A rare case of Ewing's sarcoma of the maxillary sinus.

Ewing's sarcoma is generally an aggressive, poorly differentiated bone and soft tissue tumor affecting children and young adults, it accounts for 4%-6% of all primary bone tumors and primary facial locations occur in only 1%-4% of all cases, primarily in the mandible and calvaria. Involvement of the paranasal sinuses is rare. Here we report the case of an 11-year-old girl with no medical, surgical, or traumatic history, who presented for 1 month and progressive evolution of swelling of the left cheek, associated with pain, nasal obstruction, rhinorrhea, and a slight weight loss not quantified. A craniofacial computed tomography (CT) scan showed a mixed lytic and condensing lesional tissue process centered on the left maxillary sinus, heterogeneously enhanced after contrast injection, lysing the walls of the sinus extended to the homolateral nasal cavity and slightly infiltrating the adjacent soft tissues. An incisional biopsy was performed and the pathological study proved that it was Ewing's sarcoma. She was put on neoadjuvant chemotherapy using 6 courses of vincristine, doxorubicin, ifosfamide, etoposide which resulted in a partial regression of the tumor size by 50%. Then the patient was put on combined chemotherapy and radiotherapy. A follow-up CT scan after 6 courses of vincristine, actinomycin, cyclophosphamide, and 17 sessions of radiotherapy showed lesion stability. Maxillary Ewing's sarcoma is a rare and aggressive tumor. Therefore, early diagnosis, combination therapy, and long-term follow-up are suggested in such cases to improve the survival rate.

A rare case of renal tumor in children: Clear cell sarcoma with an unusual presentation.

Clear cell sarcoma of the kidney is the most frequently misdiagnosed renal tumor in children. We report the case of a 6-year-old boy with clear cell sarcoma of the kidney with an unusual presentation, including a primary tumor of the left kidney with metastasis in the homolateral psoas muscle. The renal tumor was revealed by abdominal mass without hematuria. In a review of the literature. Clear cell sarcoma of the kidney is most commonly associated with bone and lung metastases. Muscular metastasis at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of clear cell sarcoma of the kidney. This also illustrates clear cell sarcoma of the kidney's ability to metastasize to other sites including the muscular. These tumors present a diagnostic challenge for the radiologist who should be aware of this entity to differentiate it from other renal tumors which are more frequent at this age. We aim to report the clinical, radiological features, and pathological presentation of this entity.

A rare presentation of a spinal diffuse midline glioma in a child: a case report.

Our patient had an extremely rare type of pediatric Diffuse Midline Glioma (DMG) with modified H3 K27 that occurred in the cervical spinal cord. Due to its location in the spinal cord, slow clinical presentation with torticollis for 7 months, and the non-specific radiological appearance of this tumour, it was initially considered to be a low-grade glioma. Based on imaging findings, the neurosurgery team performed a complete surgical resection, but the pathological features were consistent with a high-grade, diffuse midline glioma. Therefore, we are reporting a case of an altered high-grade DMG H3K27 glioma, which is difficult to diagnose due to its slow clinical symptoms which caused a delay in diagnosis, non-specific imaging, and with difficulty in accessing histopathological markers in low and middle income countries (LMIC).

Human leukocyte antigen immunization in transfusion-dependent Moroccan patients with beta-thalassemia major: prevalence and risk factors.

INTRODUCTION: Beta-thalassemia major patients need a regular blood transfusion to have an initial normal growth. However, these patients have an increased risk of developing alloantibodies. Our main goal was to study HLA alloimmunization in Moroccan Beta-thalassemia patients by confronting it with transfusion and demographic criteria, exploring the involvement of HLA typing profile in the development of HLA antibodies and in turn determining risk factors for their development. METHODS: The study consisted of 53 Moroccan pediatric patients with Beta-thalassemia major. Screening for HLA alloantibodies was performed using Luminex technology Whereas HLA genotyping was done with sequence-specific primers (PCR-SSP). RESULTS: In this study, 50.9% of patients have been identified as positive for HLA antibodies, with 59.3% having both HLA Class I and Class II antibodies. A significant increase frequency of DRB1*11 allele was revealed in non-immunized patients (34.6% vs. 0%, p = 0.001). Our results also revealed that the majority of our HLA immunized patients were women (72.4% vs. 27.6%, p = 0.001), and transfused with more than 300 units of RBC units (66.7% vs. 33.3%, p = 0.02). There were statistically significant differences when comparing these frequencies. CONCLUSIONS: This paper revealed that the transfusion dependent Beta-thalassemia major patients are exposed to risk of developing HLA antibodies following transfusions with leukoreduced RBC units. The HLA DRB1*11 was a protective factor against HLA alloimmunization in our beta-thalassemia major patients.

