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BACKGROUND : There remain concerns regarding the technical feasibility of endoscopic resection for large gastrointestinal stromal tumors (GISTs), mainly relating to the risk of tumor rupture and the adequacy of the resection margins. This study aimed to evaluate the feasibility and therapeutic outcomes of the newly developed no-touch endoscopic full-thickness resection (NT-EFTR) technique for GISTs. METHODS : In this retrospective study, 92 patients with gastric GISTs undergoing NT-EFTR were included. Clinicopathological, endoscopic, and follow-up data were collected and analyzed. RESULTS : The median tumor size was 2.5âcm and en bloc resection was achieved in all patients with negative surgical margins. The median time of the NT-EFTR procedure was 59.5 minutes. Large tumors (>â3.0âcm), extraluminal tumor growth pattern, and large gastric defects were significant contributors to long operative times. Patients were discharged within 4 days postoperatively. During follow-up, all patients were free from local recurrence and distant metastasis. CONCLUSIONS : NT-EFTR was a feasible method for the resection of gastric GISTs and can be expected to achieve complete radical resection. Large tumors with extraluminal growth and large gastric defects impact procedural difficulty.
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Ressecção Endoscópica de Mucosa , Tumores do Estroma Gastrointestinal , Neoplasias Gástricas , Humanos , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/patologia , Estudos Retrospectivos , Resultado do Tratamento , Neoplasias Gástricas/cirurgia , Neoplasias Gástricas/patologia , Ressecção Endoscópica de Mucosa/métodos , Gastroscopia/métodosRESUMO
BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare encephalopathy characterized by multiple symmetrical brain lesions, mainly involving thalami. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition of unknown cause characterized by fever, sore throat, rash and joint pain. Both entities are considered to be triggered by infections and associated with hypercytokinemia. CASE PRESENTATION: A 46-year-old male was diagnosed with AOSD at local hospital because of 3-week-long high fever, sore throat, arthralgia, transient skin rash, lymphadenopathy, leukocytosis, hyperferritinemia, and absence of antinuclear antibodies (ANA) and rheumatoid factor (RF). Corticosteroids were not used because of delayed diagnosis. Three weeks after the onset, the patient suddenly fell unconscious and was transferred to our hospital. Brain CT and MRI revealed symmetrical lesions involving thalami, striatum and brain stem, consistent with ANE. One day after admission, his condition aggravated and brain CT revealed hemorrhage in the lesions. He died 3 days after admission. CONCLUSION: We report a rare case of ANE preceded by AOSD. The underlying mechanism is still unclear. Early recognizing of the two conditions is difficult but prognostically important.
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Encefalopatias , Faringite , Doença de Still de Início Tardio , Adulto , Artralgia , Encefalopatias/complicações , Febre/etiologia , Humanos , Leucocitose , Masculino , Pessoa de Meia-Idade , Faringite/complicações , Doença de Still de Início Tardio/complicações , Doença de Still de Início Tardio/diagnósticoRESUMO
BACKGROUND: Lymphoplasmacyte-rich meningioma (LPRM) is a rare form of meningioma characterized by prominent lymphoplasmacytic infiltrates into the tumor. Report of flat growth of LPRM mimicking pachymeningitis is rare in the literature. CASE PRESENTATION: A 55-year-old female who suffered from episodes of headache and seizures has been diagnosed with pachymeningitis for 4 years because post contrast brain MRI demonstrated enhanced carpet-like dura lesion in the left frontal lobe. The lesion kept unchanged on yearly follow-ups until a recent brain MRI found the lesion grew significantly into a mass. The lesion was resected and pathology suggested LPRM. CONCLUSION: LPRM may present as carpet-like growth pattern on MRI. Long-term follow-up in patients with pachymeningitis is necessary.
