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BACKGROUND: Inadequate numbers of trained healthcare providers (HCPs), contribute to poor pediatric oncology (PO) outcomes, particularly in low- and lower middle-income countries (L/LMICs). An understanding of the characteristics of the workforce challenges are vital for addressing these problems. METHODS: The Pediatric Oncology East and Mediterranean (POEM) Group surveyed PO centers in countries of the North Africa, Middle East, Central Asia and Indian subcontinent on infrastructure and workforce capacity, service availability, and training opportunities for HCPs. Participating centers were categorized by the World Bank income levels for their countries and correlated with services, workload and staffing characteristics, and training needs. RESULTS: Fifty of 82 member-centers (61%) from 21 countries responded to the survey. 299 pediatric oncologists and 1,176 nurses treated 12,496 new PO patients/year, with a 1,451 beds utilization. The majority (71%) of new cases occurred in L/LMICs. The availability of HCPs correlated with country income level, as did pediatric subspecialty access, while availability of support services was unrelated. Twenty-five centers in 11 countries offered PO fellowship training for physicians, whereas 13 PO nurse training centers in 9 countries had the capacity to train 273 nurses annually. The survey respondents indicated that, among their existing workforce, an average of 3·5 physicians and 14 nurses per institution would benefit from additional PO training opportunities. CONCLUSIONS: The participating centers exhibited intra-regional heterogeneity in financial resources, infrastructure, workload, workforce, and medical services. Our findings provide insight into the disparities and regional resources available to POEM, which can be mobilized to rectify specific deficiencies. This article is protected by copyright. All rights reserved.
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PURPOSE: Primary malignant bone tumors of the hand are rare in children. Resection and reconstruction of the digit are challenging and have been described in case reports. This retrospective study describes the functional and oncologic outcomes of resection and reconstruction using a nonvascularized fibular bone graft in a cohort of children. METHODS: A total of 5 children were included. The mean age at diagnosis was 7.6 years (range, 1.6-12 years). Histologic diagnosis showed Ewing sarcoma in 3 and osteosarcoma in 2 patients. Four tumors were located in the metacarpal bones of the fingers, and 1 was located in the thumb. Four patients were treated with chemotherapy. All the patients were treated with wide resection and a cement spacer. This was followed by second stage reconstruction using a nonvascularized fibular bone graft. In tumors of the fingers, carpometacarpal joint fusion with a neighboring carpal bone was performed, whereas a pseudoarthrosis was created between the graft and the base of the proximal phalanx. In the thumb's case, the opposite was done, with fusion at the metacarpophalangeal joint and a pseudoarthrosis at the carpometacarpal joint. RESULTS: The mean follow-up duration was 5.5 years (range, 2-9 years). Surgical margins were negative in all the patients. At their latest follow-up visit, none of the patients developed systemic or local recurrence. Two complications required a revision surgery, one due to graft subluxation and the other due to nonunion. At their final follow-up examination, the mean total arc of movement was 80° (range, 60°-100°), and all the patients were able to resume their grasping and writing capabilities. CONCLUSIONS: The resection and reconstruction of primary malignant bone tumors of the metacarpals using a nonvascularized fibular bone graft in children can preserve the cosmesis and function of the digit without jeopardizing oncologic outcomes. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.
