Sacro/patologia , Sacro/efeitos da radiação , Sacro/cirurgia , Neoplasias da Coluna Vertebral/radioterapia , Resultado do TratamentoRESUMO
Early and frequent evaluation of thyroid profile in COVID-19 infected patients is crucial as it will influence thyroid disease sequelae and management in those patients; moreover, it will facilitate setting an appropriate management plan.
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BACKGROUND: The survival of pineoblastoma patients is low, particularly in infants and those with metastatic disease. This study aimed to analyze the prognostic factors affecting the outcome of Pineoblastoma in different age groups. METHODS: A retrospective study included 33 patients. Twenty-two patients older than 3 years had upfront surgery, followed by induction CSI then 6 cycles of chemotherapy. Eleven patients younger than 3 years underwent surgery, followed by induction chemotherapy then radiation therapy. Focal irradiation (54 Gy) was administrated in six patients, and CSI (23.4 Gy) with booster dose 30.6 Gy to the tumor bed in two patients followed by 4 cycles of chemotherapy. RESULTS: Patient's age showed a significant impact on the outcome (P value = 0.001 for EFS and 0.002 for OS). The metastases' presence did not impact the outcome negatively. The survival of patients with metastatic disease did not differ between age groups. However, age had a significant impact on the outcome of M0 disease, with 3-year EFS and OS of 65.3% and 74%, respectively, in the older group compared to 0% for both rates in younger patients. CSI showed a positive impact on survival. For all cases, the 3-year OS and EFS were 46.7% and 44.4%, respectively. CONCLUSIONS: A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients' outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastoma's dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. Molecular typing should be considered to label patients who need a more intensified approach.
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Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/radioterapia , Terapia Combinada , Humanos , Lactente , Pinealoma/diagnóstico , Pinealoma/radioterapia , Prognóstico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Radiation therapy (RT) is used for pediatric craniopharyngioma in the definitive, adjuvant, or salvage settings. Proton RT may be useful owing to tumor proximity to eloquent anatomy. We report clinical outcomes for a large cohort treated with proton therapy. METHODS: We conducted a retrospective review of pediatric patients (≤21 years) treated with surgery and proton therapy for craniopharyngioma between August 2002 and October 2018. Clinical characteristics, treatment course, and outcomes were recorded. Acute toxicity was graded using Common Terminology Criteria for Adverse Events, version 5.0. Late toxicity was assessed using neuroendocrine, neuro-ophthalmologic, and neuropsychological testing. RESULTS: Among 77 patients, median age at diagnosis was 8.6 years (range, 1.3-20); median age at radiation was 9.6 years (range, 2.3-20.5). Most common presenting symptoms were headache (58%), visual impairment (55%), and endocrinopathy (40%). Patients underwent a median of 2 surgical interventions (range, 1-7) before protons. At initial surgery, 18% had gross total resection, 60% had subtotal resection, and 22% had biopsy/cyst decompression. Median RT dose was 52.2 Gy (relative biologic effectiveness). Common acute toxicities were headache (29%), fatigue (35%), and nausea/vomiting (12%). Only 4% developed any acute grade 3 toxicity. Nine patients experienced cyst growth requiring replanning or surgical decompression. At a median of 4.8 years from RT (range, 0.8-15.6), there were 6 local failures and 3 deaths, 2 related to disease progression. Effect of tumor and treatment contributed to late toxicity including Moyamoya syndrome (13%), visual impairment (40%), and endocrine deficiency requiring hormone replacement (94%). Subclinical decline in functional independence and adaptive skills in everyday life was detected at follow-up. CONCLUSIONS: Surgery and proton therapy results in excellent disease control for pediatric craniopharyngioma. Severe acute toxicity is rare. Late toxicities from tumor, surgery, and radiation remain prevalent. Endocrine and ophthalmology follow-up is necessary, and neuropsychological testing may identify patients at risk for treatment-related cognitive and adaptive functioning changes.
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Neoplasias Hipofisárias/radioterapia , Terapia com Prótons , Adolescente , Criança , Pré-Escolar , Craniofaringioma/complicações , Craniofaringioma/patologia , Craniofaringioma/radioterapia , Craniofaringioma/cirurgia , Fadiga/etiologia , Feminino , Cefaleia/etiologia , Humanos , Lactente , Masculino , Doença de Moyamoya/etiologia , Náusea/etiologia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/cirurgia , Terapia com Prótons/efeitos adversos , Lesões por Radiação/patologia , Estudos Retrospectivos , Resultado do Tratamento , Carga Tumoral , Transtornos da Visão/etiologia , Vômito/etiologia , Adulto JovemRESUMO
Methylene tetrahydrofolate reductase (MTHFR) gene mutations could be the cause of infertility in hypothyroid patients. Hence, it is worthy to screen for MTHFR gene mutations in infertile hypothyroid females and their partners if infertility persists after optimizing thyroid function.
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OBJECTIVE: To determine the prognostic value of quantitative fluorine-18-fluorodeoxyglucose (F-FDG) standardized uptake value (SUV) in patients with pediatric rhabdomyosarcoma (RMS). PATIENTS AND METHODS: Consecutive 98 (50 males and 48 females) (age range: 4 months to 17.5 years, mean age: 5.8 ± 4.5) patients with pathologically proven RMS who underwent PET/computed tomography for initial staging were retrospectively assessed for whether primary SUVmax and the primary/liver SUVmax ratio could predict event-free survival (EFS) and overall survival (OS) for 36 months using receiver operating characteristic curve analysis. Univariate and multivariate analyses were used to determine the reliability of these metabolic parameters and various clinical factors. RESULTS: Higher SUVmax was significantly related to the presence of regional or distant metastasis with worse prognosis. With receiver operating characteristic curve marked cut-off values of 3.6 and 2.1 for primary SUVmax and the primary/liver SUVmax ratio, respectively, both EFS and OS proved to be higher in patients with SUVmax ranked below the determinate values. Patients with a primary/liver SUVmax ratio below the cut-off value of 2.1 had OS (60.8%) and EFS (48.1%) compared with 44.5 and 14.8% for patients with lesions exceeding the cut-off point of uptake; however, this failed to achieve statistical significance. In the evaluation of primary SUVmax, similar results were obtained with P values of 0.76 and 0.62, respectively. High SUVmax was more prevalent among patients with less favorable clinical and pathological features including unfavorable primary site, alveolar pathology, and high-risk group. CONCLUSION: F-FDG PET/computed tomography may be considered an additional prognostic predictor of outcome in RMS patients, where higher F-FDG uptake seems to be linked to lower survival and correlated to different unfavorable parameters.