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Background: The survival of children with acute lymphoblastic leukemia (ALL) has greatly improved in recent decades. Aim: We aimed at evaluating the outcome of children with ALL and their prognostic factors. Materials & Methods: A prospective study included 200 newly diagnosed pediatric ALL patients from 2009 to 2017. All patients were treated according to the modified total therapy study XIIIB for higher risk ALL adopted from St. Jude Children's Research hospital (SJCRH), USA. Results: Favorable age (< 10 y) and total leucocytic count (TLC) (≥ 50x109/L) occurred in 75% and 69%, respectively. B-precursor ALL represented 82.5%. Central nervous system (CNS) involvement occurred in 4.5%. High-risk patients represented 47%. Complete remission was achieved in 92.5% and was significantly affected by the early morphologic response of BM d15, while it wasn’t related to the phenotype. Eight- year overall survival, disease-free survival, and event-free survival were 77 ± 3%, 70 ± 4%, and 66 ± 3%, respectively. Adverse events included induction deaths (4.5 %(, refractory leukemia (3%), relapse (23.2%), secondary AML (0.5%), and deaths in remission (3.5%). By multivariate analysis, unfavorable age group ≥ 10 years, hyperleukocytosis, and patients with slow early response were the only ones associated with unfavorable impact on the outcome. There was statistical significant difference between standard and high risk for B-precursor ALL but not for T- precursor ALL. Conclusion: We concluded that treating children with ALL through modified TXIIIB protocol improved the survival at our institute than previous and the early response to treatment has a strong implication on the outcome.

BackgroundThe travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe.MethodsA cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries.ResultsCapture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI −12.4 to −5.4, pConclusionsFewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

Background Malignant ovarian germ cell tumors (MOGCTs) are rare and represent 1–1.5% of all cancers in children and adolescents. The aim of this study is to analyze the clinicopathological pattern at presentation and management and outcome of MOGCTs in children and adolescents. Patients and methods Retrospective study included all girls diagnosed with MOGCTs between January 2005 and January 2015 in Pediatric and Surgical Oncology Departments at South Egypt Cancer Institute, Assiut University. Data were collected from patients' records including initial presentation, diagnosis (tumor markers and imaging), surgical staging and pathologic types. Management (surgical and chemotherapy details) and outcomes were also analyzed. Results Forty girls aged between 4 to 17years (mean age of 9.5years) with diagnosis of MOGCTs during study period were included. The most common presenting symptoms and signs were abdominal swelling, abdominal pain, and pelvic mass. Precocious puberty was noted in two patients. Surgical interventions in most patients were unilateral salpingo-oophorectomy ( n =20). Early stages I and II were reported in 15 and 12 patients respectively, while 10 patients had stage-III disease and 3 patients had stage IV. Yolk sac tumors were reported in 27.5% of patients. All patients were treated with platinum based chemotherapy. The 7-year overall survival was higher for patients with early stages (I and II) compared with advanced stages (III and IV) (100% versus 30.8% respectively. Conclusions Early presentation with appropriate management using fertility sparing surgery and platinum-based chemotherapy provides excellent survival with fertility preservation in children and adolescents. Based on the lower survival of patients with advanced disease, efforts should focus on increasing the awareness in the community of the importance of early diagnosis of ovarian tumors. Level of evidence II (retrospective study).

Background and Objectives: Risk-adapted therapy for children with HL is directed toward high survival, minimal toxicity and optimal quality of life, with long term follow up. We assess the impact of prognostic factors associated with local treatment failure of pediatric HL patients with unfavorable criteria treated with combined modality: Alternating ABVD (Doxorubicin, Bleomycin, Vinblastine and Decarbazine) and COEP (Cyclophosphamide, Oncovin, Etoposide and Prednisone) chemotherapy and response-based, involved-field radiation for newly diagnosed unfavorable pediatric HL patients, also will detect toxicities and long-term complications observed in the patients. Methods: This prospective study was carried out from January 2010 to January 2018, with a median follow up of 74 months (range 8 - 103 months). 54 patients were eligible for this study stratified into two groups: intermediate risk (IR) and high-risk group (HR). Patients were treated with (4 - 6 cycles) and (6 - 8 cycles) respectively of alternating ABVD/COEP chemotherapy followed by involved-field radiation therapy (IFRT): 15 Gy for patients achieved complete response, and 25.5 Gy for those achieved a partial response. Results: 27 patients were IR and 27 patients were HR. There were 16 treatment failures; 5 patients had progressive disease; and 11 patients had a relapse. 9 patients died from their disease progression. The 5-year overall survival (OS) and event-free survival (EFS) rates (±SE) were 81.8% ± 5.7% and 71.8% ± 6.2% respectively. Multivariate analysis revealed that the only independent factor for inferior OS was radiotherapy. Conclusion: Treatment results of unfavourable HL patients in our study are satisfactory for with IR group but not for HR group who needs intensification of therapy. Radiotherapy is considered as a cornerstone in the treatment of the patients with unfavourable criteria with better assessment of early responders needed by PET-CT to identify patients at risk for relapse.