Subject(s)
Bone Neoplasms , Osteosarcoma , Plastic Surgery Procedures , Pseudarthrosis , Bone Neoplasms/pathology , Bone Neoplasms/surgery , Bone Transplantation , Child , Child, Preschool , Fibula/surgery , Fibula/transplantation , Humans , Infant , Osteosarcoma/pathology , Osteosarcoma/surgery , Pseudarthrosis/surgery , Retrospective Studies , Treatment OutcomeABSTRACT
Childhood cancer is a priority in Egypt due to large numbers of children with cancer, suboptimal care and insufficient resources. It is difficult to evaluate progress in survival because of paucity of data in National Cancer Registry. In this study, we studied survival rates and trends in survival of the largest available cohort of children with cancer (n = 15 779, aged 0-18 years) from Egypt between 2007 and 2017, treated at Children's Cancer Hospital Egypt-(CCHE), representing 40% to 50% of all childhood cancers across Egypt. We estimated 5-year overall survival (OS) for 14 808 eligible patients using Kaplan-Meier method, and determined survival trends using Cox regression by single year of diagnosis and by diagnosis periods. We compared age-standardized rates to international benchmarks in England and the United States, identified cancers with inferior survival and provided recommendations for improvement. Five-year OS was 72.1% (95% CI 71.3-72.9) for all cancers combined, and survival trends increased significantly by single year of diagnosis (P < .001) and by calendar periods from 69.6% to 74.2% (P < .0001) between 2007-2012 and 2013-2017. Survival trends improved significantly for leukemias, lymphomas, CNS tumors, neuroblastoma, hepatoblastoma and Ewing Sarcoma. Survival was significantly lower by 9% and 11.2% (P < .001) than England and the United States, respectively. Significantly inferior survival was observed for the majority of cancers. Although survival trends are improving for childhood cancers in Egypt/CCHE, survival is still inferior in high-income countries. We provide evidence-based recommendations to improve survival in Egypt by reflecting on current obstacles in care, with further implications on practice and policy.
Subject(s)
Neoplasms/mortality , Adolescent , Cancer Care Facilities , Central Nervous System Neoplasms/mortality , Child , Child, Preschool , Cohort Studies , Egypt , England , Female , Hepatoblastoma/mortality , Humans , Infant , Infant, Newborn , Kaplan-Meier Estimate , Leukemia/mortality , Lymphoma/mortality , Male , Neuroblastoma/mortality , Regression Analysis , Retrospective Studies , Sarcoma, Ewing/mortality , United StatesABSTRACT
BACKGROUND: Inadequate numbers of trained health care providers (HCPs) contribute to poor pediatric oncology (PO) outcomes, particularly in low- and lower middle-income countries (L/LMICs). An understanding of the characteristics of the workforce challenges is vital for addressing these problems. METHODS: The Pediatric Oncology East and Mediterranean (POEM) Group surveyed PO centers in countries of North Africa, Middle East, Central Asia, and Indian subcontinent on infrastructure and workforce capacity, service availability, and training opportunities for HCPs. Participating centers were categorized by the World Bank income levels for their countries and correlated with services, workload and staffing characteristics, and training needs. RESULTS: Fifty of 82 member centers (61%) from 21 countries responded to the survey. Two hundred ninety-nine pediatric oncologists and 1176 nurses treated 12 496 new PO patients/year, with a 1451-bed utilization. The majority (71%) of new cases occurred in L/LMICs. The availability of HCPs correlated with country income level, as did pediatric subspecialty access, while availability of support services was unrelated. Twenty-five centers in 11 countries offered PO fellowship training for physicians, whereas 13 PO nurse training centers in nine countries had the capacity to train 273 nurses annually. The survey respondents indicated that, among their existing workforce, an average of 3.5 physicians and 14 nurses per institution would benefit from additional PO training opportunities. CONCLUSIONS: The participating centers exhibited intraregional heterogeneity in financial resources, infrastructure, workload, workforce, and medical services. Our findings provide insight into the disparities and regional resources available to POEM, which can be mobilized to rectify specific deficiencies.
Subject(s)
Developing Countries , Health Workforce , Medical Oncology/education , Neoplasms , Pediatrics/education , Child , Humans , Middle East , Pediatricians , WorkforceABSTRACT
Aim: Scarce data assessing the real value of whole lung irradiation (WLI) in Ewing's sarcoma (ES) with lung-only metastasis, with published conflicting results. We studied the impact of WLI in a homogenous pediatric population. Materials & methods: Retrospective study evaluating the survival outcomes of WLI in these patients. Results: Out of 163 metastatic ES; 41 patients were eligible for WLI. 30 patients (73.1%) received WLI (+ve) while 11 patients (26.8%) did not receive WLI (-ve). Five-year event-free survival was statistically significant in WLI (+ve). Five-year pulmonary relapse-free survival showed trend for improvement with WLI (+ve), while 5-year overall survival was not statistically significant between the two arms. Conclusion: WLI added significantly to the long term clinical outcome of metastatic ES patients, with no irreversible toxicity.
