Objective:To investigate the clinicopathological features, classification, and genetic characteristics of common lymphatic malformation (CLM) in superficial soft tissue.Methods:A retrospective study of 110 patients with the diagnosis of CLM at the Henan Province People′s Hospital, China from August 2019 to August 2022 was performed. The clinicopathological features, relevant immunohistochemical (IHC) staining results, and fluorescence quantitative PCR of PIK3CA mutation were analyzed, and patients were followed up.Results:Among the 110 CLM patients, there were 53 males and 57 females; 65 cases (65/110, 59.1%) were first detected when the patients were≤2 years old. The most common location was the head and neck in 41 cases (41/110, 37.3%). Clinically, 102 cases (102/110, 92.7%) were solitary, 83 cases (83/110, 75.5%) were skin-colored, 69 cases (69/110, 62.7%) had indistinct borders, and 10 cases (10/110, 9.1%) had diffuse and severe macroscopic manifestations. There were 52 macrocystic type (52/110, 47.3%), 23 microcystic type (23/110, 20.9%), and 35 combined type (35/110, 31.8%). The macrocystic CLM presented as soft, translucent masses with large cystic cavities on the cut surface, and histologically they were composed of large, irregularly dilated channels that were thicker with irregular smooth muscle and lymphocytic infiltration. Microcystic CLM showed wartlike projections or translucent blisters on the skin, with small honeycomb structures on the cut surface, and histologically consisted of round or angular dilated small lymphatic vessels with little or no smooth muscle. The combined CLM had both macrocystic and microcystic morphologies. IHC staining showed that the lymphatic endothelial cells were positive for LYVE-1, D2-40, PROX1, CD31, and VEGFR3 but negative for CD34; in the macrocystic and combined CLM vessel walls were positive for SMA. Eight of 13 CLM had PIK3CA mutation. All patients were followed up, and 24 (24/110, 21.8%) had relapses, which more frequently occurred in combined type, followed by microcystic type.Conclusions:CLM is a congenital vascular malformation composed of dilated, abnormal lymphatic channels, with PIK3CA mutation. There are significant differences in clinicopathological characteristics among the different types. Since microcystic and combined CLM are prone to recurrence, accurate pathological subtyping is necessary to guide treatment and to predict prognosis.

Objective:To compare the diagnostic efficacy of ultrasound and MRI in fibro-adipose vascular anomaly (FAVA).Methods:The clinical data of patients with suspected FAVA who underwent ultrasound and MRI examinations at Henan Provincial People’s Hospital from January 2011 to October 2021 were retrospectively analyzed. The imaging findings from ultrasound and MRI were analyzed, and then compared with the pathological findings. To evaluate the diagnostic efficacy of ultrasound and MRI in diagnosing FAVA by assessing sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate. Paired χ2 test (McNemar test) was used to compare the coincidence rate of ultrasound and MRI, as well as their combined diagnosis. A significance level of P < 0.05 was considered statistically significant. Results:A total of 50 patients were included in the study, comprising 24 males and 26 females, with their ages ranging from 1 to 50 years and an average age of (16.2 ± 10.5) years. Pathology confirmed 43 FAVA patients and 7 non-FAVA patients. The sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate of ultrasound in the diagnosis of FAVA were 83.7%, 71.4%, 94.7%, 41.7%, and 82.0%, respectively. The sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate of MRI in the diagnosis of FAVA were 69.8%, 85.7%, 96.8%, 31.6%, and 72.0%, respectively. The sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate of FAVA were 90.7%, 71.4%, 95.1%, 55.6%, and 88.0%, respectively. The diagnostic accuracy of ultrasound was higher than that of MRI, but the difference was not statistically significant ( χ2 = 1.41, P = 0.235). The coincidence rate of combined diagnosis was higher than that of ultrasound ( χ2= 0.71, P = 0.401) and MRI ( χ2= 4.00, P = 0.039), with a statistically significant difference. Conclusion:Both ultrasound and MRI are highly valuable in diagnosing FAVA. The combined usage of ultrasound and MRI can enhance the accuracy of preoperative FAVA diagnosis.