Background T-cell acute lymphoblastic leukemia (T-ALL) accounts for about 15% of pediatric ALL. With wider use of intensive chemotherapy, the prognosis for childhood T-ALL has improved. Further gains in treatment outcome will likely require methods to identify patients who continue to fail on contemporary protocols. This study aimed to evaluate pediatric patients with T-ALL at 2 different Arabic cancer centers regarding their clinicopathologic, immunophenotypic, and cytogenetic features and outcome. Patients and Methods This retrospective study included all children with T-ALL treated between 2003 and 2013 at 2 oncology centers in the Middle East. Patients were divided into (group I) treated with Berlin-Frankfurt-Munster (BFM)-90 treatment protocol between February 2003 and June 2007 and (group II) includes all patients treated thereafter by the Total Therapy Study XIII protocol for high-risk ALL. Results This study included 103 patients with a median age of 8.9 years. The male to female ratio was 2.6:1. The median initial white blood cell count was 123 × 10 9 /L. Central nervous system leukemia was detected in 15%. The early T-cell precursor (ETP)-ALL phenotype was found in 16.5%. The 5-year overall survival was 20.7% ± 67.5% and 72.9% ± 5.7% ( P P = .023); and the 5-year event-free survival was 28.6% ± 12.1% and 71.1% ± 6.2% ( P = .003) for group I and II, respectively. Conclusion The outcome of patients with T-ALL significantly improved in patients who received the treatment protocol of ALL with high-risk criteria. This protocol eliminates the bad outcomes effect of several clinical and immunophenotypic markers. Patient with the ETP-ALL phenotype had a nonsignificant inferior outcome compared with the non–ETP-ALL group.

Importance In low resource countries, there has been scarcity of research on the risk factors associated with neutropenic enterocolitis, a serious complication that commonly develops during treatment of cancer patients. Objective To identify the pattern of intestinal complications in pediatric leukemia patients treated with intensive chemotherapy, including those with neutropenic enterocolitis; to assess the outcome; and to evaluate the risk factors associated with the mortality in these patients. Methods During the period from June 2015 to December 2016, a prospective study was carried out on pediatric patients diagnosed with acute leukemia who received induction/or re‐induction phases of chemotherapy at South Egypt Cancer Institute. Patients with documented episodes of intestinal complications were included in the study. Recovery or death from an episode of intestinal complication was utilized as the primary outcome measure for the study. Using univariable and multivariable methods, potential risk factors associated with mortality were delineated by logistic regression analysis, both for the entire intestinal complications episodes as a whole and for those episodes of neutropenic enterocolitis only. Results Out of 88 documented episodes of intestinal complications from 77 patients; 58 episodes were identified as neutropenic enterocolitis from 47 patients. In those patients who were having episodes of neutropenic enterocolitis, the presence of abdominal tenderness (OR 4.529, 95%CI 1.062–19.317, P = 0.041); a longer duration of neutropenia (OR 1.215, 95%CI 1.030–1.434, P = 0.021); and hemodynamic instability (OR 17.023, 95%CI 4.095–70.772, P < 0.001), were found to be independently associated with worse outcome. Interpretation In Upper Egypt, the use of intensive systemic chemotherapy during the induction phase of acute leukemia was found to be associated with potentially lethal intestinal complications. A high index of clinical suspicion is warranted.

Children with leukemia are subjected to pain during various procedures. Distraction is one of the easiest and least costly methods of non-pharmacological pain relief. This study aimed to identify the efficacy of distraction on pain relief during lumber puncture in children with leukemia. Methods: A quasi experimental research design conducted among 90 patients at South Egypt Cancer Institute - Assiut University. children were allocated to either breathing, colored cards, or control group, thirty child for each group .Two tools were used, Tool one: Structured interview sheet to collect socio - demographic data and tool two where children's pain was assessed by FLACC scale. Findings: Pain score in the breathing and colored cards groups was highly significantly lesser than the control group. There was no significant difference between the two sexes while as the age increases, the tolerance to pain increases too. Conclusion: This study showed that breathing exercises and colored cards can significantly reduce the pain of intrathecal injection. Recommendations: Considering the key role of nurses in a health care team, the researchers hope that the results of this study can help them learn this method and implement it in hospitalized children who undergo painful procedures.

