Correlation between the miR-618 rs2682818 C>A polymorphism and venous malformation susceptibility.

Venous malformations are the most common congenital vascular malformations, and the incidence rate is high. Previous studies have confirmed that a variety of polymorphisms within the miRNA functional region are associated with tumor susceptibility. We examined the correlation between miR-618 rs2682818 C>A and risk of developing venous malformation in a southern Chinese population (1113 patients and 1158 controls). TaqMan genotyping of miR-618 rs2682818 C>A was conducted utilizing real-time fluorescent quantitative PCR. The miR-618 rs2682818 polymorphism was not correlated with susceptibility to venous malformation (CA/AA vs. CC: adjusted odds ratio [AOR] = 1.00, 95% confidence interval [CI] = 0.81-1.25, p = 0.994; AA vs. CC/CA: AOR = 1.10, 95% CI = 0.73-1.65, p = 0.646). Stratified analysis of different subtypes of venous malformation revealed that there was no significant difference in the rs2682818 C>A polymorphism genotypes across these subtypes. Our results indicate that miR-618 rs2682818 C>A polymorphism is not correlated with the susceptibility to venous malformation.

Eye-Preserving Therapies for Advanced Retinoblastoma: A Multicenter Cohort of 1678 Patients in China.

PURPOSE: This study attempted to estimate the impact of eye-preserving therapies for the long-term prognosis of patients with advanced retinoblastoma with regard to overall survival and ocular salvage. DESIGN: Retrospective cohort study covering all 31 provinces (38 retinoblastoma treating centers) of mainland China. PARTICIPANTS: One thousand six hundred seventy-eight patients diagnosed with group D or E retinoblastoma from January 2006 through May 2016. METHODS: Chart review was performed. The patients were divided into primary enucleation and eye-preserving groups, and they were followed up for survival status. The impact of initial treatment on survival was evaluated by Cox analyses. MAIN OUTCOME MEASURES: Overall survival and final eye preservation. RESULTS: After a median follow-up of 43.9 months, 196 patients (12%) died, and the 5-year overall survival was 86%. In total, the eyeball preservation rate was 48%. In this cohort, 1172 patients (70%) had unilateral retinoblastoma, whereas 506 patients (30%) had bilateral disease. For patients with unilateral disease, 570 eyes (49%) underwent primary enucleation, and 602 patients (51%) received eye-preserving therapies initially. During the follow-up (median, 45.6 months), 59 patients (10%) from the primary enucleation group and 56 patients (9.3%) from the eye-preserving group died. Multivariate Cox analyses indicated no significant difference in overall survival between the 2 groups (hazard ratio [HR], 1.25; 95% confidence interval [CI], 0.85-1.84; P = 0.250). For patients with bilateral disease, 95 eyes (19%) underwent primary enucleation, and 411 patients (81%) received eye-preserving therapies initially. During the follow-up (median, 40.1 months), 12 patients (13%) from the primary enucleation group and 69 patients (17%) from the eye-preserving group died. For bilateral retinoblastoma with the worse eye classified as group E, patients undergoing primary enucleation exhibited better overall survival (HR, 2.35; 95% CI, 1.10-5.01; P = 0.027); however, this survival advantage was not evident until passing 22.6 months after initial diagnosis. CONCLUSIONS: Eye-preserving therapies have been used widely for advanced retinoblastoma in China. Patients with bilateral disease whose worse eye was classified as group E and who initially underwent eye-preserving therapies exhibited a worse overall survival. The choice of primary treatment for advanced retinoblastoma should be weighed carefully.

Radiogenomics correlation between MR imaging features and mRNA-based subtypes in lower-grade glioma.