Subject(s)
Lung Neoplasms/radiotherapy , Lung Neoplasms/secondary , Sarcoma, Ewing/pathology , Child , Child, Preschool , Combined Modality Therapy , Disease Management , Female , Humans , Lung Neoplasms/diagnosis , Lung Neoplasms/mortality , Male , Prognosis , Survival Analysis , Treatment OutcomeABSTRACT
PURPOSE: Infantile hepatic hemangioendothelioma (IHHE) is the most common hepatic vascular tumor in children. We report on the treatment outcome of our large single-center experience of patients with IHHE over a 9-year period. MATERIALS AND METHODS: A retrospective analysis of all IHHE patients treated at the Children Cancer Hospital Egypt from April 2008 through April 2017. RESULTS: In total, 28 patients (18 females, 10 males) were diagnosed with IHHE with a median age at diagnosis of 3 months. The lesions were multifocal (n=12), focal (n=10), and diffuse (n=6). Six (21.4%) patients initially had low T3 and T4. Eleven patients did not receive any treatment, whereas 1 patient underwent resectional surgery. Sixteen patients received drug treatment, 9 of whom responded well to first-line propranolol/prednisolone, whereas 7 patients needed salvage treatment. Twenty-five patients are alive, whereas 3 patients have died. CONCLUSIONS: Overall, patients with IHHE do well, a significant percentage of whom do not require drug therapy, particularly for those with small focal lesions. In patients with multifocal/diffuse disease, there is a high incidence of low T3 and T4 and while some of these patients did well without additional therapy, those with rapidly progressive lesions during treatment may do poorly.
Subject(s)
Hemangioendothelioma/mortality , Liver Neoplasms/mortality , Prednisolone/therapeutic use , Propranolol/therapeutic use , Surgical Procedures, Operative/mortality , Antineoplastic Agents, Hormonal/therapeutic use , Combined Modality Therapy , Female , Follow-Up Studies , Hemangioendothelioma/pathology , Hemangioendothelioma/therapy , Humans , Infant , Infant, Newborn , Liver Neoplasms/pathology , Liver Neoplasms/therapy , Male , Retrospective Studies , Survival Rate , Treatment Outcome , Vasodilator Agents/therapeutic useABSTRACT
In the span of the last 48 years, only 33 cases of children with orbital Ewing sarcoma have been reported. This study is to present 3 cases that were admitted to Children's Cancer Hospital Egypt 57357, during the period from 2009 to 2013. We have 2 cases treated using the hospital standard Ewing sarcoma treatment protocol, to completion, whereas the third discontinued treatment. All tumors have confirmed CD99 positivity, although translocation (11;22) was positive in 1 patient and negative in the third. With earlier diagnosis and adequate surgical resection and integration of chemotherapy and radiotherapy 1 patient survived for about 4 years, whereas the other 2 cases died due to disease progression or recurrence.
