The fracture resistance of pulpotomized primary molars restored with zirconia crowns, lithium disilicate or resin based ceramic endocrowns.

BACKGROUND: Endocrown in pediatric dentistry was rooted in the fundamental principles of preserving healthy dental tissues, leveraging contemporary adhesive methodologies. AIM: This research aimed on assessing and comparing the fracture resistance of pulpotomized primary molars when rehabilitated with zirconia crowns and two distinct types of endocrowns, namely E-Max and Brilliant Crios. METHODS: The study involved thirty, anonymized, freshly extracted second primary molars that underwent pulpotomy. These teeth were then evenly divided into three groups, each consisting of ten specimens: the zirconia crown, the E-Max endocrown, and the Brilliant Crios endocrown groups. Post-pulpotomy, the teeth were prepared for their respective restorations. Subsequent to this preparation, the zirconia crowns, E-Max endocrowns, and Brilliant Crios endocrowns were secured. To evaluate the fracture resistance using a computer-controlled testing machine (Instron), a progressively increasing load was applied to each group until fracture occurred. The gathered data were then analyzed for outliers and subjected to normality testing using the Shapiro-Wilk and/or Kolmogorov-Smirnov tests, with a significance threshold set at 0.05. RESULTS: There was no statistically significant difference in fracture resistance of pulpotomized primary molars among lithium disilicate (E-Max) group (mean=1367.59N), Brilliant Crios group (mean=1349.73N) and zirconia group (mean=1240.82N). CONCLUSION: Endocrowns can be considered a promising restoration for pulpotomized primary molars.

Surgical resection and outcome of malignant ovarian germ cell tumors in children-a national multicentric study compared to international results.

PURPOSE: To evaluate the outcome of pediatric malignant ovarian germ cell tumors treated by three tertiary Egyptian institutions, and to compare our national experience to internationally published data. METHODS: This is a retrospective analysis of all patients presenting between September 2014 and September 2019. Management protocol was Children's Oncology Group (COG) in all participating centers. Overall survival (OS), event-free survival (EFS) and univariate prognostic factors were estimated by Kaplan-Meier and log-rank test. Additionally, a review of various practices that reported survival outcome was conducted. MAIN RESULTS: Thirty-seven patients were included with a median age of 10.5 years (1-18 years). Thirty-five patients had unilateral salpingo-oophorectomy. Mixed germ cell and yolk sac tumors represented 75.7% of patients. There were 7 (19%), 14 (37.8%), 12 (32.4%) and 4 (10.8%) stage I, II, III and IV, respectively. Seven patients were low risk (LR), 26 intermediate risk (IR) and 4 high risk (HR). Platinum-based chemotherapy was administered as per risk stratification. Follow-up to March 2020 revealed that five patients had relapsed. There were no statistical significances of pathological types and patients' age regarding OS (p value 0.392 and 0.281, respectively) and EFS (p value 0.420 and 0.437, respectively). Three-year OS was 84%: 100% for stages I and II, and 62% for stages III and IV (p = 0.003); 100% for LR, 89% for IR, and 24% for HR (p < 0.001). Three-year EFS was 87%: 96% for stages I and II, and 71% for stages III and IV (p = 0.025); 100% for LR, 92% for IR, and 26% for HR (p < 0.001). CONCLUSION: Surgical resection combined with chemotherapy achieves excellent outcome for such tumors in both, present study and previous reports. On the basis of our results, COG staging and risk stratification were significantly correlated with prognosis, whereas tumor pathology and age had no significant impact. Prognostic factors are controversial among studies, and further research is still required.

Can Pediatric Risk of Mortality Score (PRISM III) Be Used Effectively in Initial Evaluation and Follow-up of Critically Ill Cancer Patients Admitted to Pediatric Oncology Intensive Care Unit (POICU)? A Prospective Study, in a Tertiary Cancer Center in Egypt.