BACKGROUND: To investigate associations between lower-grade glioma (LGG) mRNA-based subtypes (R1-R4) and MR features. METHODS: mRNA-based subtyping was obtained from the LGG dataset in The Cancer Genome Atlas (TCGA). We identified matching patients (n = 145) in The Cancer Imaging Archive (TCIA) who underwent MR imaging. The associations between mRNA-based subtypes and MR features were assessed. RESULTS: In the TCGA-LGG dataset, patients with the R2 subtype had the shortest median OS months (P < 0.05). The time-dependent ROC for the R2 subtype was 0.78 for survival at 12 months, 0.76 for survival at 24 months, and 0.76 for survival at 36 months. In the TCIA-LGG dataset, 41 (23.7%) R1 subtype, 40 (23.1%) R2 subtype, 19 (11.0%) R3 subtype and 45 (26.0%) R4 subtype cases were identified. Multivariate analysis revealed that enhancing margin (ill-defined, OR: 9.985; P = 0.003) and T1 + C/T2 mismatch (yes, OR: 0.091; P = 0.023) were associated with the R1 subtype (AUC: 0.708). The average accuracy of the ten-fold cross validation was 71%. Proportion of contrast-enhanced (CE) tumour (> 5%, OR: 14.733; P < 0.001) and necrosis/cystic changes (yes, OR: 0.252; P = 0.009) were associated with the R2 subtype (AUC: 0.832). The average accuracy of the ten-fold cross validation was 82%. Haemorrhage (yes, OR: 8.55; P < 0.001) was positively associated with the R3 subtype (AUC: 0.689). The average accuracy of the ten-fold cross validation was 87%. Proportion of CE tumour (> 5%, OR: 0.14; P < 0.001) was negatively associated with the R4 subtype (AUC: 0.672). The average accuracy of the ten-fold cross validation was 71%. For the prediction of the R2 subtype, the nomogram showed good discrimination and calibration. Decision curve analysis demonstrated that prediction with the R2 model was clinically useful. CONCLUSIONS: Patients with the R2 subtype had the worst prognosis. We demonstrated that MRI features can identify distinct LGG mRNA-based molecular subtypes.

Oligodendroglial tumours: subventricular zone involvement and seizure history are associated with CIC mutation status.

BACKGROUND: CIC-mutant oligodendroglial tumours linked to better prognosis. We aim to investigate associations between CIC gene mutation status, MR characteristics and clinical features. METHODS: Imaging and genomic data from the Cancer Genome Atlas and the Cancer Imaging Archive (TCGA/TCIA) for 59 patients with oligodendroglial tumours were used. Differences between CIC mutation and CIC wild-type were tested using Chi-square test and binary logistic regression analysis. RESULTS: In univariate analysis, the clinical variables and MR features, which consisted 3 selected features (subventricular zone[SVZ] involvement, volume and seizure history) were associated with CIC mutation status (all p < 0.05). A multivariate logistic regression analysis identified that seizure history (no vs. yes odd ratio [OR]: 28.960, 95 confidence interval [CI]:2.625-319.49, p = 0.006) and SVZ involvement (SVZ- vs. SVZ+ OR: 77.092, p = 0.003; 95% CI: 4.578-1298.334) were associated with a higher incidence of CIC mutation status. The nomogram showed good discrimination, with a C-index of 0.906 (95% CI: 0.812-1.000) and was well calibrated. SVZ- group has increased (SVZ- vs. SVZ+, hazard ratio [HR]: 4.500, p = 0.04; 95% CI: 1.069-18.945) overall survival. CONCLUSIONS: Absence of seizure history and SVZ involvement (-) was associated with a higher incidence of CIC mutation.

Pigment epithelium-derived factor/vascular endothelial growth factor ratio plays a crucial role in the spontaneous regression of infant hemangioma and in the therapeutic effect of propranolol.

Infantile hemangioma (IH) is a benign tumor that is formed by aberrant angiogenesis and that undergoes spontaneous regression over time. Propranolol, the first-line therapy for IH, inhibits angiogenesis by downregulating activation of the vascular endothelial growth factor (VEGF) pathway, which is hyperactivated in IH. However, this treatment is reportedly ineffective for 10% of tumors, and 19% of patients relapse after propranolol treatment. Both pro-angiogenic and anti-angiogenic factors regulate angiogenesis, and pigment epithelium-derived factor (PEDF) is the most effective endogenous anti-angiogenic factor. PEDF/VEGF ratio controls many angiogenic processes, but its role in IH and the relationship between this ratio and propranolol remain unknown. Results of the present study showed that the PEDF/VEGF ratio increased during the involuting phase of IH compared with the proliferating phase. Similarly, in hemangioma-derived endothelial cells (HemEC), which were isolated with magnetic beads, increasing the PEDF/VEGF ratio inhibited proliferation, migration, and tube formation and promoted apoptosis. Mechanistically, the VEGF receptors (VEGFR1 and VEGFR2) and PEDF receptor (laminin receptor, LR) were highly expressed in both IH tissues and HemEC, and PEDF inhibited HemEC function by binding to LR. Interestingly, we found that propranolol increased the PEDF/VEGF ratio but did so by lowering VEGF expression rather than by upregulating PEDF as expected. Furthermore, the combination of PEDF and propranolol had a more suppressive effect on HemEC. Consequently, our results suggested that the PEDF/VEGF ratio played a pivotal role in the spontaneous regression of IH and that the combination of PEDF and propranolol might be a promising treatment strategy for propranolol-resistant IH.