Objective:To compare the diagnostic efficacy of ultrasound and MRI in fibro-adipose vascular anomaly (FAVA).Methods:The clinical data of patients with suspected FAVA who underwent ultrasound and MRI examinations at Henan Provincial People’s Hospital from January 2011 to October 2021 were retrospectively analyzed. The imaging findings from ultrasound and MRI were analyzed, and then compared with the pathological findings. To evaluate the diagnostic efficacy of ultrasound and MRI in diagnosing FAVA by assessing sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate. Paired χ2 test (McNemar test) was used to compare the coincidence rate of ultrasound and MRI, as well as their combined diagnosis. A significance level of P < 0.05 was considered statistically significant. Results:A total of 50 patients were included in the study, comprising 24 males and 26 females, with their ages ranging from 1 to 50 years and an average age of (16.2 ± 10.5) years. Pathology confirmed 43 FAVA patients and 7 non-FAVA patients. The sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate of ultrasound in the diagnosis of FAVA were 83.7%, 71.4%, 94.7%, 41.7%, and 82.0%, respectively. The sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate of MRI in the diagnosis of FAVA were 69.8%, 85.7%, 96.8%, 31.6%, and 72.0%, respectively. The sensitivity, specificity, positive predictive value, negative predictive value, and coincidence rate of FAVA were 90.7%, 71.4%, 95.1%, 55.6%, and 88.0%, respectively. The diagnostic accuracy of ultrasound was higher than that of MRI, but the difference was not statistically significant ( χ2 = 1.41, P = 0.235). The coincidence rate of combined diagnosis was higher than that of ultrasound ( χ2= 0.71, P = 0.401) and MRI ( χ2= 4.00, P = 0.039), with a statistically significant difference. Conclusion:Both ultrasound and MRI are highly valuable in diagnosing FAVA. The combined usage of ultrasound and MRI can enhance the accuracy of preoperative FAVA diagnosis.

Objective:To investigate the clinical features and related risk factors of multi-drug resistant organism (MDRO) infection after liver transplantation, so as to guide clinical identification of high-risk patients and reduce the occurrence of MDRO infection.Methods:Of 187 patients undergoing liver transplantation in the First Affiliated Hospital of Nanjing Medical University from February 2019 to September 2020 were enrolled, including 150 males and 37 females, aged (50.6±9.6) years. Data related to MDRO infection were collected, and independent risk factors were identified using univariate analysis and multiple logistic regression analysis.Results:Among the 187 patients, MDRO infection occurred in 39, and 9 patient deaths were directly related to MDRO infection. Lung is the most common infection site, accounting for 82.1% (32/39) of MDRO infection. The most common pathogenic bacteria were Acinetobacter baumannii and Carbapenem-resistant enterobacteriaceae, accounting for 46.8%(36/77) and 41.7%(32/77) of MDRO infection, respectively. Multiple logistic regression analysis showed that postoperative mechanical ventilation ≥ 48 h ( OR=3.430, 95% CI: 1.124-10.467, P=0.030), intensive care unit (ICU) stay ≥7 d ( OR=9.013, 95% CI: 3.054-26.594, P<0.001) were independent risk factors of postoperative MDRO infection. Conclusions:Early removal of endotracheal intubation and discharge from ICU are important to reduce the risk of MDRO infection after liver transplantation.

