Background: Non-Hodgkin’s lymphoma is an aggressive malignant disease in children and adolescents. Although it is the fourth most common malignancy in Saudi children as reported in Saudi cancer registry, less informa...Background: Non-Hodgkin’s lymphoma is an aggressive malignant disease in children and adolescents. Although it is the fourth most common malignancy in Saudi children as reported in Saudi cancer registry, less information is available about pediatric Non-Hodgkin lymphoma and its outcome in Saudi Arabia. Study Objectives: To provide demographic data, disease characteristics, treatment protocol, toxicity and outcome of treatment in children & adolescents with Non-Hodgkin’s lymphoma treated at KFMC. This study will form base line for future studies about pediatric Non-Hodgkin’s lymphoma in KFMC, which may help to improve outcome for children with cancer in Saudi Arabia. Study Patients and Method: We retrospectively analyzed 28 children and adolescents diagnosed to have Non-Hodgkin’s lymphoma at KFMC between December 2006 and December 2013, followed-up through June 2014. Results: Of the 28 patients, 10 (35.7%) girls and 18 (64.3%) boys, the male-to-female ratio was 1.8;1. The median age at time of diagnosis was 6.4 years old (range 2.0 to 13.0 years old). The majority of patients (64.3%) were aged between 5 and 12 years old. Burkitt’s lymphoma BL/BLL was the most common pathological subtype (60.7%), and DLBCL was the second most common subtype (21.4%). Abdominal and Retroperitoneal involvement was the most common primary site (78.6%) including the ileocaecal region. Most of the children presented with advanced Stage III and IV (75%), Cytogenetic study which screens specifically for the t (8;14) (q24;q32) a characteristic genetic feature of Burkitt’s Lymphoma was obtained from 21 patients, variant rearrangement was observed in 3/21 samples and complex chromosomes karyotype in addition to IGH/MYC rearrangement was observed in 2/21 samples. Those patients presented with very aggressive lymphoma and combined BM and CNS involvement. We use the French-American-British Mature B-Cell Lymphoma 96 Protocol (FAB LMB 96) for treatment fornewly diagnosed Mature B-Cell type NHL and high risk ALL CCG 1961 Protocol for lymphoblastic lymphoma and international Anaplastic Large Cell Lymphoma 99 Study Protocol for ALCL. The median follow-up in patients not experiencing an adverse event was 53.1 months. The estimated 3-year EFE and OS rates in the entire cohort of patients with newly diagnosed NHL treated in the KFMC were 85.2% and 89.2% respectively;Overall survival (OS) rate of patients with mature B-cell-NHL was 88.9%. Conclusion: The outcomes and survival in our small series appeared to be excellent compared with those reported in other international trials even though most of our patients presented in advanced stage of the disease. We feel that the importance of the current study is to document the relative distribution of various types of pediatric non-Hodgkin’s lymphomas and age-specific distribution in different Histological subtypes.展开更多
Introduction: Neuroblastoma is the most common extracranial solid tumor in childhood and survival rate has improved during the last few decades. Only a few studies, related to Neuroblastoma in Saudi Arabian children, ...Introduction: Neuroblastoma is the most common extracranial solid tumor in childhood and survival rate has improved during the last few decades. Only a few studies, related to Neuroblastoma in Saudi Arabian children, have been performed. We report epidemiologic data and our clinical experience from the department of Pediatric Hematology Oncology (PHO), King Fahad Medical City (KFMC), Riyadh, Saudi Arabia. Method: A retrospective observational study of all patients, with diagnosis of Neuroblastoma, who attended PHO-KFMC from July 2006 to June 2014 was performed. The survival periods (overall survival and disease-free survival) and the final outcomes for patients treated and followed at KFMC were recorded. The survival data were statistically correlated with the clinical, pathological and biological features of patients and tumors and compared to national and international cohorts. Results: Eight-year data were available for the 42 patients of which 22 (52.4%) were male and 20 (47.6%) were females. Age at diagnosis ranged 0 - 91 months with a mean and median of 26.3 and 18.5 months respectively. 16 (38.1%) patients were under one year and 26 (61.9%) above 1 year of age. The event-free survival (EFS) and overall survival (OS) rates were 66.5% and 71.5% respectively. EFS and OS among those who were <1 year age at presentation was 75% and 82%, whereas ≥1 yr age group had 59% and 62% survival rates respectively. Patients with tumors in the adrenal had considerably lower EFS (59%) and OS (63%);in comparison to patients with tumors sites other than the adrenal who had EFS and OS of 85% and 89% respectively. Both EFS and OS survival rates at the end of follow-up interval were 100.0%, in the low and intermediate risk groups. In contrast, patients in the high risk group had EFS and OS rates of 44% and 48% respectively. This difference was statistically significant (p < 0.05). Conclusion: Our results are very encouraging and comparable with known published international cohorts, and reveal an excellent outcome for stage 1, 2, 3 & 4 s. The prognosis for advanced (stage 4) disease remains rather poor. A collaborative Saudi-wide effort, with an emphasis on research in detecting clinical and biologic characteristics of aggressive disease and tailoring therapy, is needed.展开更多