Treatment of Corticosteroid-Resistant Vascular Tumors Associated with the Kasabach-Merritt Phenomenon in Infants: An Approach with Transcatheter Arterial Embolization Plus Vincristine Therapy.

PURPOSE: To investigate the effectiveness and application of transcatheter arterial embolization (TAE) plus systemic vincristine for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants. MATERIALS AND METHODS: TAE was performed in 17 infants (average age, 4.3 mo ± 2.4; range, 1-10 mo) with corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon, followed by intravenous vincristine once weekly for systemic chemotherapy. The effects and complications were observed and evaluated after a cycle (1 cycle: TAE plus treatment with vincristine every 4 weeks). Cycles were repeated in infants with platelet counts < 150 × 10(9)/L. RESULTS: In 17 patients, 36 treatment cycles were successfully performed. The platelet count for all patients increased to ≥ 100 × 10(9)/L for the first time at 6.0 days ± 3.5; the platelet level of 15 infants was maintained at levels > 150 × 10(9)/L at 57.5 days ± 16.5. Before treatment, two infants had a normal fibrinogen level (2.21 g/L and 2.34 g/L); the fibrinogen level in the other 15 infants was first found to be increased to ≥ 2.0 g/L at 7.0 days ± 3.4 and was stabilized at levels > 2.0 g/L at 55.9 days ± 13.8 after treatment. Complications were graded as major in four cases and as minor in 13 cases. CONCLUSIONS: TAE plus vincristine can rapidly improve levels of platelets and fibrinogen, and it is an effective method for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants.

Osteopontin enhances the expression of HOTAIR in cancer cells via IRF1.

Osteopontin (OPN), a secreted phosphoglycoprotein, plays important roles in tumor growth, invasion, and metastasis for many types of cancers. The long, noncoding RNA HOTAIR has been strongly associated with the invasion and metastasis of cancer cells. In this study, we found that recombinant human OPN could induce HOTAIR expression in a time- and dose-dependent manner, and our data also showed that OPN transcriptionally activated the expression of HOTAIR in cancer cells. Furthermore, through chromatin immunoprecipitation and luciferase activity assays, we found that IRF1 could bind to the HOTAIR promoter region and decrease its transcriptional activity, and cellular overexpression of IRF1 downregulated the level of HOTAIR. The receptor CD44 has also been verified as a regulator of OPN-induced HOTAIR expression. Interestingly, our data demonstrated that OPN could regulate PI3K/AKT and IRF1 expression and signaling, thereby influencing the expression of HOTAIR. In hepatocellular carcinoma samples, levels of HOTAIR correlated with the expression of OPN and IRF1. We therefore conclude that OPN, as an extracellular matrix protein, can stimulate the expression of HOTAIR by attenuating the inhibitory effect of IRF1, and this results in promotion of the invasion and metastasis of cancer cells.

Further considerations on hepatic lesion discrimination using diffusion MR imaging.

With great interest, we read the article "Differential diagnosis between hepatic metastases and benign focal lesions using DWI with parallel acquisition technique: a meta-analysis" (by Wei CG et al.). This article suggests that diffusion-weighted imaging was useful for differentiation between hepatic metastases and benign focal lesions. The result is encouraging. We would like to contribute regarding the value of DWI with parallel acquisition technique in the differentiation of metastases and benign hepatic lesions.

Primary renal synovial sarcoma: computed tomography imaging findings.

