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BMJ Case Reports Jun 2015We present a case of a 19-year-old man with cervical lymphadenopathy diagnosed with classical Hodgkin's lymphoma 9 years after gross total resection of a third...
We present a case of a 19-year-old man with cervical lymphadenopathy diagnosed with classical Hodgkin's lymphoma 9 years after gross total resection of a third ventricular juvenile pilocytic astrocytoma (JPA). Chemotherapy or radiation therapy was not a part of his initial JPA treatment. Owing to his two primary neoplasms, genetic testing was performed, which revealed heterozygous polymorphisms of unknown significance for CDH1 and p53, and negative BRAF mutation analysis. Our case reports development of classical Hodgkin's lymphoma after JPA in the absence of antecedent radiation and/or chemotherapy, and identifiable genetic predisposition.
Topics: Adolescent; Adult; Astrocytoma; Brain Neoplasms; Genetic Predisposition to Disease; Genetic Testing; Hodgkin Disease; Humans; Male; Polymorphism, Genetic; Young Adult
PubMed: 26113587
DOI: 10.1136/bcr-2015-209343 -
Neuro-oncology Practice Dec 2014Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic encephalopathic state associated with reversible cerebral vasogenic edema. It is an increasingly...
BACKGROUND
Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic encephalopathic state associated with reversible cerebral vasogenic edema. It is an increasingly recognized occurrence in the oncology population. However, it is very uncommon in patients with primary brain tumors (PBTs). The aim of this study was to analyze the clinicoradiological features and report the clinical outcomes of PRES in PBT patients.
METHODS
We identified 4 cases with PBT who developed PRES at MD Anderson Cancer Center (MDACC) between 2012 and 2014. Clinical and radiological data were abstracted from their records. In addition, we also solicited 8 cases from the literature.
RESULTS
The median age at PRES onset was 19 years, male-to-female ratio was 1:1, and the syndrome occurred in patients with ependymoma ( = 4), glioblastoma ( = 3), diffuse intrinsic pontine glioma (DIPG; = 3), juvenile pilocytic astrocytoma ( = 1), and atypical meningioma ( = 1). Two glioblastomas and 2 DIPG cases received bevacizumab and vandetanib before the onset of symptoms, respectively. The most common clinical presentation was seizures ( = 7). Three MDACC patients recovered completely in 3-4 weeks after the onset of symptoms. One patient died due to active cancer and several comorbidities including PRES.
CONCLUSIONS
Hypertension seems to be the most important coexisting risk factor for development of PRES; however, the potential effects of chemotherapeutic agents in the pathogenesis of PRES should also be examined. The clinicoradiological course of PRES in PBT patients did not vary from the classical descriptions of PRES found in other causes. PRES must be considered as part of the differential diagnosis in patients with PBTs presenting with seizures or acute encephalopathy.
PubMed: 26034631
DOI: 10.1093/nop/npu024 -
Pediatric Blood & Cancer Sep 2015Due to increasing concerns regarding air pollution and childhood cancer, we conducted a population-based study evaluating the association between traffic-related...
BACKGROUND
Due to increasing concerns regarding air pollution and childhood cancer, we conducted a population-based study evaluating the association between traffic-related hazardous air pollutants (1,3-butadiene, benzene, diesel particulate matter [DPM]) and the incidence of childhood central nervous system (CNS) tumors.
PROCEDURE
Information on children diagnosed with a CNS tumor at <15 years of age, in Texas, for the period of 2001-2009 (n = 1,949) was obtained from the Texas Cancer Registry. Information on the corresponding at-risk population was obtained from the United States (U.S.) Census. Annual census tract-level pollutant concentrations, estimated by the U.S. Environmental Protection Agency, were categorized based on quartiles (low, medium, medium-high, and high) of the statewide distribution. Multivariable Poisson regression was used to calculate adjusted incidence rate ratios (aIRR). Juvenile pilocytic astrocytomas (JPAs) (n = 384), other astrocytomas (n = 372), ependymomas (n = 142), medulloblastomas (n = 235), and primitive neuroectodermal tumors (PNET) (n = 47) were evaluated.
