Diffuse Enhancement Research Articles

Accuracy of diagnostic score by echocardiography for the detection of cardiac amyloidosis in patients with proven extracardiac amyloidosis

Abstract Background In 2021, a position statement of the ESC Working Group on Myocardial and Pericardial Diseases suggested to use an echocardiographic score (i.e., ECHO score) to confirm cardiac amyloidosis (CA) thus to avoid heart biopsy in patients with left ventricular (LV) hypertrophy >12 mm and proven extracardiac amyloidosis. Purpose To test the accuracy of ECHO-score in a broad spectrum of patients with proven cardiac amyloidosis and anatomical LV hypertrophy measured by echocardiography. Methods One hundred and twenty patients with proven CA (both from ATTR and AL forms) underwent comprehensive echocardiography including parietal wall thickness (+3 points), Doppler E/e' ratio (+1 point), TAPSE (+2 points), LV global longitudinal strain (1 point) and systolic longitudinal strain apex to base ratio (+3 points) and compared to 13 controls. A score ≥8 points in the presence of LV wall >12 mm which predicts CA, was investigated in patients with LV hypertrophy from 12.1 to 15.0 mm, from 15.1 to 20 mm and over 20 mm. Cardiovascular magnetic resonance (CMR) imaging was available in 63 patients for extracellular volume measurement >0.40% and diffuse late gadolinium enhancement assessment, both present to confirmed amyloidosis. Results ATTR and AL amyloidosis accounted for 77% and 23%, respectively. Age averaged 76±7yo and 72% of patients were male. LV ejection fraction averaged 61±5%. The ECHO score averaged 6.4±2.5 in patients with LV hypertrophy from 12.1 to 15.0 mm (n=29), 7.9±2.6 in patients with LV hypertrophy from 15.1 to 20.0 mm (n=59) and 9.8±1.7 when LV hypertrophy was >20 mm (n=32). Figure 1 depicts the sensitivity of ECHO score alone and in addition to CMR as a function of LV hypertrophy range. In patients with LV hypertrophy >20 mm, ECHO score alone has a sensitivity near 100% and CMR is useless to confirm cardiac amyloidosis. Between 15.1 to 20.0 mm of hypertrophy, both ECHO and CMR are necessary to reach a sensitivity of 100%. Unfortunately, below 15 mm of LV hypertrophy, neither ECHO nor CMR altogether predict cardiac amyloidosis with good accuracy, meaning heart biopsy is still useful. Conclusion Upon the degree of LV hypertrophy in a patient with extrcardiac amyloidosis, ECHO score alone or in combination with MRI are efficient only if myocardial parietal thickness is above 15 mm. Funding Acknowledgement Type of funding sources: None.

DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR: A RARE CAUSE OF MALIGNANT PLEURAL EFFUSION

DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR: A RARE CAUSE OF MALIGNANT PLEURAL EFFUSION

Dimits transition in three-dimensional ion-temperature-gradient turbulence

We extend our previous work on the two-dimensional (2-D) Dimits transition in ion-scale turbulence (Ivanov et al., J. Plasma Phys., vol. 86, 2020, 855860502) to include variations along the magnetic field. We consider a three-field fluid model for the perturbations of electrostatic potential, ion temperature, and ion parallel flow in a constant-magnetic-curvature geometry without magnetic shear. It is derived in the cold-ion, long-wavelength asymptotic limit of the gyrokinetic theory. Just as in the 2-D model, a low-transport (Dimits) regime exists and is found to be dominated by a quasistatic staircase-like arrangement of strong zonal flows and zonal temperature. This zonal staircase is formed and maintained by a negative turbulent viscosity for the zonal flows. Unlike the 2-D model, the three-dimensional (3-D) one does not suffer from an unphysical blow up beyond the Dimits threshold where the staircase becomes nonlinearly unstable. Instead, a well-defined finite-amplitude saturated state is established. This qualitative difference between the 2-D and 3-D models is due to the appearance of small-scale ‘parasitic’ modes that exist only if we allow perturbations to vary along the magnetic field lines. These modes extract energy from the large-scale perturbations and provide an effective enhancement of large-scale thermal diffusion, thus aiding the energy transfer from large injection scales to small dissipative ones. We show that in our model, the parasitic modes always favour a zonal-flow-dominated state. In fact, a Dimits state with a zonal staircase is achieved regardless of the strength of the linear drive, provided the system is sufficiently extended along the magnetic field and sufficient parallel resolution is provided.