Objective.Several studies showed better outcome in adolescents and young adults with acute lymphoblastic leukemia (ALL) treated with pediatrics protocols than similarly aged patients treated with adults protocols, while other studies showed similar outcome of both protocols. We conducted this study to compare the outcome of our pediatrics and adults therapeutic protocols in treatment of adolescents ALL.Patients and Methods.We retrospectively reviewed files of 86 consecutive adolescent ALL patients aged 15–18 years who attended to outpatients clinic from January 2003 to January 2010. 32 out of 86 were treated with pediatrics adopted BFM 90 high risk protocol while 54 were treated with adults adopted BFM protocol. We analyzed the effect of different treatment protocols on achieving complete remission (CR), disease-free survival (DFS), and overall survival (OS).Results.The 2 patients groups have almost similar characteristics. The CR was significantly higher in pediatrics protocol 96% versus 89%(P=0.001). Despite the fact that the toxicity profiles were higher in pediatrics protocol, they were tolerable. Moreover, the pediatrics protocol resulted in superior outcome in EFS 67% versus 39% (P=0.001), DFS 65% versus 41%(P=0.000), and OS 67% versus 45%(P=0.000).Conclusion.Our study’s findings recommend using intensified pediatrics inspired protocol to treat adolescents with acute lymphoblastic leukemia.

Risk stratification allows tailoring of treatment protocol using, for selected patients, reduced total chemotherapy exposure, including decreases in alkylator therapy and avoidance of agents with recognized risk of late complications (anthracyclines), elimination of irradiation and reduction of radiotherapy dose. Patients and Methods: Twenty-nine newly diagnosed pediatric rhabdomyosarcoma patients attended the pediatric oncology department between January 2008 and May 2011. Patients were divided into 3 groups according to age, stage, group, pathology and site of the tumor. Treatment protocol tailored according to risk group. Results: Twenty-nine newly diagnosed pediatric rhabdomyosarcoma patients were evaluated. Seven patients had low risk, Intermediate risk included 12 patients, and 10 patients had high risk. After three years median follow up, event free survival was 51.7% for all patients however it was 86%, 67% and 10% for low, intermediate and high risk respectively (P = 0.0002). There was statistical difference for survival among different sites, histology, clinical group and stage as risk factors within each risk group, no statistically survival significance of any of these factors within the same risk group. Conclusion: Risk stratification is the best single predictor factor for pediatric rhabdomyosarcoma and allows tailoring of the treatment protocol. For selected patients, reductions in total chemotherapy exposure, elimination of irradiation in selected low risk patients and reduction of radiotherapy dose according to postoperative margin and nodal status is safe.

ntroduction: Lymphadenopathy is a common complaint in children. It's defined as abnormality in size or character of lymph node. It may be localized or generalized. It may be part of a group of signs and symptoms or the sole finding and chief complaint. Several reasons can lead to Lymphadenopathy. Castleman's disease is a rare differential diagnosis of generalized or localized Lymphadenopathy. It's a rare lymph proliferative disorder that rarely occurs in children. Case presentation: We reported two cases, the first one was 13 years old female with multicenteric castleman's disease associated with constitutional manifestations and unusual hyaline vascular pathology successfully treated with monthly combination chemotherapy, the second one was 2.5 years old male with unicentric hyaline vascular castleman's disease with unusual young age at presentation successfully treated with surgical excision. Conclusion: Castleman's disease is a rare cause of Lymphadenopathy. Although, it's rare in children, it can occur at any age. Most of cases of multicenteric castleman's disease are plasma cell variant, yet hyaline vascular type may occur. Multicenteric castleman's disease can be successfully treated with monthly combination chemotherapy. Most cases of unicentric castleman's disease are hyaline vascular type. Although, surgical removal of unicentric type is usually sufficient to achieve complete remission, recurrence can still occur.