Saphenous Nerve Schwannoma: A Rare Differential Diagnosis of Knee Pain in Children.

Schwannomas are uncommon benign tumors of the peripheral nerves with a low risk of malignant transformation. They rarely affect children, can affect any part of the body but rarely occur in the lower extremity and typically present with a palpable mass, pain or neurological signs. Imaging helps to orient the diagnosis and anatomopathological examination helps to confirm it. We report a case of a 12-year-old girl who presented with left knee pain with subcutaneous mass overlying the tibial tuberosity medially. Clinical examination revealed a positive Tinel's sign. Magnetic resonance imaging (MRI) of the knee was performed, which revealed an encapsulated subcutaneous soft tissue mass overlying the tibial tuberosity medially, eccentric to the course of the infrapatellar branch of the saphenous nerve. The patient was operated with total intracapsular excision of the lesion and the anatomopathological study of the surgical specimen came back in favor of a schwannoma. Postoperatively, the patient showed a good recovery with disappearance of pain and swelling.

Evaluating the baseline survival outcomes of the "six Global Initiative for Childhood Cancer index cancers" in Africa.

Limited survival data for the six Global Initiative for Childhood Cancer (GICC) priority cancers are available in Africa. Management of pediatric malignancies in Africa is challenging due to lack of resources, setting-specific comorbidities, high rates of late presentation and treatment abandonment. Reporting of outcome data is problematic due to the lack of registries. With the aim of evaluating the feasibility of baseline outcomes for the six index cancers, we present a descriptive analysis of respective survival rates in Africa. The survival rates were between 18% (lower middle-income countries) to 82.3% (upper middle-income countries) for acute lymphoblastic leukemia, between 26.9% (low-income countries) to 77.9% (upper middle-income countries) for nephroblastoma, between 23% (low-income countries) to 100% (upper middle-income countries), for retinoblastoma, 45% (low-income countries) to 95% (upper middle-income countries) for Hodgkin lymphoma and 28% (low-income countries) to 76% (upper middle-income countries) for Burkitt lymphoma. Solutions to improve survival rates and reported outcomes include establishing and funding sustainable registries, training and to actively include all countries in consortia from different African regions.HighlightsContinental differences in childhood cancer management such lack of resources, setting-specific comorbidities, high rates of late presentation and treatment abandonment, present challenges to the achievement of Global Initiative for Childhood Cancer goals.The available data registries do not adequately inform on the true incidences and outcomes of childhood cancers in Africa.The pathophysiology of some childhood cancers in Africa are associated with high-risk prognostic factors.Outcomes can be improved by greater regional collaboration to manage childhood cancer based on local resources and tumor characteristics.Some individual countries have reached the Global Initiative for Childhood Cancer goals for single cancers and it should be possible for more African countries to follow suit.

Current status of African pediatric oncology education efforts aligned with the Global Initiative for Childhood Cancer.