Subject(s)
Kidney Neoplasms/complications , Orbital Neoplasms/etiology , Orbital Neoplasms/therapy , Sarcoma, Ewing/complications , Child , Child, Preschool , Combined Modality Therapy , Humans , Infant , Kidney Neoplasms/pathology , Male , Neoplasm Staging , Orbital Neoplasms/secondary , Prognosis , Sarcoma, Ewing/pathologyABSTRACT
Background: Pure germinomas account for 40% of pineal tumors and are characterized by the lack of appreciable tumor markers, thus requiring a tumor biopsy for diagnosis. MicroRNAs (miRNA) have emerged as potential non-invasive biomarkers for germ cell tumors and may facilitate the non-invasive diagnosis of pure pineal germinomas. Material and methods: A retrospective chart review was performed on all patients treated at the Children's Cancer Hospital Egypt diagnosed with a pineal region tumor between June 2013 and March 2021 for whom a research blood sample was available. Plasma samples were profiled for miRNA expression, and DESeq2 was used to compare between pure germinoma and other tumor types. Differentially expressed miRNAs were identified. The area under the curve of the receive;r operating characteristic curve was constructed to evaluate diagnostic performance. Results: Samples from 39 pediatric patients were available consisting of 12 pure germinomas and 27 pineal region tumors of other pathologies, including pineal origin tumors [n = 17; pineoblastoma (n = 13) and pineal parenchymal tumors of intermediate differentiation (n = 4)] and others [n = 10; low-grade glioma (n = 6) and atypical teratoid rhabdoid tumor (n = 4)]. Using an adjusted p-value <0.05, three miRNAs showed differential expression (miR-143-3p, miR-320c, miR-320d; adjusted p = 0.0058, p = 0.0478, and p = 0.0366, respectively) and good discriminatory power between the two groups (AUC 90.7%, p < 0.001) with a sensitivity of 25% and a specificity of 100%. Conclusion: Our results suggest that a three-plasma miRNA signature has the potential to non-invasively identify pineal body pure germinomas which may allow selected patients to avoid the potential surgical complications.
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Osteosarcoma is a primary malignant bone tumor affecting adolescents and young adults. This study aimed to identify proteomic signatures that distinguish between different osteosarcoma subtypes, providing insights into their molecular heterogeneity and potential implications for personalized treatment approaches. Using advanced proteomic techniques, we analyzed FFPE tumor samples from a cohort of pediatric osteosarcoma patients representing four various subtypes. Differential expression analysis revealed a significant proteomic signature that discriminated between these subtypes, highlighting distinct molecular profiles associated with different tumor characteristics. In contrast, clinical determinants did not correlate with the proteome signature of pediatric osteosarcoma. The identified proteomics signature encompassed a diverse array of proteins involved in focal adhesion, ECM-receptor interaction, PI3K-Akt signaling pathways, and proteoglycans in cancer, among the top enriched pathways. These findings underscore the importance of considering the molecular heterogeneity of osteosarcoma during diagnosis or even when developing personalized treatment strategies. By identifying subtype-specific proteomics signatures, clinicians may be able to tailor therapy regimens to individual patients, optimizing treatment efficacy and minimizing adverse effects.
Subject(s)
Bone Neoplasms , Osteosarcoma , Adolescent , Child , Young Adult , Humans , Phosphatidylinositol 3-Kinases , Proteomics , Osteosarcoma/genetics , Proteoglycans , Bone Neoplasms/geneticsABSTRACT
AIMS: Ewing's Sarcoma is an extremely aggressive tumor in children. The disease is associated with highly metastatic rate, especially at the time of diagnosis, contributing to a lower survival rate and poor prognosis. The study aimed to identify predictive biomarkers for metastatic Ewing's sarcoma through in-depth analysis of the plasma proteome profile of pediatric Ewing's sarcoma patients. MAIN METHODS: Plasma samples from Ewing's sarcoma patients and control individuals were profiled using both shotgun and dimethyl-labeled proteomics analysis. Subsequently, Ewing's sarcoma patients were further stratified according to their metastatic state and chemotherapy response. Western blot was used for validation. Univariate and multivariate analyses were performed to determine proteome metastasis predictors. Receiver operating characteristic (ROC) analysis was done to assess the diagnostic significance of the potential plasma Ewing's sarcoma biomarkers. KEY FINDINGS: Our results revealed a set of proteins significantly associated with the metastatic Ewing's sarcoma disease profile. These proteins include ceruloplasmin and several immunoglobulins. Additionally, our study disclosed significant differentially expressed proteins in pediatric Ewing's sarcoma, including CD5 antigen-like, clusterin, and dermcidin. Stable isotope dimethyl labeling and western blot further confirmed our results, strengthening the impact of such proteins in disease development. Furthermore, an unbiased ROC curve evaluated and confirmed the predictive power of these biomarker candidates. SIGNIFICANCE: This study presented potential empirical predictive circulating biomarkers for determining the disease status of pediatric Ewing's sarcoma, which is vital for early prediction.