RESULTS
Census tracts with medium and medium-high 1,3-butadiene concentrations had higher astrocytoma incidence rates (aIRR [95% confidence interval (CI)]: 1.46 [1.05-2.01] and 1.69 [1.22-2.33], respectively) compared with low concentrations. Census tracts with medium DPM concentrations had higher astrocytoma (aIRR [95%CI]: 1.42 [1.05-1.94]) and medulloblastoma (aIRR [95%CI]: 1.46 [1.01-2.12]) incidence rates compared with low concentrations. Increased concentrations of 1,3-butadiene and benzene were strongly associated with increased PNET incidence rates, but were not statistically significant. No associations were detected with JPA or ependymoma incidence.
CONCLUSIONS
In one of the largest studies of its kind, our results suggest positive associations between hazardous air pollutants and incidence of astrocytoma (1,3-butadiene and DPM) and medulloblastoma (DPM).
Topics: Adolescent; Air Pollutants; Air Pollution; Astrocytoma; Benzene; Butadienes; Central Nervous System Neoplasms; Child; Child, Preschool; Ependymoma; Ethnicity; Female; Humans; Incidence; Infant; Male; Medulloblastoma; Neuroectodermal Tumors, Primitive; Particulate Matter; Poverty Areas; Registries; Socioeconomic Factors; Texas; Vehicle Emissions
PubMed: 25962758
DOI: 10.1002/pbc.25549 -
Oncology (Williston Park, N.Y.) Apr 2015
PubMed: 25930896
DOI: No ID Found -
No To Hattatsu = Brain and Development Nov 2014I retrospectively reviewed the histopathologic features of surgical specimens taken consecutively from 600 patients with intractable epilepsy, and showed the scope of... (Review)
Review
I retrospectively reviewed the histopathologic features of surgical specimens taken consecutively from 600 patients with intractable epilepsy, and showed the scope of variation in lesions responsible for epileptogenesis. The patients were divided into three groups on the basis of age at seizure onset: 94 patients with infantile onset (before 1 year of age), 307 patients with juvenile onset (between 1 and 12 years of age), and 199 patients with adolescent/adult onset (at 13 years of age or beyond). In the infant group, seizure duration was significantly shorter than in the other groups, and malformations caused by abnormalities of cortical development, including focal cortical dysplasia (FCD) type II a/b, tuberous sclerosis, hemimegalencephaly, and polymicrogyria were predominant, whereas in the juvenile and adolescent/adult groups, other lesions such as hippocampal sclerosis (HS), tumors, FCD type I, and vascular lesions were frequently observed. FCD type III a was noted in nearly half of patient with HS in both juvenile and adolescent/adult groups. The causative tumors included dysembryoplastic neuroepithelial tumors, gangliogliomas, astrocytomas, and other glioneuronal and glial tumors. Thus, various histopathological entities and types, showing clear predominance depending on the age at seizure onset, were observed in patients with epilepsy. These features appear to provide information on the pathomechanisms of the lesions and their clinical relevance in affected patients.
Topics: Age of Onset; Brain; Child; Epilepsy; Humans; Infant; Neurons; Retrospective Studies
PubMed: 25558583
DOI: No ID Found -
Journal of Neuro-oncology Mar 2015To assess frequency of neural stem cell compartment (NSC) involvement in adult and pediatric gliomas [World Health Organization (WHO) grades 1-4], and to assess whether... (Comparative Study)
Comparative Study
To assess frequency of neural stem cell compartment (NSC) involvement in adult and pediatric gliomas [World Health Organization (WHO) grades 1-4], and to assess whether NSC involvement at presentation impacts on survival, recurrence rates, and/or transformation from low grade (WHO grade 1-2) to high grade disease (WHO grades 3-4). Cranial MRIs for 154 pediatric and 223 adult glioma patients treated from 2000 to 2012 were reviewed. NSC involvement was documented. Tumors were stratified by age (adult vs. pediatric), histology, tumor grade, tumor location, and involvement of midline structures. Odds ratios (OR) for death were calculated based on NSC status at presentation. Rates of transformation and recurrence rates (ORR) were compared using Fisher's Exact Test. Time to recurrence (TTR) was calculated using student t test. Among recurrent and transformed tumors, we also assessed the rate of NSC involvement at time of recurrence or transformation. 74.8 % of tumors had NSC involvement. Higher rates of NSC involvement were seen among adult (p = .0001); high grade (p = .0001)); grade 2 versus grade 1 (p = .0001) and other grade 1 histologies (p = .0001) versus JPA (juvenile pilocytic astrocytoma) patients); grade 2-4 tumors (p = .0001); and supratentorial tumors (p < .0001). No transformation was noted among pediatric low grade tumors or adult grade 1 tumors. 22/119 (18.5 %) adult grade 2 tumors transformed. Rates of transformation were not impacted by NSC status (p = .47). ORR was 15.1 %, and was greater for NSC+ tumors at presentation (p = .05). 36/41 recurrences (87.8 %) involved NSC at time of recurrence. OR for death was 2.62 (1.16-5.9), p = .02 for NSC+ tumors at presentation. Adult and pediatric gliomas (all grades) frequently involve NSC at presentation, although rates are lower in pediatric JPA and all infratentorial tumors. NSC involvement at presentation increases OR death and reduces TTR for pediatric gliomas (all grades) and adult low grade gliomas, and shows a strong trend toward increased ORR.