BACKGROUND: A synovial sarcoma arising from the kidney is extremely rare. To date, few data are available on their radiological features. PURPOSE: To identify the computer tomography (CT) imaging findings of primary renal synovial sarcomas (PRSSs). MATERIAL AND METHODS: Five cases of PRSS confirmed by histopathological and cytogenetic studies were retrospectively analyzed. All patients had undergone unenhanced and multiphase enhanced CT examinations (one patient underwent CT twice). The CT characteristics, including shape, size, margin, attenuation, and enhancement pattern after intravenous contrast medium injection, were analyzed. RESULTS: The study involved two female and three male patients (mean age, 27.4 years; range, 15-43 years). Unenhanced CT showed completely or partly well-defined masses, with heterogeneous (n = 5) or homogeneous (n = 1) patchy low density. On multiphase contrast-enhanced CT, in five of the six CT examinations, the tumors appeared as solid-cystic masses with cyst walls or pseudo-capsules, and demonstrated moderately heterogeneous (n = 5) and/or septate enhancement (n = 2), with a "rapid wash-in and slow wash-out" pattern of enhancement in the solid component. Only one tumor showed a simple cyst appearance and developed an irregular, intratumoral, septate soft density 8 months later. A renal vein and inferior vena cava thrombus was noticed in one patient, while lymphadenopathy was not observed in any patient. CONCLUSION: PRSS should be included in the differential diagnosis when an adolescent or young adult presents with a renal neoplasm appearing as a solid-cystic mass with well-defined borders, a cystic wall or pseudo-capsule, heterogeneous or septate enhancement, a "rapid wash-in and slow wash-out" pattern of enhancement in the solid component, and no sign of lymphadenopathy.

Combination of radiofrequency ablation with transarterial chemoembolization for hepatocellular carcinoma: an up-to-date meta-analysis.

The aim of this meta-analysis was to compare the effectiveness of combination of radiofrequency ablation (RFA) and transarterial chemoembolization (TACE) with that of RFA alone in patients with hepatocellular carcinoma (HCC). Randomized controlled trials comparing RFA plus TACE with RFA alone for HCC were included into this meta-analysis, and the search strategy followed the requirement of the Cochrane Library Handbook. Overall survival rate and recurrence-free survival rate were analyzed and compared by using Review Manager (version 5). We identified 7 randomized controlled trials comprising 571 patients who were treated by RFA plus TACE versus RFA alone for HCC. Meta-analyses showed that the combination of RFA and TACE was associated with a significantly higher overall survival rates (OR1 year = 2.39, 95 % CI, 1.35-4.21, P = 0.003; OR3 years = 1.85, 95 %CI 1.26-2.71, P = 0.002), and recurrence-free survival rate (OR1 year = 2.00, 95 % CI 1.26-3.18, P = 0.003; OR3 years = 2.13, 95 %CI 1.41-3.20, P < 0.001). Additionally, the quality of the evidence was high for the 1- and 3-year survival rate; no evidence of publication bias was observed. The combination of RFA with TACE can improve the overall survival rate and the recurrence-free survival rate for patients with HCC.

Imaging characteristics of primary intracranial teratoma.

BACKGROUND: Primary intracranial teratomas are rare intracranial neoplasms, and are subdivided into mature, immature, and those with malignant transformation. To date, only a few studies of teratoma imaging have been reported. PURPOSE: To describe and characterize the magnetic resonance imaging (MRI) findings in a series of 18 patients (16 men/boys and 2 women/girls; mean age, 14.5 years) with pathologically proven teratomas. MATERIAL AND METHODS: Findings from medical records and imaging examinations in 18 patients with pathologically confirmed intracranial teratomas from 2001 to 2011 were retrospectively reviewed at our two institutions. Two radiologists evaluated the lesion location, shape, size, number, edge, homogeneous or heterogeneous appearance, attenuation, signal intensity, and degree of enhancement. RESULTS: All tumors were located within the pineal (n = 13), parasellar (n = 2), or suprasellar (n = 3) regions. The lesions appeared of mixed intensity on MRI, reflecting the histologic heterogeneity, including fibrosis, fatty tissue, calcification, cysts, and keratinocytes. In mature teratomas (n = 9), seven of nine tumors showed non-enhanced multilocularity or heterogeneous enhancement of the cyst wall on contrast-enhanced T1-weighted (T1W) images. Two of nine tumors showed moderate, heterogeneous enhancement in the solid portion of the lesion; whereas in immature (n = 7) or malignant transformation (n = 2) teratomas, heterogeneous, ring-like, intratumoral patchy enhancement was noted on T1W images with contrast. CONCLUSION: Primary intracranial teratomas are usually localized in the pineal and the suprasellar regions, and often present an ovoid or lobulated mass with or without multilocularity on MRI. Marked enhancement of the solid portion or the thick wall of the tumor was the key feature for distinguishing mature teratoma and malignant teratoma.