文摘Background: Non-Hodgkin’s lymphoma is an aggressive malignant disease in children and adolescents. Although it is the fourth most common malignancy in Saudi children as reported in Saudi cancer registry, less information is available about pediatric Non-Hodgkin lymphoma and its outcome in Saudi Arabia. Study Objectives: To provide demographic data, disease characteristics, treatment protocol, toxicity and outcome of treatment in children & adolescents with Non-Hodgkin’s lymphoma treated at KFMC. This study will form base line for future studies about pediatric Non-Hodgkin’s lymphoma in KFMC, which may help to improve outcome for children with cancer in Saudi Arabia. Study Patients and Method: We retrospectively analyzed 28 children and adolescents diagnosed to have Non-Hodgkin’s lymphoma at KFMC between December 2006 and December 2013, followed-up through June 2014. Results: Of the 28 patients, 10 (35.7%) girls and 18 (64.3%) boys, the male-to-female ratio was 1.8;1. The median age at time of diagnosis was 6.4 years old (range 2.0 to 13.0 years old). The majority of patients (64.3%) were aged between 5 and 12 years old. Burkitt’s lymphoma BL/BLL was the most common pathological subtype (60.7%), and DLBCL was the second most common subtype (21.4%). Abdominal and Retroperitoneal involvement was the most common primary site (78.6%) including the ileocaecal region. Most of the children presented with advanced Stage III and IV (75%), Cytogenetic study which screens specifically for the t (8;14) (q24;q32) a characteristic genetic feature of Burkitt’s Lymphoma was obtained from 21 patients, variant rearrangement was observed in 3/21 samples and complex chromosomes karyotype in addition to IGH/MYC rearrangement was observed in 2/21 samples. Those patients presented with very aggressive lymphoma and combined BM and CNS involvement. We use the French-American-British Mature B-Cell Lymphoma 96 Protocol (FAB LMB 96) for treatment fornewly diagnosed Mature B-Cell type NHL and high risk ALL CCG 1961 Protocol for lymphoblastic lymphoma and international Anaplastic Large Cell Lymphoma 99 Study Protocol for ALCL. The median follow-up in patients not experiencing an adverse event was 53.1 months. The estimated 3-year EFE and OS rates in the entire cohort of patients with newly diagnosed NHL treated in the KFMC were 85.2% and 89.2% respectively;Overall survival (OS) rate of patients with mature B-cell-NHL was 88.9%. Conclusion: The outcomes and survival in our small series appeared to be excellent compared with those reported in other international trials even though most of our patients presented in advanced stage of the disease. We feel that the importance of the current study is to document the relative distribution of various types of pediatric non-Hodgkin’s lymphomas and age-specific distribution in different Histological subtypes.
文摘Introduction: Neuroblastoma is the most common extracranial solid tumor in childhood and survival rate has improved during the last few decades. Only a few studies, related to Neuroblastoma in Saudi Arabian children, have been performed. We report epidemiologic data and our clinical experience from the department of Pediatric Hematology Oncology (PHO), King Fahad Medical City (KFMC), Riyadh, Saudi Arabia. Method: A retrospective observational study of all patients, with diagnosis of Neuroblastoma, who attended PHO-KFMC from July 2006 to June 2014 was performed. The survival periods (overall survival and disease-free survival) and the final outcomes for patients treated and followed at KFMC were recorded. The survival data were statistically correlated with the clinical, pathological and biological features of patients and tumors and compared to national and international cohorts. Results: Eight-year data were available for the 42 patients of which 22 (52.4%) were male and 20 (47.6%) were females. Age at diagnosis ranged 0 - 91 months with a mean and median of 26.3 and 18.5 months respectively. 16 (38.1%) patients were under one year and 26 (61.9%) above 1 year of age. The event-free survival (EFS) and overall survival (OS) rates were 66.5% and 71.5% respectively. EFS and OS among those who were <1 year age at presentation was 75% and 82%, whereas ≥1 yr age group had 59% and 62% survival rates respectively. Patients with tumors in the adrenal had considerably lower EFS (59%) and OS (63%);in comparison to patients with tumors sites other than the adrenal who had EFS and OS of 85% and 89% respectively. Both EFS and OS survival rates at the end of follow-up interval were 100.0%, in the low and intermediate risk groups. In contrast, patients in the high risk group had EFS and OS rates of 44% and 48% respectively. This difference was statistically significant (p < 0.05). Conclusion: Our results are very encouraging and comparable with known published international cohorts, and reveal an excellent outcome for stage 1, 2, 3 & 4 s. The prognosis for advanced (stage 4) disease remains rather poor. A collaborative Saudi-wide effort, with an emphasis on research in detecting clinical and biologic characteristics of aggressive disease and tailoring therapy, is needed.