Subject(s)
Bone Neoplasms , Sarcoma, Ewing , Humans , Child , Sarcoma, Ewing/diagnosis , Bone Neoplasms/metabolism , Prognosis , ProteomeABSTRACT
Background: Childhood cancer in low-and middle-income countries is a global health priority, however, the perception that treatment is unaffordable has potentially led to scarce investment in resources, contributing to inferior survival. In this study, we analysed real-world data about the cost-effectiveness of treating 8886 children with cancer at a large resource-limited paediatric oncology setting in Egypt, between 2013 and 2017, stratified by cancer type, stage/risk, and disease status. Methods: Childhood cancer costs (USD 2019) were calculated from a health-system perspective, and 5-year overall survival was used to represent clinical effectiveness. We estimated cost-effectiveness as the cost per disability-adjusted life-year (cost/DALY) averted, adjusted for utility decrement for late-effect morbidity and mortality. Findings: For all cancers combined, cost/DALY averted was $1384 (0.5 × GDP/capita), which is very cost-effective according to WHO-CHOICE thresholds. Ratio of cost/DALY averted to GDP/capita varied by cancer type/sub-type and disease severity (range: 0.1-1.6), where it was lowest for Hodgkin lymphoma, and retinoblastoma, and highest for high-risk acute leukaemia, and high-risk neuroblastoma. Treatment was cost-effective (ratio <3 × GDP/capita) for all cancer types/subtypes and risk/stage groups, except for relapsed/refractory acute leukaemia, and relapsed/progressive patients with brain tumours, hepatoblastoma, Ewing sarcoma, and neuroblastoma. Treatment cost-effectiveness was affected by the high costs and inferior survival of advanced-stage/high-risk and relapsed/progressive cancers. Interpretation: Childhood cancer treatment is cost-effective in a resource-limited setting in Egypt, except for some relapsed/progressive cancer groups. We present evidence-based recommendations and lessons to promote high-value in care delivery, with implications on practice and policy. Funding: Egypt Cancer Network; NIHR School for Primary Care Research; ALSAC.
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INTRODUCTION: Malignant pediatric lymphoma accounts for 10-15% of all pediatric cancers, (representing 2-3% of all malignancies), with a peak incidence between 5-9 years. Chemotherapy is usually the first and most common mode of treatment. The choice of treatment and prediction of prognosis depend on the histological type of tumor, initial staging, evaluating treatment response, and detection of early recurrence. Conventional imaging modalities have many limitations. PET/CT is more accurate, however so far the literature lacks the results of a large group of patients. AIM OF STUDY: To report the role of PET/CT in the above-mentioned objectives at the newly established Children's Cancer Hospital in Cairo, Egypt, which is one of the busiest dedicated pediatric oncology centers of such purposes in the world. All findings were proven by histopathology, clinically, and by clinical follow-up. PATIENT POPULATION: A total of 152 patients (35 girls and 117 boys) with histologically proven malignant lymphoma (117 HD, 35 NHL) were included in this study. They were divided into four groups. Group I: 41 patients for initial staging. Group II: 51 patients for evaluating early treatment response after two to three cycles of chemotherapy. Group III: 42 patients for evaluating treatment response 4-8 weeks after the end of their treatment. Group IV: 18 patients evaluated for long-term follow-up. Results of PET/CT were compared with the other conventional imaging modalities (CIM). RESULTS: The sensitivity, specificity, accuracy, and positive and negative predictive values of PET/CT and CIM were as follows: In Group I: PET/CT modified staging and treatment in 11 out of 41 cases (26.8%), upstaged 5(12.2%) patients and down-staged six (14.6%) patients. Group II: 100%, 97.7%, 98%, 85.7%, 100%, respectively, for PET/CT and 83%, 66.6%, 68.6%, 25%, 96.7% for CIM respectively Group III: At the end of chemotherapy 100%, 90.9%, 92.8%, 75%, 100%, respectively, for PET/CT and 55.5%, 57.5%, 57.1%, 26.3%, 82.6% for CIM, respectively. Group IV: For long-term follow-up, all the parameters scored 100% for PET/CT, 100%, 38.4%, 72.2%, 50%, 100% for CIM, respectively. CONCLUSION: PET/CT in pediatric lymphoma is more accurate than CIM. We recommend that it should be the first modality for all purposes in initial staging, evaluating treatment response and follow-up.