Education of the pediatric oncology workforce is an important pillar of the World Health Organization CureAll technical package. This is not only limited to healthcare workers, but all stakeholders in the childhood cancer management process. It includes governmental structures, academic institutions, parents and communities. This review evaluated the current educational and advocacy training resources available to the childhood cancer community, the contribution of SIOP Africa in the continental educational needs and evaluated future needs to improve the management of pediatric malignancies in reaching the Global Initiative for Childhood Cancer goals. Childhood cancer, unlike adult cancers, has not been prioritized in African cancer control plans nor the teaching and advocacy surrounding pediatric oncology. The availability of formal training programs for pediatric oncologists, pediatric surgeons and radiotherapy specialists are limited to particular countries. In pharmacy and nutritional services, the exposure to pediatric oncology is limited while training in advocacy doesn't exist. Many nonacademic stakeholders are creating the opportunities in Africa to gain experience and train in these various fields, but formal training programs should still be advocated for. LEARNING POINTSThe African continent has various resources to increase the capacity of childhood cancer care stakeholders to increase their knowledge.African pediatric oncology teams rely on a multitude of international sources for training while developing their own.There is a greater need for formal, standardized cancer training especially for pediatric surgeons, radio-oncologists and nurses.Greater inclusion of pathologists, pediatric oncology pharmacists and dieticians into multidisciplinary care and childhood cancer training should be facilitated and resourced.Successful advocacy programs and tool kits exist in parts of Africa, but the training in advocacy is still underdeveloped.

Conference report on the 14th International Society of Paediatric Oncology African Continental Meeting, 16-18 March 2022, Kampala, Uganda.

Together with the Africa Continental Branch of the International Society of Paediatric Oncology (SIOP Africa), the Uganda Cancer Institute, a tertiary governmental institution for specialised cancer care services, research and training, hosted the 14th continental meeting of SIOP Africa from the 16-18 March 2022. Under the conference theme, 'Innovate for Africa', the hybrid conference brought together close to 400 international delegates to discuss innovations and experiences, as well as share the latest research in the field of paediatric oncology. The World Health Organisation 2030 Global Initiative for Childhood Cancer provided the main starting point for the conference with a comprehensive pre-conference workshop programme, from multiple stakeholders and organisations and the themes for the plenaries towards improving survival to the main breakout sessions. Delegates discussed various ways of improving outcomes in Africa, despite the challenges faced individually and collectively ranging from education, management systems and treatment guidelines to future governmental and NGO involvement in African cancer care. The main achievements of the conference were various commitments for collaboration, investing in junior investigators, development of registries and systems for improved childhood care on the African continent, while working towards greater access to advanced management options such as targeted therapies and bone marrow transplant services.

Implementing the WHO Global Initiative for Childhood Cancer in Morocco: Survival study for the six indexed childhood cancers.

BACKGROUND: In 2018, the World Health Organization (WHO) launched the Global Initiative for Childhood Cancer (GICC). The goal is to achieve a global survival rate of at least 60% for all children with cancer by 2030. Morocco was designated as a pilot country for this initiative. PROCEDURE: This retrospective study included a cohort of children aged 0-15 years, with one of the six indexed cancers (acute lymphoblastic leukemia [ALL], Burkitt lymphoma [BL], Hodgkin lymphoma, retinoblastoma [RB], Wilms tumor or nephroblastoma, low-grade glioma), diagnosed between January 1, 2017 and December 31, 2019 at the six Moroccan Pediatric Hematology and Oncology units. Patients were followed-up until August 31, 2020. The Kaplan-Meier method was used to estimate survival rates, the log-rank test for comparing survival curves, and the Cox model for identifying prognostic factors. RESULTS: Data on 878 patients were included in the study. The most frequently reported cancer type was ALL (n = 383, 43.6%), followed by Wilms tumor (n = 139, 15.8%) and BL (n = 133, 15%). Most patients were less than 5 years of age (n = 446, 50.9%) and the male/female ratio was 1.46. The 1, 2, and 3-year overall survival rates were 80.1%, 73.6%, and 68.2%, respectively. In a multivariable Cox regression model, care center, cancer type, age group, and distance to the care center were statistically significantly associated to survival. Patients aged 10 years and older and patients living more than 100 km from the care center were more likely to die (respectively, HR = 1.39, p = .045 and HR = 1.44, p = .010). CONCLUSION: The reported results represent the baseline for measuring the impact of GICC implementation in Morocco.

Cancer in sub-Saharan Africa: a Lancet Oncology Commission.