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Neoplasias Meníngeas , Meningioma , Meningite , Dura-Máter/diagnóstico por imagem , Dura-Máter/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Meningioma/diagnóstico por imagem , Meningioma/patologia , Meningite/diagnóstico , Meningite/patologia , Pessoa de Meia-Idade , NeuroimagemRESUMO
INTRODUCTION: Rosette-forming glioneuronal tumor (RGNT) is a rare variety of slow growing mixed glioneuronal tumor involving primarily fourth ventricular region. This is a comprehensive analysis of a 22-year-old woman with RGNT composed of mainly cystic components. In addition, the case showed multiple lesions located in brain parenchyma which mimicked cerebral cysticercosis. Here, we analyzed this case and listed some characteristics of RGNTs in reported literature which occurring in atypical locations for further understanding it. CASE REPORT: A 22-year-old woman presented with a history of transient dizziness, nausea, and vomiting. Magnetic resonance imaging (MRI) showed multiple cystic lesions in brain parenchyma and then the patient was diagnosed with cerebral cysticercosis possibility. Empirical anti-infective therapy in addition to a follow-up post 2 weeks of MRI examination showed the lesions unchanged. Finally, a biopsy of the right cerebellar hemisphere lesions verified RGNT. CONCLUSION: RGNT is an uncommon tumor classified as grade I glioma by World Health Organization (WHO) with slightly longer course. The imaging findings of RGNT are not specific especially in atypical areas. RGNT is rare, but we should also consider the possibility in diagnosis and differential diagnosis.
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Neoplasias do Ventrículo Cerebral , Glioma , Neurocisticercose , Feminino , Quarto Ventrículo , Humanos , Imageamento por Ressonância Magnética , Neurocisticercose/diagnóstico por imagem , Adulto JovemRESUMO
AIM: This study is to compare the lung image quality between shelter hospital CT (CT Ark) and ordinary CT scans (Brilliance 64) scans. METHODS: The patients who received scans with CT Ark or Brilliance 64 CT were enrolled. Their lung images were divided into two groups according to the scanner. The objective evaluation methods of signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were used. The subjective evaluation methods including the evaluation of the fine structure under the lung window and the evaluation of the general structure under the mediastinum window were compared. Kappa method was used to assess the reliability of the subjective evaluation. The subjective evaluation results were analyzed using the Wilcoxon rank sum test. SNR and CNR were tested using independent sample t tests. RESULTS: There was no statistical difference in somatotype of enrolled subjects. The Kappa value between the two observers was between 0.68 and 0.81, indicating good consistency. For subjective evaluation results, the rank sum test P value of fine structure evaluation and general structure evaluation by the two observers was ≥ 0.05. For objective evaluation results, SNR and CNR between the two CT scanners were significantly different (P<0.05). Notably, the absolute values ââof SNR and CNR of the CT Ark were larger than Brilliance 64 CT scanner. CONCLUSION: CT Ark is fully capable of scanning the lungs of the COVID-19 patients during the epidemic in the shelter hospital.
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COVID-19/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Unidades Móveis de Saúde/normas , Tomografia Computadorizada por Raios X/instrumentação , Tomografia Computadorizada por Raios X/normas , Adulto , Idoso , COVID-19/epidemiologia , China/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Pandemias , SARS-CoV-2 , Razão Sinal-RuídoRESUMO
BACKGROUND: Papillary glioneuronal tumor (PGNT) is a rare, recently described distinct low-grade brain neoplasm. This study was performed to characterize the clinicopathologic and neuroradiologic features of PGNTs. METHODS: We reviewed the medical records of 16 patients with PGNT who underwent surgery, including 11 males and five females (median age 27 years). The clinical, neuroradiologic, histopathologic, and immunohistochemical findings were documented. RESULTS: Headache was the principal presentation. Neuroimaging showed contrast-enhancing, cystic-solid or cystic masses with a mural nodule, mostly involved the frontal or parietal lobes. Histologically, the tumors were characterized by glial fibrillary acidic protein (GFAP)-positive small cuboidal cells lining hyalinized vascular pseudopapillae and synaptophysin and/or NeuN-positive interpapillary neuronal elements. Other findings included small angiomatous areas in ten, small islands of neuropil and rosettes in seven, and microvascular proliferation and/or nuclear atypia in six. Mitoses or necrosis were absent. All lacked isocitrate dehydrogenase 1 (IDH1) R132H protein expression. Low expression of p53 was observed in three cases. Ki67 labeling index ranged from less than 1 to 3 %. All but one was totally resected. Median follow-up was 65 months, and one patient had tumor recurrence. CONCLUSIONS: PGNTs display distinct clinicopathologic and imaging characteristics and indicate a favorable prognosis. However, recurrences sometimes occur. Immunohistochemistry facilitates the appropriate diagnosis of these tumors. Complete resection of the tumor is important for a favorable outcome.
