Neuroblastoma in adults: Incidence and survival analysis based on SEER data

PURPOSE: Clinical data on survival outcomes of adult patients (defined as 20 years of age and older) with neuroblastoma are scarce due to the rarity of the disease. Small single institution reports have described worse outcomes for adults than pediatric patients. MATERIALS/METHODS: Data from the public access Surveillance Epidemiology and End Results database were reviewed for a 30-year period between 1973 and 2002 for the diagnosis of neuroblastoma. Analyses compared frequency and survival rates of neuroblastoma among children and adults with particular focus on the incidence, case characteristics, and long-term survival of the adult patients. RESULTS: The observed 3- and 5-year survival rates were lowest among adult patients (45.9% and 36.3%, respectively), whereas infants fared best with 86.0% 3-year and 84.6% 5-year overall survival. There was a steady decline in the incidence of adult neuroblastoma from 0.47 cases per million per year in 1973-1977 to 0.12 cases per million per year in 1998-2002. When the long-term adult neuroblastoma survival was examined in detail for 1-, 3-, 5-, 7-, and 10-year follow-up periods, there was continuous decrease in survival during the first 7 years after the diagnosis with subsequent stabilization for both observed (OS) and relative survival (RS) rates. There was a notable trend towards improved outcome over the past three decades. CONCLUSIONS: Adults with neuroblastoma have significantly worse outcome than children. This may be due to tumor biology, more virulent clinical course, or possibly due to the fact that adults are less sensitive or have poor tolerance to pediatric chemotherapy regimens.     

Treatment challenges and outcomes for pediatric intracranial ependymoma at a single institution in Iran

Background: The scarcity of information on pediatric ependymoma in Iran motivated this study. Our main objectives were to determine outcomes, identify clinical management challenges at a nongovernment hospital in Iran, and devise guidelines for improving care.

Procedure: A retrospective chart review was performed for pediatric patients with ependymoma who were younger than 15 years and treated at MPCTRC between 2007 and 2015. Records included patient demographics, treatment regimens used, duration of follow-up, and outcomes. Clinical outcomes [ie, 3-year overall survival (OS) and progression-free survival (PFS)] were determined based on the age at diagnosis (younger or older than 3 years) by using the Kaplan–Meier method.

Results: In total, 73 eligible patients were enrolled; 20 patients were in the younger group, and 53 were in the older group. The majority (91.8%, n = 67) of patients underwent initial gross-total or partial surgical resection, and 6 (8.2%) had a biopsy. Twenty-one patients experienced ependymoma recurrence. The median time to relapse was 1 year. The median duration of follow-up and PFS were 25 and 17 months, respectively. The 3-year OS and PFS were 61% and 59.5%, respectively. At the time of this project, 27 patients had died, and 35 were alive with no evidence of disease.

Conclusion: Our study demonstrated inferior outcomes of Iranian children with ependymoma. To improve our care for these children, a paradigm shift must occur that includes radiation therapy as standard of care, second-look surgery, a multidisciplinary team approach, and potentially twinning initiatives.     

Poor performance status,urban residence and female sex predict inferior survival in pediatric advanced stage mature B-NHL in an Indian tertiary care center

Background: Advanced stage is a known prognostic factor in B-Non-Hodgkin Lymphoma (NHL); however, factors within advanced stage and overall data on pediatric B-NHL from India are lacking. Methods: This is a retrospective study wherein all consecutive pediatric (≤18 years) patients of advanced stage B-NHL (St. Jude stage 3 and 4) treated at our center from Jan 2003 to June 2016 with BFM-90 protocol were evaluated for outcome and pathology review. Results: Total 140 patients were analyzed with median age 8 years; M:F ratio was 5.2:1; 36% patients presented within 30 days of symptom onset and 58% had rural residence. Burkitt lymphoma (66%) was commonest histopathological subtype; bone marrow was involved in 15% and CSF in 8% cases. Undernourishment was observed in 30% patients and 51% had ECOG performance status of 3&4. At 5 years, EFS was 52 ± 4% (CI 0.43–0.60) and OS was 61 ± 4% (CI 0.52–0.68). On multivariate analysis, poor performance status (p < 0.001) and urban residence (p = 0.016) emerged as significant negative prognostic factors for EFS; while for OS, female sex (p = 0.006), poor performance status (p < 0.001) and urban residence (p = 0.023) predicted inferior outcome. Conclusion: This is the largest study from south Asia on advanced stage pediatric B-NHL and it suggests undernourishment, poor performance status and gender bias to be unique features at presentation. Although, outcomes are comparable with other data from resource-challenged nations, yet they are 15–20% inferior than trial data from other developed countries. Further, poor performance status, female sex and urban residence for poor outcome were identified as unique prognostic factors.     