In sub-Saharan Africa (SSA), urgent action is needed to curb a growing crisis in cancer incidence and mortality. Without rapid interventions, data estimates show a major increase in cancer mortality from 520 348 in 2020 to about 1 million deaths per year by 2030. Here, we detail the state of cancer in SSA, recommend key actions on the basis of analysis, and highlight case studies and successful models that can be emulated, adapted, or improved across the region to reduce the growing cancer crises. Recommended actions begin with the need to develop or update national cancer control plans in each country. Plans must include childhood cancer plans, managing comorbidities such as HIV and malnutrition, a reliable and predictable supply of medication, and the provision of psychosocial, supportive, and palliative care. Plans should also engage traditional, complementary, and alternative medical practices employed by more than 80% of SSA populations and pathways to reduce missed diagnoses and late referrals. More substantial investment is needed in developing cancer registries and cancer diagnostics for core cancer tests. We show that investments in, and increased adoption of, some approaches used during the COVID-19 pandemic, such as hypofractionated radiotherapy and telehealth, can substantially increase access to cancer care in Africa, accelerate cancer prevention and control efforts, increase survival, and save billions of US dollars over the next decade. The involvement of African First Ladies in cancer prevention efforts represents one practical approach that should be amplified across SSA. Moreover, investments in workforce training are crucial to prevent millions of avoidable deaths by 2030. We present a framework that can be used to strategically plan cancer research enhancement in SSA, with investments in research that can produce a return on investment and help drive policy and effective collaborations. Expansion of universal health coverage to incorporate cancer into essential benefits packages is also vital. Implementation of the recommended actions in this Commission will be crucial for reducing the growing cancer crises in SSA and achieving political commitments to the UN Sustainable Development Goals to reduce premature mortality from non-communicable diseases by a third by 2030.

Non Hodgkin Lymphoma Among Children: Pathological Aspects and Diagnostic Challenges.

Non-Hodgkin lymphoma (NHL) are common malignancies in children. Available data on clinico-pathological aspects of pediatric NHL in developping countries are limited and diagnostic approach appears more delicate with absence of molecular studies. The objectives of our study are: analyzing the pathological spectrum of NHL among children and highlighting challenges in the diagnosis including: limited biopsic material; unususal subtyptes, age group, or localization. We retrospectively analyzed clinico pathological characteristics of 101 NHL's cases among children diagnosed in the Pediatric's pathology unit over a period of 4 years There were 78 (77.2%) male and 23 (22.8%) female. The median age was 7.2 years. The most common histologic subtypes of NHL were Burkitt lymphoma in 65 patients (64.4% ); followed by lymphoblastic lymphoma in 22 patients, large B-cell lymphoma in 9 patients ( 8.9%); anaplastic T cell lymphoma in 3 patients; NOS mature T cell lymphoma and pediatric type follicular lympoma in 1 patient each. In conclusion, this study Morocco illustrates the pattern of distribution of NHL and emphasizes challenges in the diagnosis of these neoplasms.

The prevalence of anti-factor VIII and anti-factor IX antibodies among patients with hemophilia in Rabat, Morocco: a single center experience.

The emergence of anti-factor VIII and anti-factor IX antibodies in hemophilia A or B is the most serious complication of hemophilia. We aim to expose through a series of patient's data, collected between 02/2009 and 02/2016 in the pediatric service of university hospital of Rabat, Morocco, the epidemiological and clinical characteristics of these patients, and to highlight the therapeutic difficulties encountered during their treatment. Out of 120 hemophiliac patients, we included 22 hemophiliac patients (18.33%, p<0.004) who developed an antibody, 21 patients with hemophilia A. Among the patients, 54.5% (n=12) exhibited moderate hemophilia, while 45.5% (n=10) had major hemophilia. The average age at diagnosis is estimated to 12±6.6 years. The circumstances of diagnosis were dominated by therapeutic inefficiency (63.64% (n=14)), then came dental extraction (9.09% (n=2)), preoperative assessment 22.73% (n=5) and hemophiliac arthropathy in a single case. The titration of antibodies in a 12-person sample ranged from 0.6 UB to 84 UB, of which (41.67% (n=5)) were low responders. The therapeutic treatment was based on fresh frozen plasma (54.55% (n=7)), recombinant activated factor VII (18.2% (n=4)), recombinant activated factor VII and PFC (18.2% (n=4)), and induction of immune tolerance. The occurrence of an inhibitory antibody represents the major residual complication of replacement therapy.