BILATERAL PULMONARY EMBOLISM MASQUERADING AS MEDIASTINAL LYMPHADENOPATHY DIAGNOSED WITH ENDOBRONCHIAL ULTRASOUND

BILATERAL PULMONARY EMBOLISM MASQUERADING AS MEDIASTINAL LYMPHADENOPATHY DIAGNOSED WITH ENDOBRONCHIAL ULTRASOUND

Mechanistic study of the solid-liquid extraction of phenolics from walnut pellicle fibers enhanced by ultrasound, microwave and mechanical agitation forces

The molecular diffusion of phenolics inside walnut pellicle fiber particles under solid-liquid extraction enhanced by ultrasound (US), orbital agitation (OA), impeller agitation (IA), and the combined microwave and impeller agitation (MW-IA) were explored. Numerical modeling considering the temperature-dependent diffusivity revealed that the internal diffusivity of phenolics was the highest under MW-IA and the lowest under OA. At 35 °C, IA (126.246 mg/g, t = 10 min) was more effective to strengthen the phenolic diffusivity than the US-39W (95.538 mg/g, t = 10 min). Due to the simultaneous enhancement of internal diffusivity and external dissolution, the MW-IA extraction reached equilibrium within 16 min, reaching the highest yield among all the treatments (176.944 mg/g). The extraction was then divided into the increasing and falling driving force periods. The comparison of phenolic diffusivity among MW-IA and IA roughly indicated that the proportion of non-thermal effect of microwave at 315 W was 889% higher than 189 W at the extraction equilibrium. Moreover, some soluble polyphenols, i.e. quercitrin and syringic acid, could be adsorbed by the cell wall after equilibrium. The correlations between any two soluble phenolics varied with the phenolic type, deducing that soluble phenolics may interact with each other either positively or negatively. Besides, the mining of phenolic data also indicated that intensive impeller agitation is a good substitution for ultrasonication to extract phenolics effectively.

Lumbar cistern Candida intradural abscess following epidural anesthesia

This report describes a case of an immunocompetent patient with an intradural abscess from Candida dubliniensis. The majority of fungal spine infections, although rare in general, are due to Aspergillus or C. albicans through systemic fungemia. To date, there have only been two reports of spondylodiscitis from C. dubliniensis. A 37-year-old immunocompetent female patient presented to the neurosurgical service for worsening headaches with nausea, vomiting, vision changes, and weight loss. MRI studies showed diffuse leptomeningeal enhancement of the distal spinal cord, conus medullaris, and nerve roots of the cauda equina extending beyond the neural foramina bilaterally. She had persistent symptoms and no clear diagnosis on lumbar puncture or systemic testing therefore L5-S1 laminectomy for an intradural tissue biopsy was performed. During surgery, cultures were taken and grew colonies of C. dubliniensis. This organism has been reported rarely in the literature as being an infectious agent, thus diagnosing remains a challenge but should be considered in patients with a suggestive history.

Too much of a good thing: Immune reconstitution inflammatory syndrome in a patient with Still's disease

Immune Reconstitution Inflammatory Syndrome (IRIS) is a potential complication when treating non HIV immunosuppressed patients with opportunistic infections. We present a case of a 49-year-old female with Adult-onset Still's disease on prednisone 40 mg daily who came to ED with right leg weakness and intractable headache for one week. She was diagnosed with Cryptococcus meningitis. Patient completed the induction phase of antifungal therapy and the steroids were tapered over four weeks. One month after discharge, a patient was brought in to ED, minimally responsive to verbal stimuli and had new left hemiparesis with persistent right leg weakness was noted on exam. An MRI of the brain was consistent with diffuse leptomeningeal enhancement compatible with meningoencephalitis. LP was notable for elevated opening pressure of 36cmH2O and CSF studies were negative for recurrence of cryptococcal infection. Given the timeline of patients presentation one month after discontinuation of steroids, and workup consistent with sterile meningitis, immune reconstitution inflammatory syndrome was identified as the likely diagnosis. The patient was started on 50 mg of Prednisone daily. Six weeks after presentation, the patient's mental status returned to baseline, left hemiparesis resolved, and right lower extremity strength significantly improved. Clinicians should have a high index of suspicion for CNS IRIS in patients presenting with new neurologic findings in the setting of rapid discontinuation of steroids due to infection. IRIS in HIV patients with cryptococcal meningitis is a well-established entity; the purpose of this case report is to bring attention to similar inflammatory syndrome in non-HIV patients with cryptococcal meningitis.

