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1.
AJNR Am J Neuroradiol ; 42(12): 2245-2250, 2021 12.
Article in English | MEDLINE | ID: mdl-34674998

ABSTRACT

BACKGROUND AND PURPOSE: Posterior fossa type A (PFA) ependymomas have 2 molecular subgroups (PFA-1 and PFA-2) and 9 subtypes. Gene expression profiling suggests that PFA-1 and PFA-2 tumors have distinct developmental origins at different rostrocaudal levels of the brainstem. We, therefore, tested the hypothesis that PFA-1 and PFA-2 ependymomas have different anatomic MR imaging characteristics at presentation. MATERIALS AND METHODS: Two neuroradiologists reviewed the preoperative MR imaging examinations of 122 patients with PFA ependymomas and identified several anatomic characteristics, including extension through the fourth ventricular foramina and encasement of major arteries and tumor type (midfloor, roof, or lateral). Deoxyribonucleic acid methylation profiling assigned ependymomas to PFA-1 or PFA-2. Information on PFA subtype from an earlier study was also available for a subset of tumors. Associations between imaging variables and subgroup or subtype were evaluated. RESULTS: No anatomic imaging variable was significantly associated with the PFA subgroup, but 5 PFA-2c subtype ependymomas in the cohort had a more circumscribed appearance and showed less tendency to extend through the fourth ventricular foramina or encase blood vessels, compared with other PFA subtypes. CONCLUSIONS: PFA-1 and PFA-2 ependymomas did not have different anatomic MR imaging characteristics, and these results do not support the hypothesis that they have distinct anatomic origins. PFA-2c ependymomas appear to have a more anatomically circumscribed MR imaging appearance than the other PFA subtypes; however, this needs to be confirmed in a larger study.


Subject(s)
Ependymoma , Infratentorial Neoplasms , Cohort Studies , Ependymoma/diagnostic imaging , Ependymoma/genetics , Ependymoma/pathology , Humans , Infratentorial Neoplasms/diagnostic imaging , Magnetic Resonance Imaging , Neuroimaging
2.
AJNR Am J Neuroradiol ; 37(12): 2370-2375, 2016 Dec.
Article in English | MEDLINE | ID: mdl-27469209

ABSTRACT

BACKGROUND AND PURPOSE: Pilocytic astrocytomas, the most common posterior fossa tumors in children, are characterized by KIAA1549-BRAF fusions and shows excellent 5-year survival rates. Pilocytic astrocytoma with gangliocytic differentiation, a recently defined pilocytic astrocytoma variant that includes glial and neuronal elements similar to a ganglioglioma, may be distinguished from a classic ganglioglioma by molecular, radiologic, and histopathologic features. This study investigated whether imaging could distinguish posterior fossa pilocytic astrocytoma with and without gangliocytic differentiation. MATERIALS AND METHODS: Preoperative MRIs (± CTs) of 41 children (age range, 7 months to 15 years; mean age, 7.3 ± 3.7 years; 58.5% male) with pilocytic astrocytoma with gangliocytic differentiation (n = 7) or pilocytic astrocytoma (n = 34) were evaluated; differences in tumor location, morphology, and minimum relative ADC between tumor types were compared (Wilcoxon rank sum test, Fisher exact test). Histopathology and BRAF fusion/mutation status were reviewed. Associations of progression-free survival with diagnosis, imaging features, and BRAF status were examined by Cox proportional hazards models. RESULTS: Pilocytic astrocytoma with gangliocytic differentiation appeared similar to pilocytic astrocytoma but had lower minimum relative ADC (mean, 1.01 ± 0.17 compared with 2.01 ± 0.38 for pilocytic astrocytoma; P = .0005) and was more commonly located within midline structures (P = .0034). BRAF status was similar for both groups. Non-total resection (hazard ratio, 52.64; P = .0002), pilocytic astrocytoma with gangliocytic differentiation diagnosis (hazard ratio, 4.66; P = .0104), and midline involvement (hazard ratio, 3.32; P = .0433) were associated with shorter progression-free survival. CONCLUSIONS: Minimum relative ADC and tumor location may be useful adjuncts to histopathology in differentiating pilocytic astrocytoma with gangliocytic differentiation from pilocytic astrocytoma. Shorter progression-free survival in pilocytic astrocytoma with gangliocytic differentiation is likely due to a propensity for involvement of midline structures and poor resectability.