Mapping Pediatric Oncology Clinical Trial Collaborative Groups on the Global Stage.

PURPOSE: The global pediatric oncology clinical research landscape, particularly in Central and South America, Africa, and Asia, which bear the highest burden of global childhood cancer cases, is less characterized in the literature. Review of how existing pediatric cancer clinical trial groups internationally have been formed and how their research goals have been pursued is critical for building global collaborative research and data-sharing efforts, in line with the WHO Global Initiative for Childhood Cancer. METHODS: A narrative literature review of collaborative groups performing pediatric cancer clinical research in each continent was conducted. An inventory of research groups was assembled and reviewed by current pediatric cancer regional and continental leaders. Each group was narratively described with identification of common structural and research themes among consortia. RESULTS: There is wide variability in the structure, history, and goals of pediatric cancer clinical trial collaborative groups internationally. Several continental regions have longstanding endogenously-formed clinical trial groups that have developed and published numerous adapted treatment regimens to improve outcomes, whereas other regions have consortia focused on developing foundational database registry infrastructure supported by large multinational organizations or twinning relationships. CONCLUSION: There cannot be a one-size-fits-all approach to increasing collaboration between international pediatric cancer clinical trial groups, as this requires a nuanced understanding of local stakeholders and resources necessary to form partnerships. Needs assessments, performed either by local consortia or in conjunction with international partners, have generated productive clinical trial infrastructure. To achieve the goals of the Global Initiative for Childhood Cancer, global partnerships must be sufficiently granular to account for the distinct needs of each collaborating group and should incorporate grassroots approaches, robust twinning relationships, and implementation science.

Impact of the COVID-19 pandemic on pediatric oncology providers globally: A mixed-methods study.

BACKGROUND: Coronavirus disease 2019 (COVID-19) disrupted pediatric oncology care globally, increasing demands on health care providers (HCPs) who adapted to continue care. This study sought to characterize the pandemic's impact on pediatric oncology HCPs worldwide. METHODS: A 60-item survey focused on changes to clinical care, resources, and effects on clinicians. A diverse subgroup of institutions was purposefully selected for focus groups that explored teamwork, communication, and changes to care delivery. RESULTS: The survey included 311 responses from 213 institutions representing 79 countries. Sixteen institutions participated in 19 multidisciplinary focus groups in 8 languages. Decreased clinical staff availability was cited by 51% of institutions as a major impact. Staffing modifications included decreased provider availability (66% of institutions), roles or responsibility changes, and transfer outside the specialty. Physical effects included frequent COVID-19 illness; 8% of respondents reported HCP deaths. Fifty percent of providers did not have the necessary personal protective equipment. HCPs also experienced psychological distress and financial concerns. Findings indicated more frequent impact on nurses than other providers. Impacts were described across all hospital resource levels, with staffing modifications more frequent in countries with higher COVID-19 incidence (P < .001) and mortality rate (P = .004). Focus groups revealed negative impacts were stabilized by increased teamwork, communication, contributions outside usual roles, policies aimed at optimizing safety, and feeling that they were contributing. CONCLUSIONS: COVID-19 had a profound impact on the pediatric oncology workforce, creating challenging modifications to staffing and resulting in physical, psychological, and financial distress. Despite these challenges, HCPs caring for children with cancer came together to continue to provide high-quality care.

Primitive myxoid mesenchymal tumor of infancy: Case report and review of the literature.