Trion-phonon interaction in atomically thin semiconductors

Optical and transport properties of doped monolayer semiconductors are dominated by trions, which are three-particle compounds formed by two electrons and one hole or vice versa. In this work, we investigate the trion-phonon interaction on a microscopic footing and apply our model to the exemplary case of a molybdenum diselenide (MoSe2) monolayer. We determine the trion series of states and their internal quantum structure by solving the trion Schr\"odinger equation. Transforming the system into a trion basis and solving equations of motion, including the trion-phonon interaction within the second-order Born-Markov approximation, provides a microscopic access to the trion dynamics. In particular, we investigate trion propagation and compute the diffusion coefficient and mobility. In the low density limit, we find that trions propagate less efficiently than excitons and electrons due to their stronger coupling with phonons and their larger mass. For increasing densities, we predict a drastic enhancement of diffusion caused by the build-up of a large pressure by the degenerate trion gas, which is a direct consequence of the fermionic character of trions. Our work provides microscopic insights into the trion-phonon interaction and its impact on the diffusion behaviour in atomically thin semiconductors.

Imaging features of juvenile xanthogranuloma.

Juvenile xanthogranuloma is rare in children and there are limited data on its imaging features. To analyze the computed tomography (CT) and magnetic resonance imaging (MRI) features of juvenile xanthogranuloma in children. A retrospective review was performed of clinical and radiographic data of histologically confirmed juvenile xanthogranuloma between January 2009 and June 2020. Fourteen children (4 girls, 10 boys; age range: 1day to 13years, mean age: 73months) were included in thestudy: 4/14 had CT only, 5/14 had MRI only and 5/14 had CT and MRI. Sites of extracutaneous juvenile xanthogranuloma involvement included subcutaneous soft tissue (8/14), liver (2/14), lungs (2/14), kidney (2/14), nose (2/14), pancreas (1/14), central nervous system (1/14) and greater omentum (1/14), mainly manifested as single or multiple nodules or masses in different organs. On CT, the lesions mainly manifested as an iso-hypo density mass with mild or marked enhancement. On MRI, the lesions mainly manifested as slightly hyperintense on T1 and slightly hypointense on T2, with decreased diffusivity and homogeneous enhancement. Juvenile xanthogranuloma was not included in the imaging differential diagnosis in any case. Juvenile xanthogranuloma mainly manifests as single or multiple nodules or masses in different organs. Slight hyperintensity on T1 and slight hypointensity on T2 with decreased diffusivity and homogeneous enhancement are relatively characteristic imaging findings of juvenile xanthogranuloma. Combined with its typical skin lesions and imaging features, radiologists should include juvenile xanthogranuloma in the differential diagnosis when confronted with similar cases.

Impact of the coagulation of dust particles on Mars during the 2018 global dust storm

Coagulation of particles occurs when two particles collide and stick together. In the Martian atmosphere, coagulation of dust would increase the dust effective particle size, as small particles accrete to larger particles. Murphy et al. (1990) concluded that Brownian coagulation of dust in the Martian atmosphere was not significant, due to the low dust particle mixing ratios, while Montmessin et al. (2002) and Fedorova et al. (2014) showed that it mostly involves dust particle radii smaller than 0.1 μm. However, the effects of coagulation have never been explored in 3D, during a global dust storm, and in presence of larger numbers of small particles. Here we revisit this issue by using the NASA Ames Mars Global Climate Model (MGCM) to investigate the temporal and spatial changes in dust particle sizes during the 2018 global dust storm due to dust coagulation and the overall impact of these processes on Mars' climate. Our parameterization for dust coagulation includes the effect of Brownian motion, Brownian diffusion enhancement, and gravitational collection. We show that Brownian motion and Brownian diffusion enhancement dominate gravitational collection. Coagulation has a significant impact during the global storm, with coagulation rates increased by a factor of 10 compared to non-storm conditions. The mean effective particle radius can be increased by a factor of up to 2 due to coagulation, leading to a 20 K colder atmosphere above 30 km altitude. Overall, our parameterization improves the representation of the decay phase of the storm relative to MCS dust observations. Coagulation also remains a significant process affecting dust outside the storm period if large numbers of submicron-sized particles are involved. As coagulation removes the small sub-micron particles within a relatively short time, it may therefore be possible, in GCMs, to lift larger amounts of submicron-sized particles from the surface without excess dust buildup in the atmosphere, thus improving the agreement with some of the observations without diverging from the observed column opacities.