AIM To investigate the prevalence and virological characteristics of occult hepatitis B virus (HBV) infections in patients with hematological malignancies in South Egypt. METHODS Serum samples were collected from 165 patients with hematological malignancies to monitor titers of HBV DNA, hepatitis B surface antigen (HBsAg), and antibodies to HBV core (anti-HBc) and surface antigens. Serum samples negative for HBsAg and positive for anti-HBc were subjected to nucleic acid extraction and HBV DNA detection by real-time polymerase chain reaction. DNA sequences spanning the S region were analyzed in cases with occult HBV infection. In vitro comparative study of constructed 1.24-fold wild type and S protein mutant HBV genotype D clones was further performed. RESULTS HBV DNA was detected in 23 (42.6%) of 54 patients with hematological malignancies who were HBsAg negative, but anti-HBc positive, suggesting the presence of occult HBV infection. The complete HBV genome was retrieved from 6 occult HBV patients, and P120T and S143L were detected in 3 and 2 cases, respectively. Site directed mutagenesis was done to produce 1.24-fold genotype D clones with amino acid mutations T120 and L143. The in vitro analyses revealed that a lower level of extracellular HBsAg was detected by chemiluminescence enzyme immunoassay (CLEIA) with the clone containing T120 mutation, compared with the wild type or the clone with S143L mutation despite the similar levels of extracellular and intracellular HBsAg detected by Western blot. Southern blot experiments showed that the levels of intracellular HBV DNA were not different between these clones. CONCLUSION Occult HBV infection is common in patients with hematological malignancies and associated with P120T and S143L mutations. 120T mutation impairs the detection of HBsAg by CLEIA.

AIM: To investigate characteristics of hepatitis B virus (HBV) implicated in HBV reactivation in patients with hematological malignancies receiving immunosuppressive therapy. METHODS: Serum samples were collected from 53 patients with hematological malignancies negative for hepatitis B surface antigen (HBsAg) before the start of and throughout the chemotherapy course. HBV reactivation was diagnosed when the HBsAg status changed from negative to positive after the initiation of chemotherapy and/or when HBV DNA was detected by real-time detection polymerase chain reaction (RTD-PCR). For detecting the serological markers of HBV infection, HBsAg as well as antibodies to the core antigen (anti-HBc) and to the surface antigen were measured in the sera by CEIA. Nucleic acids were extracted from sera, and HBV DNA sequences spanning the S gene were amplified by RTD-PCR. The extracted DNA was further subjected to PCR to amplify the complete genome as well as the specific genomic sequences bearing the enhancer II/core promoter/pre-core/core regions (nt 1628-2364). Amplicons were sequenced directly. RESULTS: Thirty-five (66%) of the 53 HBsAg-negative patients were found to be negative serologically for anti-HBc, and the remaining 18 (34%) patients were positive for anti-HBc. Five of the 53 (9.4%) patients with hematologic malignancies experienced HBV reactivation. Genotype D1 was detected in all five patients. Four types of mutant strains were detected in the S gene product of HBV strains and were isolated from 3 patients with HBV reactivation: T/S120, L143, and I126. HBV DNA was detected in the pretreatment HBsAg-negative samples in one of the five patients with HBV reactivation. In this patient, sequences encompassing the HBV full genome obtained from sera before the start of chemotherapy and at the time of de novo HBV hepatitis were detected and it showed 100% homology. Furthermore, in the phylogenetic tree, the sequences were clustered together, thereby indicating that this patient developed reactivation from an occult HBV infection. CONCLUSION: Past infection with HBV is a risk factor for HBV reactivation in Egypt. Mandatory anti-HBc screening prior to chemotherapy in patients with hematological malignancies is recommended.

Abdominal Non-Hodgkin lymphomas (NHL) are the most common extra nodal presentation of pediatric NHL. Our aim is to assess the role of surgery as a risk factor and to evaluate the impact of risk-adjusted systemic chemotherapy on survival of patients with stages II and III disease. This study included 35 pediatric patients with abdominal NHL treated over five years at South Egypt Cancer Institute (SECI), Assiut University, between January 2005 and January 2010. The data of every patient included: Age, sex, and presentation, staging work up to determine extent of the disease and the type of resection performed, histopathological examination, details of chemotherapy, disease free survival and overall survival. The study included 25 boys and 10 girls with a median age of six years (range: 2.5:15). Thirty patients (86%) presented with abdominal pain, 23 patients (66%) presented with abdominal mass and distention, 13 patients (34%) presented with weight loss, and intestinal obstruction occurred in six patients (17%). The ileo-cecal region and abdominal lymph nodes were the commonest sites (48.5%, 21% respectively). Burkitt's lymphoma was the most common histological type in 29 patients (83%). Ten (28.5%) stage II (group A) and 25 (71.5%) stage III (group B). Complete resections were performed in 10 (28.5%), debulking in 6 (17%) and imaging guided biopsy in 19 (54%). A11 patients received systemic chemotherapy. The median follow up duration was 63 months (range 51-78 months). The parameters that significantly affect the overall survival were stage at presentation complete resection for localized disease. In conclusion, the extent of disease at presentation is the most important prognostic factor in pediatric abdominal NHL. Surgery is restricted to defined situations such as; abdominal emergencies, diagnostic biopsy and total tumor extirpation in localized disease. Chemotherapy is the cornerstone in the management of pediatric abdominal NHL.