Topics: Adult; Astrocytoma; Brain Neoplasms; Child; Female; Follow-Up Studies; Glioma; Humans; Magnetic Resonance Imaging; Male; Neoplasm Grading; Neoplasm Recurrence, Local; Neoplastic Stem Cells; Neural Stem Cells; Prognosis; Retrospective Studies; Survival Rate; Young Adult
PubMed: 25502962
DOI: 10.1007/s11060-014-1682-0 -
Journal of Clinical Research in... Sep 2014Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable...
Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable etiology. Hypothalamic hamartoma (HH), hydrocephalus, tumors, infections, congenital defects, ischemia, radiation, or injury of the brain are the most common causes of secondary CPP. In this report, we present the case of a 2 years and 9 months old male patient who had a 30x40 mm contrast-enhancing suprasellar mass and was histopathologically diagnosed with giant HH. However, since HHs are designated as non-enhancing masses, considering the possibility of an incomplete diagnosis of a glial tumor, the patient was followed up. Clinical and radiological follow-up revealed stable findings with no evidence of tumor growth until the third year after surgery when he presented with neurological deficit due to the rapid growth of the suprasellar mass. After the second surgery, histopathological examination of the biopsy specimen revealed the lesion to be a juvenile pilocytic astrocytoma (PA). The concomitance of HH and juvenile PA is very rare. To our knowledge, this is the first report of a patient with concomitant juvenile PA and HH who developed CPP and did not have gelastic epilepsy despite the rapidly growing giant mass.
Topics: Astrocytoma; Biopsy; Child; Hamartoma; Humans; Hypothalamic Diseases; Magnetic Resonance Imaging; Male; Neoplasms, Multiple Primary; Puberty, Precocious; Treatment Outcome
PubMed: 25241615
DOI: 10.4274/Jcrpe.1306 -
Pediatric Blood & Cancer Jan 2015Increasing mass effect following radiation therapy (RT) in patients with low-grade gliomas (LGGs) can be mistaken for tumor progression and/or malignant degeneration....
BACKGROUND
Increasing mass effect following radiation therapy (RT) in patients with low-grade gliomas (LGGs) can be mistaken for tumor progression and/or malignant degeneration. Distinguishing pseudoprogression (PP) from true progression is crucial, with vastly different treatment approaches and prognoses.
PROCEDURE
Patients treated with RT for LGGs through the Children's Hospital of Pittsburgh Neuro-Oncology Program are considered to have PP and managed conservatively if they develop increased mass effect within 3 years of RT. Pre-RT tumor area was compared to the maximum tumor size following RT and the size on the last follow-up scan by a central reviewer.
RESULTS
Twenty-four children, median age 13 years, received external beam RT for LGG between March 2000 and August 2011. Thirteen patients (54.2%) developed an increase in tumor size compared to baseline beginning at a median of 6 months after RT and lasting for a median of 2.1 years (range 6.5 months to 5.1 years). Maximum tumor enlargement occurred at a median of 8 months after RT, with a range (5 months to 4.2 years). In all 13 cases, the tumor eventually decreased in size without additional anti-tumor therapy. Two patients (8.3%) developed true tumor progression. With a median follow-up of 4.9 years (range 1.0-12.4 years), all patients are alive.
CONCLUSIONS
Pseudoprogression occurred in more than half of the children with LGG following RT, typically beginning within 8 months and often running a very protracted course. Late presentations can also occur.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Diagnostic Imaging; Disease Progression; Female; Follow-Up Studies; Glioma; Humans; Male; Neoplasm Grading; Prognosis; Radiation Injuries; Radiotherapy; Survival Rate
PubMed: 25213668
DOI: 10.1002/pbc.25179 -
Cancer Genetics Sep 2014Pediatric brain tumors such as atypical teratoid rhabdoid tumors (ATRTs) are highly aggressive and predominantly occur in young children. A characteristic feature of...