Association Between the miR-100 rs1834306 A>G Polymorphism and Susceptibility to Venous Malformation.

Background: Venous malformation is related to genes and results in functional and morphologic anomalies. Genetic variations affecting the development of vessel endothelial cells are unclear. Therefore, this study aimed to investigate the potential value of the miR-100 rs1834306 A>G polymorphism as a marker of susceptibility to venous malformation. Methods: In this case-control study in southern Chinese children, we collected blood samples from 1158 controls and 1113 patients with venous malformation. TaqMan genotyping of miR-100 rs1834306 A>G was performed by real-time fluorescent quantitative polymerase chain reaction. Results: Multivariate logistic regression analysis showed that there was no significant association between the presence of the miR-100 rs1834306 A>G polymorphism and susceptibility to venous malformation by evaluating the values of pooled odds ratios and 95% confidence intervals. Similarly, among different sites, rs1834306 A>G was also not associated with venous malformation. Conclusion: Our results suggest that the miR-100 rs1834306 A>G polymorphism is not associated with susceptibility to venous malformation in southern Chinese children. These results need to be further confirmed by investigating a more diverse ethnic population of patients with venous malformations.

Abnormal regional homogeneity of resting-state brain activity in patients with HBV-related cirrhosis without overt hepatic encephalopathy.

BACKGROUND: Many studies have reported that cognitive deficits exist in cirrhotic patients without overt hepatic encephalopathy (OHE). However, the neurobiological mechanisms underlying these deficits are still not fully understood. AIM: To investigate regional activity abnormalities in patients with hepatitis B virus-related cirrhosis (HBV-RC) without OHE using resting-state functional MRI (Rs-fMRI), and to examine the relationship between regional activity abnormalities and impaired cognition. METHODS: A newly reported regional homogeneity (ReHo) approach was used to compare the local synchronization of Rs-fMRI signals in 32 patients with HBV-RC without OHE and 32 well-matched healthy controls. Cognition was measured in all patients using psychometric hepatic encephalopathy score (PHES) tests, and the relationship between ReHo variation and PHES was analysed. RESULTS: Relative to healthy controls, the cirrhosis group showed high ReHo in the prefrontal cortex, and widespread low ReHo in visual association areas (left lingual gyrus, middle temporal gyrus and right middle occipital gyrus), motor association areas (bilateral precentral gyrus and paracentral lobule) and the bilateral precuneus. Correlation analysis of the mean ReHo values in different brain areas and PHES in cirrhotic patients revealed a significantly positive correlation in the left lingual gyrus (r = 0.352; P = 0.048), right middle occipital gyrus (r = 0.453; P = 0.009) and bilateral precentral gyrus (left: r = 0.436, P = 0.013; right: r = 0.582, P < 0.001), paracentral lobule (r = 0.485; P = 0.005) and precuneus (r = 0.468; P = 0.007). CONCLUSIONS: Our results provide information on the pathophysiological mechanisms underlying cognitive alterations in cirrhotic patients and demonstrate the feasibility of using Rs-fMRI with ReHo analysis as a noninvasive modality with which to detect the progression of cognitive changes in cirrhotic patients.

Abnormal baseline brain activity in patients with HBV-related cirrhosis without overt hepatic encephalopathy revealed by resting-state functional MRI.

Neurocognitive dysfunction of varying degrees is common in patients with hepatitis B virus-related cirrhosis (HBV-RC) without overt hepatic encephalopathy (OHE). However, the neurobiological mechanisms underlying these dysfunctions are not well understood. We sought to identify changes in the neural activity of patients with HBV-RC without OHE in the resting state by using the amplitude of low-frequency fluctuation (ALFF) method and to determine whether these changes were related to impaired cognition. Resting-state functional MRI data from 30 patients with HBV-RC and 30 healthy controls matched for age, sex, and years of education were compared to determine any differences in the ALFF between the two groups. Cognition was measured with the psychometric hepatic encephalopathy score (PHES), and the relationship between these scores and ALFF variation was assessed. Compared with controls, patients showed widespread lower standardized ALFF (mALFF) values in visual association areas (bilateral lingual gyrus, middle occipital gyrus, and left inferior temporal gyrus), motor-related areas (bilateral precentral gyrus, paracentral lobule, and right postcentral gyrus), and the default mode network (bilateral cuneus/precuneus and inferior parietal lobule). Higher mALFF values were found in the bilateral orbital gyrus/rectal gyrus. In patients, mALFF values were significantly positive correlated with the PHES in the right middle occipital gyrus and bilateral precentral gyrus. Our findings of resting-state abnormalities in patients with HBV-RC without OHE suggest that neurocognitive dysfunction in patients with HBV-RC without OHE may be caused by abnormal neural activity in multiple brain regions.