Pediatric Risk of Mortality Score (PRISM III-12) is a physiology-based predictor for risk of mortality. We conducted prospective study from January 1, 2014 to 2015 in pediatric oncology intensive care unit (POICU) at South Egypt Cancer Institute, Egypt to explore the ability of 1st PRISM III-12 to predict the risk of mortality in critically ill cancer patients and the ability of serial PRISM III measured every 72 hours to follow-up the patients' clinical condition during POICU stay. In total, 123 (78 males) children were included. Median age was 5 years (1 to 15 y). Death rate was 20%. 1st PRISM III-12 mean was 19 (0 to 61). The mean 1st PRISM III-12 for survivors was significantly higher compared with nonsurvivors (15 vs. 37 respectively; P<0.001). 1st PRISM III-12 mean was significantly correlated to the reasons for admission and organ failures' number (P<0.001 and <0.001). 1st PRISM III-12 correlated weakly positive with the length of stay (r=0.2; P=0.024). Receiver operator curve for 1st PRISM III-12 was 0.913 (95% confidence interval, 0.85-0.98; P<0.001). Decline in serial PRISM III was significantly correlated with favorable (survivor) outcome (P<0.001). We concluded that PRISM III-12 can be used effectively in predicting the risk of mortality and following the clinical condition of patients during POICU stay.

The Outcome of Critically Ill Pediatric Cancer Patients Admitted to the Pediatric Intensive Care Unit in a Tertiary University Oncology Center in a Developing Country: A 5-Year Experience.

INTRODUCTION: Cancer remains a major cause of death in children, but recent advances in supportive care and progress in the use of chemotherapy have considerably improved the prognosis. The need for intensive care management in pediatric oncology patients is increasing. However, studies demonstrating their outcome in the literature are still deficient, especially in developing countries. Here, we aim to report our experience in managing patients admitted to the pediatric intensive care unit (PICU) at South Egypt Cancer Institute, a tertiary university oncology center in a developing country. PATIENTS AND METHODS: A review of all cancer patients admitted to the PICU at South Egypt Cancer Institute between January 2007 and December 2011 and an evaluation of prognostic factors that may correlate to their short-term outcome were performed. RESULTS: A total of 550 pediatric oncology patients were admitted to the PICU on 757 occasions. Hematological malignancies represented 73.6% of the cases. The median duration of PICU stay was 5 days. Sepsis and respiratory failure were the most frequent indications for PICU admission. The overall survival at the time of discharge from the PICU was 60%. Several factors were found to significantly affect the outcome of patients admitted to the PICU, including the underlying disease, the reason for admission, the intervention used, and the number of failing organs at the time of admission to the PICU. CONCLUSIONS: The prognosis of patients admitted to the PICU in developing countries is still behind those in developed ones. Late referral, especially of patients presenting with respiratory failure, sepsis, and multiorgan failure usually, requires urgent intervention with inotropic support, oxygen therapy, and mechanical ventilation and is significantly associated with poor outcomes, especially in patients with hematological malignancies.

Massive retroperitoneal cyst impersonating ovarian tumor: A case report.

INTRODUCTION: The retroperitoneum is the anatomical compartment positioned behind the peritoneal cavity. It is separated into three primary spaces: the anterior pararenal, perirenal, and posterior pararenal spaces. Retroperitoneal cystic mass is a rare surgical problem that is often wrongly identified before surgery. CASE PRESENTATION: We report a case of a 27-year-old female presenting with abdominal swelling and pain starting from 9 months. An abdominal computed tomography scan showed a right adnexal mass with a high probability of a serous ovarian. The patient was diagnosed with ovarian tumors before surgery, but it was identified with a retroperitoneal cyst during surgery. DISCUSSION: A retroperitoneal cyst's clinical signs and symptoms vary, and the diagnosis can often be challenging. Computed tomography scans are appropriate for assessing retroperitoneal pathology because they produce separate sectional images and couldn't find the correct diagnosis in previous cases. CONCLUSION: This paper shows the rare case of primary retroperitoneal lesions, which can be hard to diagnose before surgery, even though medical imaging has come a long way.

Travel burden and clinical presentation of retinoblastoma: analysis of 1024 patients from 43 African countries and 518 patients from 40 European countries.