Subject(s)
Astrocytoma/diagnostic imaging , Astrocytoma/pathology , Infratentorial Neoplasms/diagnostic imaging , Infratentorial Neoplasms/pathology , Adolescent , Child , Child, Preschool , Diffusion Magnetic Resonance Imaging/methods , Female , Humans , Infant , Male , Neuroimaging/methods
3.
Neuropathol Appl Neurobiol ; 40(3): 327-36, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24127995

ABSTRACT

AIMS: A few case series in adults have described the characteristics of epithelioid glioblastoma (e-GB), one of the rarest variants of this cancer. We evaluated clinical, radiological, histological and molecular characteristics in the largest series to date of paediatric e-GB. METHODS: Review of clinical characteristics and therapy, imaging studies and histology was performed in patients younger than 22 years with e-GB seen at our institution over 15 years. Sequencing of hotspot mutations and fluorescence in situ hybridization of relevant genes were undertaken. RESULTS: Median age at diagnosis of six patients was 7.6 years. Tumours originated in the cerebral cortex (n = 2) or diencephalon (n = 4). Three patients presented with acute, massive haemorrhage and three had leptomeningeal dissemination at diagnosis. Paediatric e-GB had the typical histological characteristics seen in adult tumours. Universal immunoreactivity for INI1 and lack of diverse protein expression were seen in all cases. One tumour had a chromosome 22q loss. Three tumours (50%) harboured a BRAF: p.V600E. One thalamic tumour had an H3F3A p.K27M. All patients received radiation therapy with (n = 3) or without chemotherapy (n = 3). All patients experienced tumour progression with a median survival of 169 days. One patient with nonmetastatic disease had early leptomeningeal progression. Two patients had symptomatic tumour spread outside the central nervous system (CNS) through a ventriculoperitoneal shunt. One additional patient had widespread metastases outside the CNS identified at autopsy. CONCLUSIONS: Paediatric e-GBs are rare cancers with an aggressive behaviour that share histological and genetic characteristics with their adult counterparts. BRAF inhibition is a potential treatment for these tumours.


Subject(s)
Brain Neoplasms/diagnosis , Glioblastoma/diagnosis , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/genetics , Brain Neoplasms/pathology , Child , Child, Preschool , Female , Glioblastoma/diagnostic imaging , Glioblastoma/genetics , Glioblastoma/pathology , Humans , Male , Radiography
4.
Pediatr Blood Cancer ; 48(5): 555-60, 2007 May.
Article in English | MEDLINE | ID: mdl-16652350

ABSTRACT

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of pathological Langerhans cells, for which the aetiology and pathogenesis remain largely unknown. PROCEDURE: Information on the 101 children with LCH registered with the population-based Manchester Children's Tumour Registry (MCTR) between 1954 and 1998 was extracted from the records of the MCTR. This included age, sex, date of diagnosis, systems affected at diagnosis and follow-up. RESULTS: The overall incidence rate for LCH was 2.6 cases per million child years. In those under 1 year of age the incidence rate was 9.0 cases per million child years, compared to 0.7 cases per million in those aged 10-14 years (P < 0.0001 for age trend). There was no evidence of seasonal variation in presentation by month of birth or first symptom. Bone was the most common site of disease involvement (67% of cases), followed by skin (37%) and soft tissue (22%). The overall survival rate has improved over time, from 57% in 1954-1968 to 74% in 1985-1998. Ninety percent of deaths were due to disease progression, the remainder were due to complications of intensive therapy. The site of LCH lesions and extent of disease present at diagnosis strongly predicted survival outcome. Patients with initial liver involvement had a 5-year survival rate of 25% compared with 93% for those with bone lesions alone at diagnosis. CONCLUSIONS: Incidence rates varied significantly by age at diagnosis, and have been stable over time. Survival has improved considerably over time, but varies strongly by age and systems affected at diagnosis.


Subject(s)
Histiocytosis, Langerhans-Cell/epidemiology , Adolescent , Age Factors , Bone and Bones/pathology , Child , Child, Preschool , Disease Progression , England/epidemiology , Female , Histiocytosis, Langerhans-Cell/mortality , Histiocytosis, Langerhans-Cell/pathology , Humans , Infant , Liver/pathology , Male , Seasons , Skin/pathology , Survival Rate
5.
Crit Care Med ; 28(7): 2248-53, 2000 Jul.
Article in English | MEDLINE | ID: mdl-10921548