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Neoplasias Encefálicas/diagnóstico por imagem , Imageamento por Ressonância Magnética , Neoplasias Neuroepiteliomatosas/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Adolescente , Adulto , Neoplasias Encefálicas/patologia , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Neuroepiteliomatosas/patologia , Oligodendroglia/patologiaRESUMO
BACKGROUND: To establish a prospective cohort to enumerate the prevalence, incidence and risk factors for dementia and mild cognitive impairment (MCI) among residents aged ≥60 in an urban community of Shanghai, China. METHODS: Participants received clinical evaluations including physical measurements, demographic and lifestyle questionnaires, physical and neurologic examinations, and neuropsychological testing. Urine and blood samples were collected, aliquoted, and stored. DNA was extracted for Apolipoprotein (APOE) genotyping. Diagnoses of dementia and MCI were made using standard criteria via consensus diagnosis. RESULTS: Among 3,141 participants aged ≥60, 1,438 (45.8%) were men. The average age of participants was 72.3 years (SD 8.1), and they had an average of 11.6 years (SD 4.4) of education. The most common chronic disease of participants was hypertension (56.4%). The frequencies of APOE-âε2, ε3 and ε4 were 7.9, 82.7 and 9.4%, respectively. We diagnosed 156 (5.0%, 95% CI 4.3-5.8%) participants with dementia. The prevalence rates of Alzheimer's disease and vascular dementia were 3.6% (95% CI 3.0-4.3%) and 0.8% (95% CI 0.5-1.1%). CONCLUSIONS: The Shanghai Aging Study is the first prospective community-based cohort study of cognitive impairment in China, with a comparable study design, procedures, and diagnostic criteria for dementia and MCI to most previous cohort studies in developed countries.
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Envelhecimento , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/epidemiologia , Demência/diagnóstico , Demência/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Apolipoproteína E4/genética , China/epidemiologia , Disfunção Cognitiva/genética , Demência/genética , Projetos de Pesquisa Epidemiológica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , População UrbanaRESUMO
Giant cell tumors (GCTs) are generally benign, locally aggressive lesions mostly located in the metaphysis of long bones. GCTs of the skull are rare and the majority of the cases have been presented as case reports. The authors retrospectively reported 18 patients with GCTs of the skull at a single institution from April 1994 to February 2012 and summarized the clinical, radiological, pathological characteristics and management of the disease. Meanwhile, a systematic review of 94 case reports of GCTs of the skull was performed. Headache and symptoms related to the involvement of intracranial nerves were the most common symptoms. Over 90 % of the tumors originated from sphenoid and temporal bones. On MRI, very low signal on T2-weighted images were found highly indicative of GCTs of the temporal bone. Univariate analysis revealed that extent of tumor resection and post-operative radiation therapy (RT) were prognostic factors significantly influencing the survival of the patients. We concluded that complete tumor resection is the optimal goal in treating this disease and adjuvant RT should be given once tumor residual is inevitable.