Falling film on an anisotropic porous medium

The stability and dynamics of a falling liquid film over an anisotropic porous medium are studied using a one-domain approach. Our stability analysis shows a significant departure from the effective no-slip boundary condition in the isotropic case. Anisotropy does not affect the threshold of linear instability. However, a non-trivial dual effect of anisotropy on the film stability is observed depending on the permeability of the porous medium. This dual effect results from the net balance of the enhancement of viscous diffusion at the top Brinkman sublayer and the mitigation of viscous damping in the core Darcy sublayer. Three-equation models have been derived from the lubrication theory approximation in terms of the exact mass balance and averaged momentum balances in the porous and liquid layers. In the nonlinear regime, anisotropy has a dual effect by damping capillary waves at large permeabilities and enhancing them at low permeabilities. Anisotropy also affects wave speeds and shapes, modifies travelling-wave branches of solutions, affects the development of a time-periodic wavetrain by inlet forcing and alters the noise-driven dynamics of the flow. These effects result from the mitigation of mass exchange at the liquid–porous interface and the contribution of the cross-stream permeability in the Brinkman top sublayer to the viscous diffusion.

Diatom frustules based dissolved oxygen sensor with superhydrophobic surface

Dissolved oxygen (DO) sensors for the practical application usually require good resistance to complex and extreme environments, such as acidic or alkaline corrosion, seawater sediment erosion, protein or bacteria adhesion, etc. Diatom frustules (DFs) are protective shell structures extracted from diatoms, which have delicate hierarchical pores and a large specific surface area, showing their utilization potential as oxygen sensing materials. Herein, we developed a superhydrophobic DO sensor which use the DFs as matrix materials. The sensor was fabricated by spraying the mixture of 1 H,1 H,2 H,2 H-perfluorooctyltriethoxysilane (PFOTS) modified DFs and oxygen-sensitive probe platinum (II)− 5,10,15,20-tetrakis-(2,3,4,5,6-pentafluorophenyl)-porphyrin (PtTFPP) on the bonding substrate. In the DO sensor, DFs provides a tough micro/nano surface structures and PFOTS offers low surface energy, both of which led to a robust superhydrophobic surface of the sensor. Because of the porous structure of DFs and the diffusion enhancement effect of fluorine on oxygen, the sensor has high DO sensitivity, I0/I100 which represents for oxygen sensitivity reaches up to 129. Additionally, the sensor showed good tolerance on mechanical abrasion, acidic or alkaline corrosion and biofouling. Owing to these good performances, the use of DFs is expected to provide a new strategy for the rational design of high performance DO sensing devices.

Global boundedness to a 3D chemotaxis-Stokes system with porous medium cell diffusion and general sensitivity

Abstract In this article, we will develop an analytical approach to construct the global bounded weak solutions to the initial-boundary value problem of a three-dimensional chemotaxis-Stokes system with porous medium cell diffusion Δ n m \Delta {n}^{m} for m ≥ 65 63 m\ge \frac{65}{63} and general sensitivity. In particular, this extended the precedent results which asserted global solvability within the larger range m > 7 6 m\gt \frac{7}{6} for general sensitivity (M. Winkler, Boundedness and large time behavior in a three-dimensional chemotaxis-Stokes system with nonlinear diffusion and general sensitivity, Calc. Var. 54 (2015), 3789–3828) or m > 9 8 m\gt \frac{9}{8} for scalar sensitivity (M. Winkler, Global existence and stabilization in a degenerate chemotaxis-Stokes system with mildly strong diffusion enhancement, J. Differ. Equ. 264 (2018), 6109–6151). Our proof is based on a new observation on the quasi-energy-type functional and on an induction argument.