Characteristic CT and MRI findings of intracranial chondroma.

BACKGROUND: Intracranial chondromas are rare benign tumors. To date, few data are available on their neuroradiological features. PURPOSE: To describe a series of patients with intracranial chondroma and to analyze and discuss the computed tomography (CT) and magnetic resonance imaging (MRI) features that may distinguish chondromas from other intracranial neoplasms. MATERIAL AND METHODS: We retrospectively analyzed clinical and medical imaging data of six patients who had pathologically confirmed intracranial chondromas in our two institutions between July 2006 and September 2011. Both CT and MRI scanning were performed in all six cases. RESULTS: Five tumors were located at the skull base and one originated from the falx. CT images revealed well-demarcated, irregular lobulated and variable density masses with obvious calcification (6/6), no or slight enhancement, without peritumoral edema, and frequently accompanied by erosion and destruction of surrounding bone (5/6). Tumor parenchyma appeared heterogeneously hypointense on T1WI, and hyperintense or mixed hyperintense and hypointense on T2WI, while the calcification appeared hypointense on T1WI and T2WI in five cases, demonstrating significant inhomogeneous enhancement on postcontrast MRI, which revealed the typical "punica granatum seeds" sign. Only one case showed homogeneous low signal intensity on T1WI and high signal intensity on T2WI, and relatively uniform obvious enhancement on postcontrast scans. CONCLUSION: These characteristic CT and MR findings, combined with the location of the lesions and the history of a long duration of clinical symptoms, may prove helpful in differentiating intracranial chondromas from other more common tumors.

Wedelolactone induces apoptosis and pyroptosis in retinoblastoma through promoting ROS generation.

Retinoblastoma is a most frequently occurring primary intraocular tumor in infancy and children, highlighting the requirement to find and develop novel and more effective therapeutic approaches. Wedelolactone (WDL), a nature compound isolated from E. prostrata, exhibits multiple biological activities through regulating various signaling pathways; however, its potential influences on retinoblastoma progression are still unknown, and thus was investigated in our study, as well as the underlying mechanisms. Here, we found that WDL treatments significantly reduced the proliferation of retinoblastoma cells by inducing apoptosis and pyroptosis through increasing Caspase-3, Caspase-1, gasdermin E (GSDME) and gasdermin D (GSDMD) activation. Mitochondrial impairment and reactive oxygen species (ROS) generation were considerably up-regulated in WDL-incubated retinoblastoma cells through a dose-dependent manner. Notably, we found that ROS scavenge significantly abolished the function of WDL to provoke apoptosis and pyroptosis in retinoblastoma cell lines, revealing that ROS was required for WDL to perform its anti-cancer role in retinoblastoma. Moreover, our in vivo experiments indicated that WDL administration significantly reduced the tumor growth in the established retinoblastoma mouse models with undetectable toxicity. Collectively, these findings highlighted the potential of WDL to inhibit the growth and induce cell death of retinoblastoma in vitro and in vivo, and thereby showed promise as a therapeutic agent for the treatment of retinoblastoma.

Contemporary Update of Retinoblastoma in China: Three-Decade Changes in Epidemiology, Clinical Features, Treatments, and Outcomes.

