Understanding the trajectory of research efforts in atypical teratoid rhabdoid tumors: a bibliometric analysis of the 50 most impactful studies to date.

PURPOSE: Atypical teratoid/rhabdoid tumor (ATRT) is a highly malignant embryonal tumor of the central nervous system (CNS) that occurs predominantly in children. More is being discovered about this disease to improve understanding and outcomes. The aim of this analysis was to evaluate citation and other bibliometric characteristics of the 50 most cited articles in the contemporary literature in order to better model the trajectory of our current efforts. METHODS: Elsevier's Scopus database was searched for the 50 most cited articles about ATRT. To look for trends, earliest 25 articles were separated from the latest 25 articles and then were compared. Various bibliometric parameters were summarized and compared using Pearson's chi-square and Mann-Whitney U tests. RESULTS: The 50 most cited articles were published between 1990 and 2016, from 5 unique countries in 29 unique journals, with genetic and retrospective observational cohort studies the most common design (n = 11 each). Overall median values were as follows: citation count, 145.4 citations (range, 67-626); citation rate per year, 11.7 (range, 3.5-51.4); number of authors 12 (range, 1-95); with 32 (64%) originating from the USA. Compared with older articles, newer articles had statistically lower citation counts (101.8 vs 189.0; P < 0.01), higher number of authors (17.3 vs 6.6; P < 0.01), and were less likely published from the USA (40% vs 88%; P < 0.01) CONCLUSIONS: The 50 most cited articles about ATRT were characterized in this analysis. There was a distinct focus in these studies on the genetic composition and consequences of these tumors. Trends over time suggest greater impact will be had in highly collaborative efforts worldwide. Moving forward, it will be of great interest to see how the findings of these basic science finding will translate into future clinical studies.

Recurring pediatric anaplastic ependymoma with rare peritoneal carcinomatosis: a case report and hypothesis of mechanism.

BACKGROUND: Although recurrent anaplastic ependymoma in pediatric patients is not uncommon, recurrent disease leading to widespread metastases to the peritoneum is extremely rare. CASE REPORT: We present a case of an 18-month old male who initially presented with posterior fossa anaplastic ependymoma, who then proceeded to present 1 year later with spinal recurrence, and then 2 years after that with widespread disease involving the intracranial ventricular system and peritoneum. CONCLUSION: We posit that surgical interventions to treat primary and recurrent presentations in combination with a conduit to the peritoneum via a ventriculoperitoneal shunt contributed to the mechanisms of this complex case.

Effect of surgery and chemotherapy on long-term survival in infants with congenital glioblastoma: an integrated survival analysis.

OBJECTIVE: Glioblastoma (GBM) during infancy is rare, and the clinical outcomes of congenital GBM are not well understood. Correspondingly, the aim of this study was to present a long-term survivor case from the authors' institution, and establish an integrated cohort of cases across the published literature to better understand the clinical course of this disease in this setting. METHODS: The authors report the outcomes of an institutional case of congenital GBM diagnosed within the first 3 months of life, and performed a comprehensive literature search for published cases from 2000 onward for an integrated survival analysis. All cases were integrated into 1 cohort, and Kaplan-Meier estimations, Fisher's exact test, and logistic regression were used to interrogate the data. RESULTS: The integrated cohort of 40 congenital GBM cases consisted of 23 (58%) females and 17 (42%) males born at a median gestational age of 38 weeks (range 22-40 weeks). Estimates of overall survival (OS) at 1 month was 67%, at 1 year it was 59%, and at 10 years it was 45%, with statistically superior outcomes for subgroups in which patients survived to be treated by resection and chemotherapy. In the overall cohort, multivariable analysis confirmed resection (p < 0.01) and chemotherapy (p < 0.01) as independent predictors of superior OS. Gestational age > 38 weeks (p < 0.01), Apgar scores ≥ 7 at 5 minutes (p < 0.01), absence of prenatal hydrocephalus (p < 0.01), and vaginal delivery (p < 0.01) were associated with greater odds of surgical diagnosis versus autopsy diagnosis. CONCLUSIONS: Congenital GBM can deviate from the expected poor prognosis of adult GBM in terms of OS. Both resection and chemotherapy confer statistically superior prognostic advantages in those patients who survive within the immediate postnatal period, and should be first-line considerations in the initial management of this rare disease.