Background We conducted a retrospective analysis to investigate treatment results and prognostic factors of pediatric neuroblastoma patients. Methods This retrospective study was carried out analyzing the medical records of patients with the pathological diagnosis of neuroblastoma seen at South Egypt Cancer Institute, Assiut University during the period from January 2001 and January 2010. After induction chemotherapy, response according to international neuoblastoma response criteria was assessed. Radiotherapy to patients with residual primary tumor was applied. Overall and event free survival (OAS and EFS) rates were estimated using Graphed prism program. The Log-rank test was used to examine differences in OAS and EFS rates. Cox-regression multivariate analysis was done to determine the independent prognostic factors affecting survival rates. Results Fifty three cases were analyzed. The median follow-up duration was 32 months and ranged from 2 to 84 months. The 3-year OAS and EFS rates were 39.4% and 29.3% respectively. Poor prognostic factors included age >1 year of age, N-MYC amplification, and high risk group. The majority of patients (68%) presented in high risk group, where treatment outcome was poor, as only 21% of patients survived for 3 year. Conclusion Multivariate analysis confirmed only the association between survival and risk group. However, in univariate analysis, local radiation therapy resulted in significant survival improvement. Therefore, radiotherapy should be given to patients with residual tumor evident after induction chemotherapy and surgery. Future attempts to improve OAS in high risk group patients with aggressive chemotherapy and bone marrow transplantation should be considered.

Objectives To report the long-term follow-up of patients with infantile Wilms tumor treated according to the International Society of Pediatric Oncology study 9 protocol. Methods We retrospectively reviewed our medical reports for diagnosed WT in patients aged 6-12 months from January 2001 to January 2009. The clinical presentation, stage, operative details, pathologic findings, and outcomes for these infants were analyzed. We reviewed the charts of these patients throughout the whole disease course through long-term follow-up, paying particular attention to the details of the clinical presentation, stage at presentation versus postoperative stage, intraoperative findings, pathologic findings, and outcomes. Results The records revealed 16 patients with a median age of 7.5 months. All patients had presented with an abdominal mass, and 25% had presented with abdominal pain and hematuria. Associated congenital anomalies were observed in 16.7% of the patients. A favorable histologic type was found in 91.7% of the patients and 8.3% had an unfavorable histologic type. The median follow-up period was 57 months. Postoperative complete remission was achieved in all patients who underwent surgery. Relapse developed in 1 patient. The 7-year disease-free survival rate was 93.8%, and the 7-year overall survival rate was 75%. Conclusions The long-term follow-up data using the International Society of Pediatric Oncology study 9 protocol revealed good outcomes. The protocol was a safe and an effective line of therapy, associated with decreased morbidity and improved survival. Also, the reduction in tumor volume resulted in easier surgical procedures, with no intraoperative complications.

We conducted a retrospective analysis to investigate the clinical outcome of combined-modality therapy using multiagent chemotherapy and involved-field radiotherapy in treatment of children with Hodgkin's lymphoma. Fifty eight cases with newly diagnosed Hodgkin's lymphoma were analyzed. The median follow-up duration was 46 months (range 3-72 months). The 4-year overall and event-free survival rates were 91.5% and 69.7% respectively. High-risk disease (stage IIIB and IV), presence of B symptoms, lymphocyte depletion subtype, bulky disease and late response to chemotherapy were poor prognostic factors. Stage-adapted combined-modality therapy resulted in satisfactory outcome in treatment of pediatric Hodgkin's lymphoma.

The study aimed to assess the diagnostic accuracy of Flow cytometry (FCM) immunophenotyping and IgH gene rearrangements (IGHRs) by real-time PCR in comparison with classic cytology for diagnosing CNS infiltration in pediatric ALL. We concluded that the diagnostic value of FCM and IGHR are two to three times more than that of cytology. Therefore, immunophenotyping by FCM is recommended for routine diagnosis of CSF infiltration. Furthermore, IGHR analysis by real-time PCR appears to be a useful addition in evaluation of CNS infiltration.