Subject(s)
Lymphoma/diagnosis , Positron-Emission Tomography/methods , Subtraction Technique , Tomography, X-Ray Computed/methods , Adolescent , Child , Child, Preschool , Female , Humans , Male , Reproducibility of Results , Sensitivity and SpecificityABSTRACT
BACKGROUND: Complete metastasectomy is the best predictor of survival in patients with osteosarcoma pulmonary metastases. There has been some controversy in the literature regarding the prognostic significance of the timing of occurrence of lung metastasis. METHODS: We reviewed the clinical course of all osteosarcoma patients with pulmonary metastases treated by metastasectomy in our hospital from January 2008 through December 2016. Each patient who underwent metastasectomy was placed into one of three groups based on whether lung metastases were present at initial presentation (Group 1), developed during chemotherapy (Group 2), or appeared after completion of chemotherapy (Group 3). Data were obtained retrospectively and follow-up was obtained until the end of June 2017. RESULTS: We identified 170 patients with pulmonary nodules of whom 99 (58.2%) underwent at least one metastasectomy (149 thoracotomies). Eleven patients had benign pulmonary nodules and were excluded. The other 88 patients were classified as Group 1 (37), Group 2 (18) or Group 3 (33). The median follow-up was 35â¯months (range 8 to 99). Postmetastasis 5-year overall survival (OS) was 38.1⯱â¯6.4%; event-free survival (EFS) was 25⯱â¯5.3%. By group, postmetastasis 5-year OS and EFS were 34.3⯱â¯13% and 18⯱â¯9.3% in Group 1, 8⯱â¯6.5% and 6.5⯱â¯5% in Group 2, and 52⯱â¯11.4% and 25⯱â¯9% in Group 3 (Pâ¯<â¯0.001). In univariate analysis, the only significant factors associated with survival were timing of occurrence of lung metastasis and the number of lung nodules found. CONCLUSION: The timing of occurrence of lung metastasis is an important prognostic factor among osteosarcoma patients eligible for metastasectomy. Patients whose metastases occurred during chemotherapy had the worst survival. LEVEL OF EVIDENCE: Level II.
Subject(s)
Bone Neoplasms/pathology , Lung Neoplasms/secondary , Metastasectomy/methods , Osteosarcoma/pathology , Adolescent , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Bone Neoplasms/mortality , Bone Neoplasms/surgery , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Lung Neoplasms/mortality , Lung Neoplasms/surgery , Male , Neoadjuvant Therapy/methods , Neoplasm Staging , Osteosarcoma/mortality , Osteosarcoma/surgery , Prognosis , Retrospective Studies , Survival Analysis , Thoracotomy , Time FactorsABSTRACT
The aim of our study was to evaluate the role of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) in prediction of response to neoadjuvant chemotherapy (NAC) in pediatric osteosarcoma (OS) patients compared to percentage of tumor necrosis after surgical excision of the tumor. Forty-six pediatric OS patients treated with neoadjuvant chemotherapy and surgery were underwent PET/CT and MRI before, after 3 cycles, and after the completion of neoadjuvant chemotherapy. Imaging parameters include maximum standardized uptake value (SUVmax1, 2, and 3), tumor liver ratio (TLR 1, 2, and 3), and MRI tumor volume (MRTV 1, 2, and 3) at initial assessment before starting NAC, after finishing three cycles and after finishing 6 cycles before tumor excision, respectively. Cutoff values of the PET/CT and MRI parameters were determined using receiver operating characteristic (ROC) curve analysis and percentage of tumor necrosis of postsurgical specimen. Fourteen patients were good responders (30.4%), with more than 90% tumor necrosis, while 31 patients were poor responders (67.4%). The results of one patient were missed. We noticed that higher sensitivity for detecting poor responders was detected by SUVmax3/1, TLR3/1, and MRTV2/1 ratio cutoff values, while higher specificity was detected by TRL2 and SUVmax3 cutoff values. ROC curve analysis of MRTV2/1 and MRTV3/1 ratio was fair in predicting poor responders. PET/CT parameters are capable of predicting histological response to NAC in OS patients with overall sensitivity and specificity higher than MRI parameters.