Gender affects survival for medulloblastoma only in older children and adults: A study from the surveillance epidemiology and end results registry

Background

Males have a higher incidence of medulloblastoma (MB) than females, but the effect of gender on survival is unclear. Studies have yielded conflicting results, possibly due to small sample sizes or differences in how researchers defined MB. We aimed to determine the effect of gender on survival in MB using a large data set and strict criteria for defining MB.

Procedure

A sample of 1,226 subjects (763 males and 463 females) was identified from 1973 to 2002, using the Surveillance Epidemiology and End Results (SEER‐9) registry. MB was strictly defined to exclude non‐cerebellar embryonal tumors (primitive neuro‐ectodermal tumors). Because children <3 years of age are known to have worse survival, patients were stratified by age <3 years at diagnosis (95 males, 82 females) and >3 years (668 males, 381 females).

Results

Overall, there was no significant difference in survival between males and females (log rank P = 0.22). However, among subjects >3 years, females had significantly greater survival than males (log rank P = 0.02). In children <3 years, there was a non‐significant trend toward poorer survival in females (median survival: males 27 months, females 13 months; log rank P = 0.24). This interaction between age group and gender was statistically significant (P = 0.03).

Conclusion

Females with MB have a survival advantage only in subjects >3 years. In children <3 years, females may even have poorer outcome. The effect of gender on survival and incidence in MB warrants additional biologic investigation, and may differ in very young children with MB. Pediatr Blood Cancer 2009;52:60–64. © 2008 Wiley‐Liss, Inc.     

Extraneural ependymoma: Distant bone,lung, liver,and lymph node metastases following bevacizumab

Extraneural metastases of ependymoma are rare, and have been reported in the lungs, lymph nodes, pleura, mediastinum, liver, diaphragmatic muscle, and bone. We report a case of anaplastic ependymoma with distant metastases to the vertebral bones, lungs, liver, and lymph nodes following treatment with bevacizumab. Recent research has hypothesized that angiogenic tumors may develop means of resistance to antiangiogenic therapies, and some evidence suggests potential for antiangiogenic therapies to promote additional means for cancer spread. Nevertheless, antiangiogenic therapies continue to demonstrate potential as potent therapies for the treatment of many cancers, and should continue to be researched for future uses. Pediatr Blood Cancer 2013; 60: 143–145. © 2012 Wiley Periodicals, Inc.     

Perception and management of cancer predisposition in pediatric cancer centers: A European-wide questionnaire-based survey

The European Union-funded COST Action (LEukaemia GENe Discovery by data sharing, mining, and collaboration) LEGEND was an international and multidisciplinary collaboration between clinicians and researchers that covered a range of aspects of genetic predisposition in childhood leukemia. Within this framework, we explored the perception and handling of genetic predisposition in the daily practice of European treatment centers. Herein, we present the results of our questionnaire-based survey. We found that the overall awareness is quite high, and respondents remarked that identification and treatment of the most common predisposition syndromes were present. Nevertheless, high demand for continuous education and routinely updated resources remains.     

Pediatric Hematology and Oncology in Norway: A Brief Historical Review

Before World War II no real subspecialization occurred in Norwegian pediatrics. In the early 1950s hematology and oncology were adopted as fields of special interest by a Jew Norwegian pediatricians. Gradually an increasing expansion has taken place, particularly in pediatric oncology, in parallel with the great advances in cancer treatment.