ABSTRACT

BACKGROUND: Although cardiac and pulmonary function can be measured precisely, evaluation of tissue perfusion remains elusive because it usually is inferred from subjective symptoms and imprecise signs of shock. The latter are indirect criteria used to assess the overall circulatory status as well as tissue perfusion but are not direct quantitative measures of perfusion. However, noninvasive transcutaneous oxygen (PtcO2) and carbon dioxide (PtcCO2) tensions, which directly measure skin oxygenation and CO2 retention, may be used to objectively evaluate skin oxygenation and perfusion in emergency patients beginning with resuscitation immediately after hospital admission. OBJECTIVE: This study was a preliminary evaluation of tissue oxygenation and perfusion by objective PtcO2 and PtcCO2 patterns in severely injured surviving and nonsurviving patients; specifically, the aim was to describe time patterns that may be used as early warning signs of circulatory dysfunction and death. DESIGN: Prospective descriptive study of a consecutive series of severely injured emergency patients. SETTING: University-affiliated Level I trauma center and intensive care unit. PATIENTS AND METHODS: Forty-eight consecutive severely injured patients were prospectively monitored by PtcO2 and PtcCO2 sensors immediately after emergency admission. RESULTS: Compared with survivors, patients who died had significantly lower PtcO2 and higher PtcCO2 values beginning with the early stage of resuscitation. All patients who maintained PtcO2 >150 torr (19.99 kPa) throughout monitoring survived. Periods of PtcO2 <50 torr (6.66 kPa) for >60 mins or PtcCO2 >60 torr (8.00 kPa) for >30 mins were associated with 90% mortality and 100% morbidity. CONCLUSION: PtcO2 and PtcCO2 monitoring continuously evaluate tissue perfusion and serve as early warning in critically injured patients during resuscitation immediately after hospital admission.


Subject(s)
Blood Gas Monitoring, Transcutaneous , Hypoxia/blood , Shock/blood , Wounds and Injuries/blood , Adult , Emergencies , Female , Glasgow Coma Scale , Hemodynamics , Humans , Injury Severity Score , Male , Prospective Studies , Resuscitation , Trauma Centers , Wounds and Injuries/classification , Wounds and Injuries/mortality , Wounds and Injuries/therapy
6.
J Crit Care ; 15(4): 151-9, 2000 Dec.
Article in English | MEDLINE | ID: mdl-11138876

ABSTRACT

PURPOSE: Traditionally hemodynamic patterns after adult respiratory distress syndrome (ARDS) are described after appropriate diagnostic criteria have been met, but studies begun after the diagnosis of ARDS miss the antecedent circulatory influences that may contribute to its development. This study tests the hypothesis that noninvasive monitoring before the appearance of ARDS may reveal early circulatory deficiencies that lead to this disorder. The aims of this study are as follows: (1) to describe the time course of hemodynamic and tissue perfusion patterns in severely traumatized postoperative patients from the period immediately after admission and during surgical repair to the time that ARDS developed or to hospital discharge in patients who did not develop ARDS, (2) to compare the time course of these patterns in survivors and nonsurvivors of ARDS, and (3) to suggest that reduced flow and perfusion may be early warning signs of ARDS. Prospective descriptive study of severely injured trauma patients noninvasively monitored in the emergency department, operating room, and intensive care unit (ICU). Early hemodynamic pattems were described in the surviving and nonsurviving patients who subsequently developed ARDS. The study was performed in a University-affiliated Level I trauma center and ICU. PATIENTS AND METHODS: A consecutively monitored series of 60 severely injured patients were prospectively monitored by cardiac output, pulse oximetry (Sapo2), and transcutaneous O2 and co2 (Ptco2 and Ptc(co2)) sensors immediately after emergency admission. Twenty-nine patients developed ARDS in their ICU course, whereas 31 were discharged from the ICU and the hospital without developing ARDS. RESULTS: Patients who developed ARDS had significantly lower cardiac index and Ptco2 and higher Ptc(co2) values beginning with the early stage compared with those who did not develop ARDS. Nonsurvivors of ARDS had lower Ptco2 values than did the survivors. CONCLUSION: Early noninvasive monitoring in the emergency department, operating room, and ICU showed reduced cardiac and tissue perfusion functions in patients who subsequently developed ARDS. These patterns were more pronounced in the ARDS patients who died; these patterns may serve as early warning of ARDS.


Subject(s)
Hemodynamics , Monitoring, Physiologic/methods , Postoperative Complications/prevention & control , Respiratory Distress Syndrome/prevention & control , Wounds and Injuries/complications , Adult , Blood Gas Monitoring, Transcutaneous , California/epidemiology , Cardiac Output , Female , Humans , Male , Oximetry , Postoperative Complications/mortality , Prospective Studies , Respiratory Distress Syndrome/etiology , Respiratory Distress Syndrome/mortality , Survival Analysis , Time Factors , Wounds and Injuries/surgery
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