Pediatric brain tumors such as atypical teratoid rhabdoid tumors (ATRTs) are highly aggressive and predominantly occur in young children. A characteristic feature of ATRT is aberrations of the SMARCB1 (hSNF5/INI1) gene. Developmental gene defects may play an important role in the biology of pediatric brain tumors. HOX genes are transcription factors that play a pivotal role in anterior-posterior body axis patterning and are misexpressed in tumors such as lung carcinoma, neuroblastoma, and glioma. HOX genes are also known to be associated with long noncoding RNAs (lncRNAs) such as HOTAIR, which induces transcriptional silencing of the HOXD locus by recruiting polycomb repressive complex 2 to the HOXD locus. In this study, transcriptome analysis using the nanoString platform was performed, and expression of the HOX and HOTAIR genes was studied in pediatric tumors: 20 ATRTs, 10 ependymomas, 10 medulloblastomas, six glioblastoma multiforme, and nine juvenile pilocytic astrocytomas (JPAs). Results indicate that in ATRTs, medulloblastomas, and JPAs, the HOTAIR and HOXC genes are highly expressed; however, HOXD8-10 genes are not silenced. In ependymomas, there is low expression of the HOXC, HOTAIR, and HOXD8-10 genes. These interesting results need to be elucidated further so that the functions of these genes in pediatric tumors is understood.
Topics: Astrocytoma; Brain Neoplasms; Chromosomal Proteins, Non-Histone; DNA-Binding Proteins; Ependymoma; Gene Expression Profiling; Genes, Homeobox; Glioblastoma; Homeodomain Proteins; Humans; Medulloblastoma; RNA, Long Noncoding; Rhabdoid Tumor; SMARCB1 Protein; Teratoma; Transcription Factors
PubMed: 25085602
DOI: 10.1016/j.cancergen.2014.05.014 -
Journal of Neurosurgery. Pediatrics Oct 2014Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to...
OBJECT
Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to use imaging to evaluate how often tumors recurred and to determine if recurrences were associated with any clinical symptoms, along with the financial costs of imaging, in patients with radiographically proven gross-total resection (GTR) at Boston Children's Hospital. These data were assessed to propose guidelines regarding postoperative surveillance.
METHODS
The authors performed a retrospective cohort analysis of the Pediatric Brain Tumor Program database from 1993 to 2003 to identify patients with glial or glioneuronal tumors initially evaluated at Boston Children's Hospital. Among the 888 patients evaluated for any type of brain tumor during this period, 67 patients had WHO Grade I glial or glioneuronal lesions with radiographically proven GTR and available follow-up data. The frequency and timing of postoperative imaging was compared with the institutional protocol. Recurrence-free survival was calculated using the Kaplan-Meier method. Financial costs of imaging were available from 2001 to 2009 and were averaged to extrapolate the postoperative surveillance costs.
RESULTS
Among the 67 patients with GTR, 13 recurrences were detected radiographically with a mean time to recurrence of 32.4 months (range 2.9-128.5 months). The mean duration of follow-up after surgery was 6.6 years. The recurrence-free survival at 2 and 5 years after GTR for all low-grade glial and glioneuronal tumors was 0.90 (95% CI 0.82-0.97) and 0.82 (95% CI 0.73-0.92), respectively. No clinical symptoms were associated with any of the recurrences, and no deaths occurred. Under the institutional protocol of surveillance imaging, the estimated cost per recurrence at 5 years was $104,094 per patient. The proposed protocol would reduce the number of MR scans in the first 5 years from 10 to 5, providing a potential cost savings of $52,047 per recurrence.
CONCLUSIONS
Given the slow-growing, clinically asymptomatic nature of low-grade glial and glioneuronal tumors coupled with the financial and psychological costs of repeated imaging, the authors propose a postoperative surveillance MRI schedule that is less intensive than current institutional practice.
Topics: Adolescent; Boston; Brain Neoplasms; Child; Child, Preschool; Disease-Free Survival; Female; Follow-Up Studies; Humans; Infant; Kaplan-Meier Estimate; Male; Neoplasm Grading; Neoplasm Recurrence, Local; Neuroimaging; Postoperative Period; Retrospective Studies; Time Factors; Young Adult
PubMed: 25062303
DOI: 10.3171/2014.6.PEDS1321