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Tumores de Células Gigantes/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias Cranianas/patologia , Adolescente , Adulto , Terapia Combinada , Feminino , Seguimentos , Tumores de Células Gigantes/mortalidade , Tumores de Células Gigantes/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/terapia , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Literatura de Revisão como Assunto , Neoplasias Cranianas/mortalidade , Neoplasias Cranianas/terapia , Taxa de Sobrevida , Adulto JovemRESUMO
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a common disease with high morbidity, with acute exacerbations manifesting as a worsening of respiratory symptoms. This study aimed to identify the frequent acute exacerbation phenotype in patients with COPD based on imaging and clinical characteristics. METHODS: Patients with COPD (n = 201) were monitored for acute exacerbations one year after their initial hospital admission and further divided into frequent and non-frequent exacerbation groups according to the frequency and severity of acute exacerbations. All patients underwent high resolution CT scans and low attenuation area less than -950Hu (LAA-950) in the whole lung was measured. Differences in visual subtypes, LAA-950, and clinical basic characteristics were compared between groups. The clinical factors influencing frequent exacerbation were determined using binary logistic regression. Finally, based on imaging and clinical factors, the receiver operating characteristic curve was used to identify the phenotype of COPD with frequent acute exacerbations. RESULTS: Patients with frequent exacerbations had a larger LAA-950 than those non-frequent exacerbations patients (pï¼0.001). Frequent acute exacerbations were associated with worsening visual subtypes. Multivariate binary logistic regression illustrated that age, smoking status, BMI, FEV1 pred, and LAA-950 were associated with frequent exacerbations of COPD. The area under the receiver operating characteristic curve for predicting frequent exacerbations based on age, smoking status, BMI, FEV1 pred, and LAA-950 was 0.907 (pï¼0.001). CONCLUSION: The combination of imaging and clinical characteristics reached high diagnostic efficacy in the identification of frequent acute exacerbations in patients with COPD.
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Doença Pulmonar Obstrutiva Crônica , Humanos , Volume Expiratório Forçado , Progressão da Doença , Doença Pulmonar Obstrutiva Crônica/complicações , Doença Pulmonar Obstrutiva Crônica/diagnóstico por imagem , Pulmão/diagnóstico por imagem , FenótipoRESUMO
BACKGROUND: Lung is the most common primary site of brain metastases (BMs). For different pathological types of BMs have some similar characteristics, it is still a challenge to confirm the origin based on their characteristics directly. BMs of small cell lung cancer (SCLC) have favorable therapeutic expectations due to their high sensitivity to radiotherapy. This study sought to identify unique characteristics of BMs in SCLC, aiming to assist in clinical decision-making. METHODS: Patients diagnosed with BMs of lung cancer who received radiotherapy from January 2017 to January 2022 were reviewed (N = 284). Definitive diagnosis of BMs of SCLC was reached for 36 patients. All patients underwent head examination using magnetic resonance imaging. The number, size, location, and signal characteristics of lesions were analyzed. RESULTS: There were 7 and 29 patients with single focus and non-single focus, respectively. Ten patients had diffuse lesions, and the remaining 26 patients had a total of 90 lesions. These lesions were divided into three groups according to size: <1, 1-3, and >3 cm (43.33%, 53.34%, and 3.33%, respectively). Sixty-six lesions were located in the supratentorial area, primarily including cortical and subcortical lesions (55.56%) and deep brain lesions (20%). Moreover, 22 lesions were located in the infratentorial area. According to diffusion-weighted imaging and T1-weighted contrast enhancement, the imaging characteristics were classified into six patterns. Hyperintensity in diffusion-weighted imaging and homogeneous enhancement was the most common pattern of BMs in SCLC (46.67%), while partial lesions showed hyperintensity in diffusion-weighted imaging without enhancement (7.78%). CONCLUSIONS: The manifestations of BMs in SCLC were multiple lesions (diameter: 1-3 cm), hyperintensity in diffusion-weighted imaging, and homogeneous enhancement. Interestingly, hyperintensity in diffusion-weighted imaging without enhancement was also one of the characteristics.
