Iranian Journal of Blood and Cancer
Volume:2 Issue: 2, Winter 2010

Neuroblastoma, one of the common malignant childhood tumors, arises from neuroblast cells derived from the neural crest and destined for the adrenal medulla and the sympathetic nervous system and shows remarkable biological heterogeneity, resulting in favorable or unfavorable outcomes. Some tumors make rapid progress with a fataloutcome. In other instances, the tumors regress spontaneously in infants or to differentiate into a benign ganglioneuroma in older patients. This heterogeneity within neuroblastoma depends on the molecular characteristics of tumor cells. Several distinct genomic alterations have been found in neuroblastoma, including MYCN amplification, DNAploidy, deletion of the short arm of chromosome 1, gain of chromosome 17q, and deletion of 11q. The difference of expression was also found in genes related to cellular growth, differentiation, and apoptosis of neural network including signaling by NTRK1 or ALK receptor tyrosine kinases, and telomerase activity. And this presentation discussesdiagnostic and prognostic molecular makers for extensive heterogeneity of euroblastoma. This should lead to more risk-adapted therapies according to the genetic markers by which individual neuroblastomas are biologically characterized.

Efficacy of rasburicase in pediatric patients with leukemia and lymphoma is proved. This study aims to weigh efficacy and safety of rasburicase versus more conventional therapy, allopurinol, and to compare their safety and properties in tumor lysis syndrome (TLS) of leukemia and lymphoma patients. The study was done with a retrospective cohort design. Patients were selected from our hematology ward admitted from 2005 through 2008. Patients were put into two groups based on their blood levels of uric acid, before initiation of chemotherapy; treatment group (the Uric Acid level of 6.5 mg/dl or more) and the prophylaxis group (the uric acid level below 6.5 mg/dl). Evaluation of effectiveness of therapy was performed after 24, 48, 72-hour, and longer periods. Of 184 patients; 69% had leukemia, and 31% lymphoma. Twenty patients were treated with rasburicase and 164 with allopurinol. Mean age of patients was 7.93± 4.247 years old. 60.8% were male and 39.2% were female.According to Chi-square test results, there was no significant difference between two agents regarding prophylaxis (chi-square = 4.247, p-value = 0.193) and treatment (chi-square = 0.780, p-value = 0.677). Most of the response to each agent was seen in the first 24 hours after drug administration. Mean level of uric acid reduced from 7.4 to 3.4mg/dl in rasburicase, and from 5.4 to 3.9 mg/dl in allopurinol group. Mean duration of treatment for rasburicase was 2 days, and for allopurinol 6 days. Adverse effects were minimal in both groups (in rasburicase 1.6% and in allopurinol 5.4%). Rasburicase seems to be highly efficient in both prophylaxis and treatment of Hyperuricemia. Due to high costs in our practice, it was only administered to 20 patients with high levels of blood uric acid or leukocytosis. It prepares patients for chemotherapy faster and decreases cost of hospital stay indirectly by lowering cost of treatment. Allopurinol, alternatively showed equal efficiency and comparable results. Thus, it can be used safely and effectively until rasburicase becomes more widely available and more cost-effective.

Patients with prolonged neutropenia and/or severe underlying immunosuppression are at the greatest risk for disseminated aspergillosis. This study was undertaken to determine the incidence of invasive aspergillosis by Platelia Aspergillus enzyme-linked immunosorbent assay ELISA kit in high risk children admitted tothe hematology ward of Dr. Faghihi hospital, Shiraz University of Medical Sciences, Iran. From Oct. 2006 to Jun. 2008, 62 patients with hematologic malignancies were followed and evaluated for invasive aspergillosis in Shiraz. All clinical samples were cultured and a direct microscopic examination was performed. Blood samples were cultured by bedside inoculation to BACTEC medium. Blood samples were collected prospectively once a week and stored at –20 ˚C until examination. All the collected blood samples were assayed for galactomannan antigen using Platelia Aspergillus ELISA kit. Patients were classified according to the diagnostic criteria set by the European Organization for Research and Treatment of Cancer-Mycosis Study Group. The female-to-male ratio was 22:40, and mean age of the patients was 9.3 years. The sensitivity, specificity, negative and positive predictive values of the ELISA method were 91%, 90%, 83.3%, and 94.7%, respectively. Galactomannan antigen test was positive in 1 proven, 8 probable, and 2 possible cases. The incidence rate of invasiveaspergillosis was found to be 16.7%. Considering the incidence of invasive aspergillosis and the corresponding morbidity and mortality rates in patients with hematologic disorders, it seems that more efficient methods are in demand for early diagnosis and thereby promoting the patients’ survival.

Hematopoietic stem cell transplantation (HSCT) has been practiced for more than 30 years. Hematological malignancies are the main indications for this treatment. However, its indications in adults are different from children. Advances in chemotherapy and target therapy have improved treatment outcome of some of the very high riskcancers, and changed indications of HSCT in children. Multi-center clinical trials evaluating outcome of childhood cancers using different therapeutic protocols are needed to precisely define the role of HSCT. A review on the current literature about HSCT indications in pediatric cancers is presented in this manuscript.

Langerhans cell histiocytosis (LCH) is a rare disorder described as three different entities including eosinophilic granuloma of bone, the Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. LCH is currently classified into single system LCH, and multisystem LCH. Patients with single system LCH have an excellent prognosis, and are mostlytreated with local therapy. Multisystem LCH is subdivided into low risk and high risk groups. A 6-week course of PRED/VBL is recommended for all patients with MS-LCH. Further therapy depends on the response to the initial course, and risk group of the patient.

Bone sarcomas are the most common malignancies of bone tissues in children, and are classified into two groups as osteosarcoma and Ewing’s Sarcoma. Treatment and prognosis depend on the subtype and grade of the tumor. The goal of this study was to evaluate the features bone sarcoma in patients referred to MAHAK children hospital since 2007 to 2009. This was a retrospective study of patients with bone sarcoma referred to MAHAK children hospital for continuing their treatment or as a new case of cancer. All of the patients had local pain, local swelling, and decreased range of motion and pathologic fractures as the presenting symptom. All patients underwentbone scan, chest computed tomography (CT) scan, chest X- ray, echocardiography, hearing tests, and bone marrow aspiration/biopsy. All of the data were analyzed by SAS software. In this study, 36 patients with bone sarcoma were included with the age range of 3 to 20 years old (55% male and 45% female). Analysis showed that local pain and local swelling were the most frequent local manifestations in patients. The most common primary tumor site was distal femur (30%). Treatment for the patients at MAHAKchildren hospital is performed according to German protocol. Out of 36 patients, 75% were followed until 2008 and 25% until 2009. According to the analysis we can conclude that these data are similar to other studies. To achieve the best results, oncologists have to modify the treatment of patients completely.

In the past 20 years, a dramatic improvement in the prognosis of patients with hepatoblastoma (HB) has been achieved by combining surgery and chemotherapy in several national and international trials. Four children (3 girl and 1 boy) aged 34 days, 4 months, 13 months, and 1 year with mean age of 6.53 (±5.15) months were presented to our center from 2002 to 2009 with abdominal mass and abdominal distention. Their abdominal ultrasonography revealed liver mass, and pathological diagnosis was hepatoblasma. They were treated with a combination of surgery and chemotherapy. Patients with non-metastatic HB were treated at Shahid Sadoughi Hospital of Yazd. A complete resection of primary tumor was achieved in all of them. The median follow- up of the patients was 38.25 (± 29.02) month and all of them (100%) remained alive with complete remission. Complete resection with chemotherapy can improve prognosis of hepatoblastoma patients.