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is an extremely rare soft tissue tumor with only a few cases. Herein, we report a case of 5-months-old girl presenting with a soft tissue mass on the sole of the left foot that recurred 2months after a first resection. The Doppler ultrasound imaging showed abundant blood flow in the tumor. Magnetic resonance imaging (MRI) showed a well demarcated tumor on the sole of the left foot. The surgically resected tumor consisted of primitive mesenchymal tumor cells dispersed in a myxoid background with delicate small blood vessels. Immunohistochemically, tumor cells expressed vimentin but were negative for AE1/AE3, Desmin, Muscle-Specific Actin, MyoD1, Myogenin, CD34, S-100 protein and CD99. Fluorescence in situ hybridization (FISH) showed absence of chromosomal translocation involving SYT and ETV6. Thus, the patient was diagnosed with a primitive myxoid mesenchymal tumor of infancy. To our knowledge, this is the first case of PMMTI affecting the sole of the foot.

Sex, gender, and retinoblastoma: analysis of 4351 patients from 153 countries.

OBJECTIVE: To investigate in a large global sample of patients with retinoblastoma whether sex predilection exists for this childhood eye cancer. METHODS: A cross-sectional analysis including 4351 treatment-naive retinoblastoma patients from 153 countries who presented to 278 treatment centers across the world in 2017. The sex ratio (male/female) in the sample was compared to the sex ratio at birth by means of a two-sided proportions test at global level, country economic grouping, continent, and for selected countries. RESULTS: For the entire sample, the mean retinoblastoma sex ratio, 1.20, was higher than the weighted global sex ratio at birth, 1.07 (p < 0.001). Analysis at economic grouping, continent, and country-level demonstrated differences in the sex ratio in the sample compared to the ratio at birth in lower-middle-income countries (n = 1940), 1.23 vs. 1.07 (p = 0.019); Asia (n = 2276), 1.28 vs. 1.06 (p < 0.001); and India (n = 558), 1.52 vs. 1.11 (p = 0.008). Sensitivity analysis, excluding data from India, showed that differences remained significant for the remaining sample (χ2 = 6.925, corrected p = 0.025) and for Asia (χ2 = 5.084, corrected p = 0.036). Excluding data from Asia, differences for the remaining sample were nonsignificant (χ2 = 2.205, p = 0.14). CONCLUSIONS: No proof of sex predilection in retinoblastoma was found in the present study, which is estimated to include over half of new retinoblastoma patients worldwide in 2017. A high male to female ratio in Asian countries, India in specific, which may have had an impact on global-level analysis, is likely due to gender discrimination in access to care in these countries, rather than a biological difference between sexes.

Pediatric cancer care in Africa: SIOP Global Mapping Program report on economic and population indicators.

INTRODUCTION: Inalignment with the World Health Organization (WHO) Global Initiative for Childhood Cancer (GICC), the International Society of Pediatric Oncology initiated a program to map global pediatric oncology services. As survival rates in Africa are low and data are scant, this continent was mapped first to identify areas with greatest need. METHODS: Beginning November 2018, an electronic survey was sent to all known stakeholders, followed by email communications and internet searches to verify data. Availability of pediatric oncologists, chemotherapy, surgical expertise, and radiotherapy was correlated with geographic region, World Bank income status, Universal Health Coverage, population < 15 and < 24 years, percentage of gross domestic product spent on healthcare, and Human Development Index (HDI). RESULTS: Responses were received from 48/54 African countries. All three treatment modalities were reportedly available in 9/48 countries, whereas seven countries reported no pediatric oncology services. Negative correlations were detected between provision of all three services and geographic region (P = 0.01), younger median population age (P = 0.002), low-income country status (P = 0.045), and lower HDI (P < 0.001). CONCLUSION: This study provides a comprehensive overview of pediatric oncology care in Africa, emphasizing marked disparities between countries: some have highly specialized services, whereas others have no services. A long-term strategy to eliminate disparities in African pediatric cancer care should be aligned with the WHO GICC aims and facilitated by SIOP Africa. MEETING ABSTRACTS: SIOP maps pediatric oncology services in Africa to address inequalities in childhood cancer services. Geel J, Ranasinghe N, Davidson A, Challinor J, Howard S, Wollaert S, Myezo K, Renner L, Hessissen L, Bouffet E. 51st Annual Congress of the International Society of Paediatric Oncology (SIOP), Lyon, France, October 2019. Pediatric Blood and Cancer Vol 66 S219-S219. Pediatric cancer care in Africa: SIOP Global Mapping Program report on economic and population indicators.