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Neoplasias Encefálicas , Neoplasias Pulmonares , Carcinoma de Pequenas Células do Pulmão , Humanos , Carcinoma de Pequenas Células do Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Imagem de Difusão por Ressonância Magnética/métodos , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/secundário , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Estudos RetrospectivosRESUMO
Rationale: Clinical application of mesenchymal stem cells (MSCs) and MSC-derived exosomes (MSC-Exos) to alleviate myocardial ischemia/reperfusion (I/R) injury is compromised by the low cell engraftment rate and uncontrolled exosomal content. As one of their active ingredients, single-component microRNA therapy may have more inherent advantages. We sought to find an ideal microRNA candidate and determine whether it could reproduce the cardioprotective effects of MSCs and MSC-Exos. Methods: Cardiac function and myocardial remodeling in MSC, MSC-Exo, or microRNA oligonucleotide-treated mouse hearts were investigated after I/R injury. The effects of microRNA oligonucleotides on cardiac cells (macrophages, cardiomyocytes, fibroblasts, and endothelial cells) and their downstream mechanisms were confirmed. Large animals were also employed to investigate the safety of microRNA therapy. Results: The results showed that microRNA-125a-5p (miR-125a-5p) is enriched in MSC-Exos, and intramyocardial delivery of their modified oligonucleotides (agomir) in mouse I/R myocardium, as well as MSCs or MSC-Exos, exerted obvious cardioprotection by increasing cardiac function and limiting adverse remodeling. In addition, miR-125a-5p agomir treatment increased M2 macrophage polarization, promoted angiogenesis, and attenuated fibroblast proliferation and activation, which subsequently contributed to the improvements in cardiomyocyte apoptosis and inflammation. Mechanistically, Klf13, Tgfbr1, and Daam1 are considered the targets of miR-125a-5p for regulating the function of macrophages, fibroblasts, and endothelial cells, respectively. Similar results were observed following miR-125a-5p agomir treatment in a porcine model, with no increase in the risk of arrhythmia or hepatic, renal, or cardiac toxicity. Conclusions: This targeted microRNA delivery presents an effective and safe strategy as a stem cell and exosomal therapy in I/R cardiac repair.
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Exossomos , MicroRNAs , Traumatismo por Reperfusão Miocárdica , Animais , Camundongos , Células Endoteliais , Exossomos/genética , Proteínas dos Microfilamentos , MicroRNAs/administração & dosagem , MicroRNAs/uso terapêutico , Traumatismo por Reperfusão Miocárdica/terapia , Traumatismo por Reperfusão Miocárdica/prevenção & controle , Miocárdio , Miócitos Cardíacos , Proteínas rho de Ligação ao GTP , SuínosRESUMO
Background: Cerebrospinal fluid oligoclonal band (CSF-OCB) is an established biomarker in diagnosing multiple sclerosis (MS), however, there are no nationwide data on CSF-OCB prevalence and its diagnostic performance in Chinese MS patients, especially in the virtue of common standard operation procedure (SOP). Methods: With a consensus SOP and the same isoelectric focusing system, we conducted a nationwide multi-center study on OCB status in consecutively, and recruited 483 MS patients and 880 non-MS patients, including neuro-inflammatory diseases (NID, n = 595) and non-inflammatory neurological diseases (NIND, n=285). Using a standardized case report form (CRF) to collect the clinical, radiological, immunological, and CSF data, we explored the association of CSF-OCB positivity with patient characters and the diagnostic performance of CSF-OCB in Chinese MS patients. Prospective source data collection, and retrospective data acquisition and statistical data analysis were used. Findings: 369 (76.4%) MS patients were OCB-positive, while 109 NID patients (18.3%) and 6 NIND patients (2.1%) were OCB-positive, respectively. Time from symptom onset to diagnosis was significantly shorter in OCB-positive than that in OCB-negative MS patients (13.2 vs 23.7 months, P=0.020). The prevalence of CSF-OCB in Chinese MS patients was significantly higher in high-latitude regions (41°-50°N)(P=0.016), and at high altitudes (>1000m)(P=0.025). The diagnostic performance of CSF-OCB differentiating MS from non-MS patients yielded a sensitivity of 76%, a specificity of 87%. Interpretation: The nationwide prevalence of CSF-OCB was 76.4% in Chinese MS patients, and demonstrated a good diagnostic performance in differentiating MS from other CNS diseases. The CSF-OCB prevalence showed a correlation with high latitude and altitude in Chinese MS patients.