Solitary fibrous tumor: A rare lesion with an unusual paravertebral presentation

BackgroundSolitary fibrous tumor (SFT) is a rare spindle-cell neoplasm [1]. Although typically originating from pleura, head and neck presentation accounts for about 6–18 % and very few cases have been described in paravertebral and posterior neck spaces [2]. Both computed tomography (CT) scans and magnetic resonance imaging (MRI) help in differential diagnosis of such lesion [3]. However, only histological and immunohistochemical studies give a conclusive diagnosis: CD34, BCL-2, and in particular STAT6 stainings are strongly orientative [4]. Radical surgery with free margin excision is the first treatment option, rarely requiring adjuvant therapy [2]. Prognosis is typically good and strictly related to histological risk assessment [5]. Rare cases of local recurrence and distant metastasis have been described in literature [2]. ObjectiveThe purpose of this video is to describe the operative technique of a transcervical removal of a rare case of right paravertebral SFT. We present the diagnostic flowchart, management strategies, surgical technique and we provide anatomical dissection parallelism, which might be of interest to the readers. Materials and methodsA 59-years-old man with a one-year right cervical asymptomatic swelling was referred to our department. A contrasted MRI documented an expansive 6.5 cm capsulated lesion in the deep posterior neck spaces with diffuse contrast enhancement and inhomogeneous appearance. A core needle biopsy was performed, and the results from the initial immunohistochemical panel were not univocal showing positivity for cytokeratins AE1/AE3, PAX8, and no reactivity for CD34. A second immunohistochemical panel was then performed, displaying diffuse nuclear positivity for STAT6, which is a surrogate marker for the NAB2-STAT6 gene fusion, a specific driver mutation of SFT. Therefore, a radical excision was performed via transcervical approach (Video 1). No post-operative complications neither cranio-cervical neurological deficit occurred. ResultsIn comparison to pre-operative histopathologic study, the definitive histological examination of the whole mass revealed a more classical morphology of SFT. It was classified as an intermediate risk SFT [5]. A complete free margin excision was confirmed. After a multidisciplinary discussion, no adjuvant therapies were suggested. A six- and twelve-months radiological follow-up with MRI showed no evidence of disease. ConclusionSFT may represent a misdiagnosed entity in head and neck spaces and a correct diagnosis through immunohistochemistry is mandatory. Radical excision with free surgical margins should be pursued as adequate goal. Since SFTs show variable risk of metastatic disease, adjuvant radiotherapy should be contemplated in high-risk diseases and a clinico-radiological follow-up with MRI is required.

Response to Comment on “Following Molecular Mobility during Chemical Reactions: No Evidence for Active Propulsion” and “Molecular Diffusivity of Click Reaction Components: The Diffusion Enhancement Question”

In their Comment (DOI: 10.1021/jacs.2c02965) on two related publications by our group (J. Am. Chem. Soc.2022, 144, 1380–1388; DOI: 10.1021/jacs.1c11754) and another (J. Am. Chem. Soc.2021, 143, 20884–20890; DOI: 10.1021/jacs.1c09455), Huang and Granick refer to the diffusion NMR measurements of molecules during a copper-catalyzed azide–alkyne cycloaddition (CuAAC) “click” reaction. Here we respond to their comments and maintain that no measurable diffusion enhancement was observed during the reaction. We expand on the physical arguments presented in our original JACS Article regarding the appropriate reference state for the diffusion coefficient and present new data showing that the use of other reference states, as suggested by Huang and Granick, will still support our conclusion that the two reactants and one product of the CuAAC reaction do not exhibit boosted mobility during the reaction.

Response to Comment on "Following Molecular Mobility during Chemical Reactions: No Evidence for Active Propulsion" and "Molecular Diffusivity of Click Reaction Components: The Diffusion Enhancement Question".

In their Comment (DOI: 10.1021/jacs.2c02965) on two related publications by our groups (J. Am. Chem. Soc. 2021, 143, 20884-20890; DOI: 10.1021/jacs.1c09455) and another (J. Am. Chem. Soc. 2022, 144, 1380-1388; DOI: 10.1021/jacs.1c11754), Huang and Granick discuss the diffusion NMR measurements of molecules during a copper-catalyzed azide-alkyne cycloaddition (CuAAC) "click" reaction. Here we respond to these comments and maintain that no diffusion enhancement was observed for any species during the reaction. We show that the relaxation agent does not interfere with the CuAAC reaction kinetics nor the diffusion of the molecules involved. Similarly, the gradient pulse length and diffusion time do not affect the diffusion coefficients. Peak overlap was completely removed in our study with the use of hydrazine as the reducing agent. The steady-state assumption does not hold for these diffusion measurements that take several minutes, which is the reason monotonic gradient orders are not suitable. Finally, we discuss the other reactions where similar changes in diffusion have been claimed. Our conclusions are fully supported by the results represented in our original JACS Article and the corresponding Supporting Information.