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INTRODUCTION: Anthracycline chemotherapy contributes to improved outcomes in Ewing sarcoma; however, the most feared complication is cardiotoxicity. Echocardiograms were routinely used to monitor cardiac function after anthracycline treatment. Nevertheless, indices chosen to assess cardiac toxicity vary significantly among different centers, and no uniform protocol has been accepted as ideal. METHODS: This retrospective study included children with Ewing sarcoma treated at Children's Cancer Hospital Egypt over 4years. All echocardiograms and related clinical assessments were reviewed. RESULTS: In total, 149 patients (median age 11years; range 1-18years) were included. Although all patients had a reduced ejection fraction compared with their baseline echocardiogram, only 39 patients developed cardiotoxicity (26%): 43% acute-onset, 36% chronic early-onset, and 21% chronic late-onset. There were no statistically significant association between the frequency of myocardial dysfunction and risk factors, including age, sex, follow-up duration, cumulative doxorubicin dose, and mediastinal irradiation. Over one-third (39%) of the patients with cardiac toxicity regained normal cardiac parameters, whereas seven patients died of acute cardiac toxicity. CONCLUSION: The routine use of echocardiography to screen for anthracycline-induced cardiac toxicity before and during chemotherapy identified myocardial dysfunction. Early medical intervention can improve cardiac parameters. Improved screening techniques with better sensitivity and predictability are needed.
Subject(s)
Anthracyclines/adverse effects , Antineoplastic Agents/adverse effects , Heart Diseases/diagnosis , Heart Diseases/etiology , Sarcoma, Ewing/complications , Adolescent , Anthracyclines/therapeutic use , Antineoplastic Agents/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cardiotoxicity , Child , Child, Preschool , Echocardiography , Female , Humans , Infant , Infant, Newborn , Male , Retrospective Studies , Risk Assessment , Risk Factors , Sarcoma, Ewing/drug therapy , Severity of Illness Index , Ventricular Dysfunction, LeftABSTRACT
PURPOSE: Was to evaluate the outcome of multimodality treatment in resectable primary Ewing sarcoma/primitive neuroectodermal tumor ES/PNET of the ribs and role of thoracoscopy in facilitating resection of these tumors. PATIENTS AND METHODS: This was a retrospective study including 22 patients with primary ES/PNET of the ribs surgically treated at Children's Cancer Hospital Egypt (CCHE) between January 2008 until the end of December 2014. RESULTS: Median age was 8.5years (range 5months to 16years.). All patients received neoadjuvant chemotherapy. Thoracoscopic exploration was performed in 15 (68%) patients. Resection included 1,2,3 and 4 ribs in (7,4,8 and 3 patients) respectively, parts of the diaphragm (3 patients), wedge resection of the lung (10 patients) and pleural nodules (2 patients). Primary closure was feasible in 11 patients and rib transposition was done in one patient. Reconstruction by proline mesh covered by muscle flap was done in 10 patients. Margins were microscopically positive in 3 patients and close in 2 patients. Postoperative radiotherapy was given in 8 patients. With a median follow-up of 38.5months, the 3-year event -free survival (EFS) and overall survival(OS) rates were 31.6% and 55.6%, respectively. CONCLUSION: Multimodality treatment is essential in the management of ES-PNET of the ribs. Neoadjuvant chemotherapy facilitates adequate resection. The role of thoracoscopy and the indications of postoperative radiotherapy need further evaluation.