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Esclerose Múltipla , Humanos , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/epidemiologia , Bandas Oligoclonais/líquido cefalorraquidiano , Estudos Retrospectivos , Estudos Prospectivos , Prevalência , População do Leste AsiáticoRESUMO
Background: We aimed to investigate perineal nerve block versus periprostatic block in pain control for men undergoing a transperineal prostate biopsy. Methods: In this prospective, randomised, blinded and parallel-group trial, men in six Chinese hospitals with suspected prostate cancer were randomly assigned (1:1) at the point of local anaesthesia to receive a perineal nerve block or periprostatic block and followed by a transperineal prostate biopsy. Centres used their usual biopsy procedure. Operators who performed anaesthesia were trained in both techniques before the trial and were masked to the randomised allocation until the time of anaesthesia and were not involved in the subsequent biopsy procedure and any assessment or analysis. Other investigators and the patients were masked until trial completion. The primary outcome was the level of the worst pain experienced during the prostate biopsy procedure. Secondary outcomes included pain (post-biopsy at 1, 6 and 24 h), changes in blood pressure, heart rate and breathing rate during the biopsy procedure, external manifestations of pain during biopsy, anaesthesia satisfaction, the detection rate of PCa and clinically significant PCa. This trial is registered on ClinicalTrials.gov, NCT04501055. Findings: Between August 13, 2020, and July 20, 2022, 192 men were randomly assigned to perineal nerve block or periprostatic block, 96 per study group. Perineal nerve block was superior for the relief of pain during the biopsy procedure (mean 2.80 for perineal nerve block and 3.98 for periprostatic block; adjusted difference in means -1.17, P < 0.001). Although the perineal nerve block had a lower mean pain score at 1 h post-biopsy compared with the periprostatic block (0.23 vs 0.43, P = 0.042), they were equivalent at 6 h (0.16 vs 0.25, P = 0.389) and 24 h (0.10 vs 0.26, P = 0.184) respectively. For the change in vital signs during biopsy procedure, perineal nerve block was significantly superior to periprostatic block in terms of maximum value of systolic blood pressure, maximum value of mean arterial pressure and maximum value of heart rate. There are no statistical differences in average value of systolic blood pressure, average value of mean, average value of heart rate, diastolic blood pressure and breathing rate. Perineal nerve block was also superior to periprostatic block in external manifestations of pain (1.88 vs 3.00, P < 0.001) and anaesthesia satisfaction (8.93 vs 11.90, P < 0.001). Equivalence was shown for the detection rate of PCa (31.25% for perineal nerve block and 29.17% for periprostatic block, P = 0.753) or csPCa (23.96% for perineal nerve block and 20.83% for periprostatic block, P = 0.604). 33 (34.8%) of 96 patients in the perineal nerve block group and 40 (41.67%) of 96 patients in the periprostatic block group had at least one complication. Interpretation: Perineal nerve block was superior to periprostatic block in pain control for men undergoing a transperineal prostate biopsy. Funding: Grant 2019YFC0119100 from the National Key Research and Development Program of China.
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Dysembryoplastic neuroepithelial tumor (DNT)-like neoplasms of the septum pellucidum are extremely rare. We reviewed 2 cases of DNT-like neoplasm of the septum pellucidum and specifically studied the immunohistochemical features and chromosomes 1p and 19q deletions. One case was a 26-year-old woman who complained of aggravated headache for 2 weeks. The other case was a 31-year-old female presenting with double vision for a month. Histological examinations showed that the lesions were composed of uniform oligodendrocytelike cells (OLCs) with obvious floating neurons in a mucin-rich background. Immunohistochemical studies revealed that both tumors were diffusely positive for Synaptophysin and Olig2. Sporadic neurons were clearly positive for NeuN. Loss of chromosome 1p/19q and isocitrate dehydrogenase 1(IDH1) mutations were not identified in both cases. It might indicate that these OLCs of DNT-like neoplasms were genetically different from glial tumors, although they showed close morphological similarities.
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Neoplasias Encefálicas/patologia , Cromossomos Humanos Par 19/genética , Cromossomos Humanos Par 1/genética , Isocitrato Desidrogenase/genética , Neoplasias Neuroepiteliomatosas/patologia , Septo Pelúcido/patologia , Adulto , Neoplasias Encefálicas/genética , Feminino , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Mutação , Neoplasias Neuroepiteliomatosas/genéticaRESUMO
In the original description, rosette-forming glioneuronal tumors (RGNTs) were restricted to the fourth ventricle and/or posterior fossa. Here, we first report an unusual case of RGNT centered in the septum pellucidum and associated with multiple masses occupying the wall of the bilateral lateral ventricles and the third ventricle. No mass was found in the fourth ventricle. Histological and immunohistochemical examination revealed that the tumor presented biphasic differentiation characterized by predominantly neurocytic rosettes and pilocytic astrocytoma-like components with obvious microvascular proliferation. Chromosome 1p/19q deletions and isocitrate dehydrogenase 1 and 2 (IDH1/2) mutations were not identified. Because this case exhibited a worrisome growth pattern, further studies and long-term follow-up are needed to determine the true nature of these tumors.