Comment on "Following Molecular Mobility during Chemical Reactions: No Evidence for Active Propulsion" and "Molecular Diffusivity of Click Reaction Components: The Diffusion Enhancement Question".

We provide arguments why we consider as inaccurate two recent JACS Articles which disagree with our laboratory's report of boosted diffusion during the copper-catalyzed azide-alkyne cycloaddition (CuAAC) "click" reaction. In the first paper (DOI: 10.1021/jacs.1c09455), Fillbrook et al. claim that their diffusion NMR experiments offer no evidence for boosted diffusion, but we argue that their use of Gd3+-chelates to speed up NMR relaxations times is flawed conceptually, the authors interpreting Gd3+-chelates as inert. Actually, the same features that make gadolinium ions useful as contrast agents in magnetic resonance imaging render them unsuitable for diffusion NMR. Nonetheless, by correctly adjusting technical aspects of the measurements, we confirm boosted diffusion even in the presence of this MRI contrast agent. The second paper of which we are skeptical, by Rezaei-Ghaleh et al. (DOI: 10.1021/jacs.1c11754), makes comparisons to a reference state that is not physically meaningful.

Anomalous diffusion and transport by a reciprocal convective flow.

Under low-Reynolds-number conditions, dynamics of convection and diffusion are usually considered separately because their dominant spatial and temporal scales are different, but cooperative effects of convection and diffusion can cause diffusion enhancement [Koyano et al., Phys. Rev. E 102, 033109 (2020)2470-004510.1103/PhysRevE.102.033109]. In this paper, such cooperative effects are investigated in detail. Numerical simulations based on the convection-diffusion equationrevealed that anisotropic diffusion and net shift as well as diffusion enhancement occur under a reciprocal flow. Such anomalous diffusion and transport are theoretically derived by the analyses of the Langevin dynamics.

Atypical rupture of a Poly Implant Prothése implant and fluid shifting: a case report

Women have undergone augmentation mammoplasty for decades, and if implant rupture is suspected, imaging modalities such as magnetic resonance imaging (MRI) and ultrasonography are available. The linguine sign, keyhole sign, and noose sign are all suggestive of rupture. However, earlier-generation implants with alternative filler materials demonstrate rupture signs that differ from those of today’s better-known implant materials. A 60-year-old female patient who had undergone augmentation mammoplasty 20 years ago presented with left breast swelling and pain in the lower-outer quadrant. Ultrasonography and MRI confirmed extracapsular diffuse wall enhancement with suspected, but not apparent, discontinuity in the patient’s left breast implant. Therefore, both implants were removed. It was determined that these were Poly Implant Prothése implants. The left breast implant differed from the contralateral implant in that it contained fluctuating fluid. This could be attributed to an osmotic gradient that caused the implant to swell and weakened the elastomer shell, resulting in micro rupture without a gross tear. Patients who undergo augmentation mammoplasty should have regular follow-up examinations, even if there are no symptoms. Furthermore, clinicians should be aware that some patients who have had breast augmentation mammoplasty may experience atypical symptoms and signs if the implant ruptures.

Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor, first described by the WHO Classification of Central Nervous System Tumors in 2016. The clinical course is variable. Most tumors have low-grade histological findings although some may have more aggressive features. The goal of this systematic review was to identify prognostic factors for poor overall survival (OS). We performed a systematic review using three databases (PubMed, Google Scholar, and Embase) and the following search terms: diffuse leptomeningeal glioneuronal tumor, DLGNT, DLMGNT. Statistical analysis was performed using Statistica 13.3. We included 34 reports in our review comprising 63 patients, published from 2016 to 2022. The median OS was 19months (range: 12-51months). Using multivariable Cox survival analysis, we showed that Ki-67 ≥ 7%, age > 9years, symptoms of elevated intracranial pressure (ICP) at admission, and the presence of contrast-enhancing intraparenchymal tumor are associated with poor OS. Receiver operating characteristic (ROC) analysis identified Ki-67 ≥ 7% as a significant predictor of poor OS. Signs or symptoms of increased ICP with imaging findings of diffuse leptomeningeal enhancement should raise suspicion for DLGNT. In our systematic review, Ki-67 ≥ 7% was the most important prognostic factor for OS in DLGNT. The presence of intraparenchymal tumor with contrast enhancement was thought to represent disease progression and, together with patient age, was associated with poor OS.