BACKGROUND: The 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. METHODS: A 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. RESULTS: Capture 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, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease. CONCLUSIONS: Fewer 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.

Neutropenic enterocolitis in pediatric leukemia patients treated with intensive chemotherapy in Upper Egypt.

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.

Global Retinoblastoma Presentation and Analysis by National Income Level.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

Clinicopathological pattern and outcome of pediatric malignant ovarian germ cell tumors: South Egypt Cancer Institute experience.

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).

Outcome and Clinical Significance of Immunophenotypic Markers Expressed in Different Treatment Protocols of Pediatric Patients With T-ALL in Developing Countries.

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-Münster (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 × 109/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 < .01); the 5-year disease-free survival was 47.1% ± 13.8% and 77.3% ± 6.0% (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.

Characteristics of escape mutations from occult hepatitis B virus infected patients with hematological malignancies in South Egypt.

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.

Genetic variants in 6-mercaptopurine pathway as potential factors of hematological toxicity in acute lymphoblastic leukemia patients.

AIM: We investigated the associations between variants in genes coding for enzymes and transporters related to the 6-mercaptopurine pathway and clinical outcomes in pediatric patients with acute lymphoblastic leukemia. MATERIALS & METHODS: Statistical association between gender, age and genotypes of selected SNPs, and the risks of hematological toxicity and relapse were investigated using a Cox proportional hazard model in 70 acute lymphoblastic leukemia patients from upper Egypt. RESULTS: We found significant associations between ITPA, IMPDH1, SLC29A1, SLC28A2, SLC28A3 and ABCC4 SNPs and one or more of the hematological toxicity manifestations (neutropenia, agranulocytosis and leukopenia); age was significantly related to relapse. CONCLUSION: Genetic polymorphisms in enzymes and transporters involved in the 6-mercaptopurine pathway should be considered during its use to avoid hematological toxicity.

Outcome of Adolescents with Acute Lymphoblastic Leukemia Treated by Pediatrics versus Adults Protocols.

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.

Incidence and characteristics of HBV reactivation in hematological malignant patients in south Egypt.

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.

Sacrococcygeal tumors: clinical characteristics and outcome of pediatric patients treated at South Egypt Cancer Institute. A retrospective analysis.

BACKGROUND: Sacrococcygeal tumors (SCT) are relatively uncommon tumors affecting neonates, infants and children. The aim of this article is to clarify any special characterizations in natural history, clinical presentation and outcome of such tumors treated at South Egypt Cancer Institute, the only research center located in South Egypt. METHODS: A retrospective analysis of children with SCT treated at the Pediatric Oncology department South Egypt Cancer Institute, Assiut University between 2004 and 2010. RESULTS: Nineteen children were included in the study. Age ranged between 10 days and 5 years. All but three had sacral mass at presentation. AFP levels ranged between normal age-related levels and 217,200 ng/ml. Initial resection was possible in 11, while eight patients with clinical suggestion of advanced malignant disease were inoperable. They received initial chemotherapy followed by delayed surgery. Yolk sac tumor (YST) was reported in 52.9% of patients. Recurrence was reported in 5 patients (3 mature teratomas and 2 YST). Five-year OS and RFS rates of patients who had malignant disease were 81.8% and 77.8% respectively. CONCLUSIONS: Older age and delay in presentation that resulted in predominance of extensive disease and malignant transformation at presentation were the main challenges we faced in managing patients with SCT in our locality.

Long-term follow-up of infantile Wilms tumor treated according to International Society of Pediatric Oncology protocol: seven years' follow-up.

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.

Role of Surgery in Stages II and III Pediatric Abdominal Non-Hodgkin Lymphoma: A 5-Years Experience.

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.

Risk-based combined-modality therapy of pediatric Hodgkin's lymphoma: a retrospective study.

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.

Treatment results and prognostic factors of pediatric neuroblastoma: a retrospective study.

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.

Immunophenotyping and immunoglobulin heavy chain gene rearrangement analysis in cerebrospinal fluid of pediatric patients with acute lymphoblastic leukemia.

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.