PURPOSE: To report three-decade changes of clinical characteristics, progress of treatments, and risk factors associated with mortality and enucleation in patients with retinoblastoma in China. DESIGN: Retrospective cohort study. METHODS: This multicenter study included 2552 patients diagnosed with retinoblastoma in 38 medical centers in 31 provinces in China from 1989 to 2017, with follow-up data. Kendall's tau-b value was used to describe correlation coefficients between the three eras (between 1989 and 2008, between 2009 and 2013, and between 2014 and 2017) and clinical or demographic features. Hazard ratios and odds ratios were applied to measure risk factors. RESULTS: A total of 324 (13%) patients died and 1414 (42%) eyes were removed. The 1-year, 3-year, and 5-year overall survival rates were 95%, 86%, and 83%, respectively. Patients were diagnosed at a better stage by International Classification for Retinoblastoma over time (Kendall's tau-b value = -0.084, P < .001). Pathological risk factors were also observed less in recent eras. New conservative therapies were adopted and used in more patients. The eye removal rate gradually decreased (Kendall's tau-b value = -0.167, P < .001). The overall survival rates were 81%, 83%, and 91% in the three eras. By multivariate Cox regression, bilateral tumors and extraocular extension were identified as risk factors for death. Among intraocular disease, Group E indicated higher risk of mortality. By multivariate logistics regression, unilateral tumors, earlier era of diagnosis, and extraocular extension were risk factors for eye salvage failure. Among intraocular retinoblastoma, Groups D and E had higher risk of eye salvage failure. CONCLUSIONS: Patients were diagnosed at an earlier stage in recent eras. Conservative therapies, including intra-arterial chemotherapy, were increasingly being used. The above changes may contribute to the decreasing enucleation rate. Although no significant impact was identified on the mortality by the three eras, a decreasing trend was shown.

Intra-arterial chemotherapy for treatment of very low birthweight infants with retinoblastoma: Two case reports.

INTRODUCTION: Intra-arterial chemotherapy (IAC) has been used successfully for unilateral and bilateral eye disease with advanced or less advanced intraocular disease and of children as young as 3 months of age. In this study, we expanded the use of IAC to eyes of two infants with very low birthweight (birth weight of 1000-1500 g) and retinoblastoma. PATIENT CONCERNS: The ophthalmologists refused to give patients intravenous chemotherapy (IVC), considering the risk of IVC because of the low body weight. DIAGNOSIS: Two patients were diagnosed unilateral eye retinoblastoma at 31 days old and 27 days old respectively when newborn fundus screening. INTERVENTIONS: Chemotherapeutic agents were injected into the ophthalmic artery during the four IAC session. The two patients' oculars were preserved due to calcification during follow-up periods to the final IAC. OUTCOMES: Complications included eyelid edema in the patient #1 and neutropenia in both patients. No fever, sepsis and other systemic toxic effects were observed. After two successful IAC treatment for each patient, their oculars were all preserved without serious complications. LESSONS: Our center for the first time reported the management of retinoblastoma in very low birthweight infants with especially challenging and we recommend this to be useful to the patient community.

Analysis of angiographic characteristics of kaposiform hemangioendothelioma and investigation of the value of transcatheter arterial embolization therapy.

BACKGROUND: This study aimed to analyze the angiographic characteristics of kaposiform hemangioendothelioma (KHE) and investigate the value of transcatheter arterial embolization (TAE) therapy. METHODS: The clinical data of infants diagnosed with KHE at the department from June 2013 to June 2020 were retrospectively analyzed. Of these, 34 infants received TAE therapy. The efficacy of the treatment was evaluated 4 weeks after the therapy. The angiographic characteristics were analyzed by comparing them with the angiographic characteristics of infantile hemangioma (IH), and the times of TAE therapy and the platelet level after each TAE therapy in infants with KHE were summarized. RESULTS: The present study showed that the capillary blush of KHE was irregular with an obscure boundary and nonuniform distribution. Many fine feeding arteries were present. The diameter of the feeding arteries was disproportionate to the volume of the tumor blush. The normal arteries were usually embedded in the tumor blush. The angiography of common IH in infants also showed tumor blush, but it was usually round with a clear boundary and uniform staining, and was distributed on 1 side of the normal arterial trunk. The infants with KHE received TAE therapy for 2 to 5 times/case, with a total of 104.0 times, with an average of 3.1±0.8/case. Among which, the platelets continued to decline for 9 times after TAE therapy and the platelets increased to ≥100×109/L in 7.8±3.2 days for 95 times after TAE therapy, The average relapse time was 30.0±15.9 days. CONCLUSIONS: The feeding arteries of KHE were numerous and fine and were not easily embolized. The application of TAE may rapidly improve the platelet level, but the long-term effect is poor.