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Neoplasias do Ventrículo Cerebral/patologia , Glioma/patologia , Septo Pelúcido/patologia , Adulto , Neoplasias do Ventrículo Cerebral/genética , Neoplasias do Ventrículo Cerebral/cirurgia , Glioma/genética , Glioma/cirurgia , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Isocitrato Desidrogenase/metabolismo , Isoenzimas/genética , Isoenzimas/metabolismo , Ventrículos Laterais/patologia , Imageamento por Ressonância Magnética , Masculino , Inclusão em Parafina , Formação de Roseta , Septo Pelúcido/cirurgia , Terceiro Ventrículo/patologia , Tomografia Computadorizada por Raios XRESUMO
Orbital schwannomas are rare in children, especially those with intracranial extension. Herein, our report refers to a 12-year-old boy who had a cranial-orbital mass with a dumbbell-like appearance. The total neoplasms was successfully removed via a transcranial approach, and the pathological diagnostic result was schwannoma. Neither radiotherapy nor chemotherapy was performed after surgery, and no recurrences were observed for 3 months. Our report suggests that orbital schwannomas should be differentiated from other types of orbital tumors with sufficient evidence and that complete surgical resection remains the first choice to cure this disease.
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BACKGROUND AND OBJECTIVES: To characterize the clinical and neuroimaging phenotypes of patients with autoantibodies to γ-aminobutyric acid type A receptor (GABAAR). METHODS: Ten patients with autoantibodies against GABAAR from Huashan Hospital Autoimmune Encephalitis cohort were identified. We used MRI assessments and clinical examinations to summarize major clinical profile and visualize and quantify lesion distribution features. The relationship between clinical features, neuroimaging phenotypes, and topology of GABAAR expression were further investigated. RESULTS: The median age at onset of 10 patients (8 male patients and 2 female patients) with anti-GABAAR encephalitis was 41.5 years (range: 17-73 years). All patients had prominent seizures and multifocal spotted or confluent lesions involved in limbic, frontal, and temporal lobes on brain MRI. Bilateral but asymmetric lesions in cingulate gyri were observed in all patients. These involved lesions could change dynamically with immunotherapies and relapse. Distribution of patients' brain MRI lesions was positively correlated with gene expression level of ß3 subunit-containing GABAAR (Spearman ρ = 0.864, p = 0.001), the main target of autoantibodies. According to topology of lesions, patients with anti-GABAAR encephalitis could be classified into 2 clinical-radiological types: confluent type with bilateral confluent lesions involved in almost all limbic, frontal, and temporal lobes and spotted type with multiple scattered small-to-medium patchy lesions. Patients with confluent type exhibited worse clinical presentations and outcomes when compared with those with spotted type (maximum modified Rankin scale [mRS]: 5 [5-5] vs 3.5 [3-4], respectively, p = 0.008; follow-up mRS: 4 [2-6] vs 0.5 [0-1], respectively, p = 0.016). DISCUSSION: Anti-GABAAR encephalitis has distinctive neuroimaging phenotype. Cingulate gyri were frequently involved in this disorder. The topology of lesions might be associated with the distribution of ß3 subunit-containing GABAAR and reflected patients' disease severity and outcomes.
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Encefalite , Doença de Hashimoto , Receptores de GABA-A , Adolescente , Adulto , Idoso , Autoanticorpos , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Ácido gama-AminobutíricoRESUMO
A 16-year-old male teenager presented with seizure and loss of consciousness for 20 min. Magnetic resonance imaging demonstrated a mass occupying the right medial temporal lobe. Histological examination revealed a non-pigmented area with spindle-shaped and large xanthomatous pleomorphic cells and a pigmented region with pigmented neoplastic cells with fascicular arrangement. Immunohistochemical studies showed the tumor was positive for GFAP and low index of Ki-67. Considering the patient's history, clinical data and pathological findings, we rendered a rare variant named pigmented pleomorphic xanthoastrocytoma.