Objective:To investigate the MRI features of fibro-adipose vascular anomaly (FAVA).Methods:The clinical, pathological and MRI data of the FAVA patients confirmed clinically and pathologically in Henan Provincial People’s Hospital from January 2016 to June 2021 were retrospectively analyzed, including 17 females and 13 males, aged 4-53 (18.5±12.8) years.Through the analysis of the MRI images of FAVA performance, including the lesion area, shape, signal, the degree of reinforcement, inner structure, outer structure and other image characteristics, summarize the MRI features.Results:The lesions located at the lower leg (12 cases), thigh (12 cases), upper arm (4 cases), forearm (1 case) and trunk (1 case). Twenty-four case of lower limbs. All cases involved the muscular layer, including 21 cases of superficial muscle layer, 7 cases of deep muscle layer, and 2 cases of both. Twenty-three cases of superficial muscle layer. Most of the affected muscles were quadriceps femoris and gastrocnemius. Morphology: all lesions were intramuscular solid masses growing parallel to the long axis of the muscle. There were 11 cases of focal type, 11 cases of locally infiltrating type, and 8 cases of diffuse type. MRI findings: all lesions showed mixed signal. On T1WI, 28 cases showed cloud-like, band-like and patchy high signal on the background of medium and high signal. On T2WI-FS, all the 30 cases showed low to medium signal areas on the background of high signal, which were dendrimer, ribbon and cloud-like. The lesions showed moderate to obvious heterogeneous progressive enhancement. Twenty-seven lesions had different shapes of vascular shadow, and 28 lesions had drainage vein shadow adjacent to the lesions, 24 of which were located at the proximal end of the lesions. Fascial tail sign was found at the periphery of the lesions in 26 cases, of which 23 cases were located at the upper and lower ends of the lesions.Conclusion:FAVA is a complex vascular malformation with unclear pathological classification. MRI usually shows a solid mass in the superficial muscle layer of the lower limbs parallel to the long axis of the muscle, with cloud-like, band-like, and patchy hyperintensity on the background of high signal on T1WI, and dendritic, band-like, and cloud-like hypointensity on the background of high signal on T2WI-FS, which is helpful for the diagnosis of FAVA. Combined with the data of fascial tail sign, draining vein and clinical manifestations, the diagnosis of FAVA can be confirmed to a certain extent, which can provide reference and basis for clinical decision making.

Kaposiform lymphangiomatosis(KLA)is an aggressive subtype of generalized lymphatic anomaly. It is difficult to diagnose because of the lack of specificity of early symptoms. Moreover, there is still a lack of effective treatment, so the prognosis of this disease is generally poor, with a 5-year survival rate of 51% and an overall survival rate of only 34%. This article reviews the progress of the genetic studies related to the development of KLA and summarizes potential drug targets. A review of the literature found that the mutations potentially associated with KLA pathogenesis included NRAS gene mutations and CBL gene mutations. The signaling pathways involved were the PI3K-AKT-mTOR pathway and the RAS-MAPK-ERK pathway. Therefore, the key molecules PI3K/AKT/mTOR and RAS/MEK in the above signaling pathway may be used as targets for KLA-targeted therapy and develop specific treatments.

Objective:To investigate the MRI features of fibro-adipose vascular anomaly (FAVA).Methods:The clinical, pathological and MRI data of the FAVA patients confirmed clinically and pathologically in Henan Provincial People’s Hospital from January 2016 to June 2021 were retrospectively analyzed, including 17 females and 13 males, aged 4-53 (18.5±12.8) years.Through the analysis of the MRI images of FAVA performance, including the lesion area, shape, signal, the degree of reinforcement, inner structure, outer structure and other image characteristics, summarize the MRI features.Results:The lesions located at the lower leg (12 cases), thigh (12 cases), upper arm (4 cases), forearm (1 case) and trunk (1 case). Twenty-four case of lower limbs. All cases involved the muscular layer, including 21 cases of superficial muscle layer, 7 cases of deep muscle layer, and 2 cases of both. Twenty-three cases of superficial muscle layer. Most of the affected muscles were quadriceps femoris and gastrocnemius. Morphology: all lesions were intramuscular solid masses growing parallel to the long axis of the muscle. There were 11 cases of focal type, 11 cases of locally infiltrating type, and 8 cases of diffuse type. MRI findings: all lesions showed mixed signal. On T1WI, 28 cases showed cloud-like, band-like and patchy high signal on the background of medium and high signal. On T2WI-FS, all the 30 cases showed low to medium signal areas on the background of high signal, which were dendrimer, ribbon and cloud-like. The lesions showed moderate to obvious heterogeneous progressive enhancement. Twenty-seven lesions had different shapes of vascular shadow, and 28 lesions had drainage vein shadow adjacent to the lesions, 24 of which were located at the proximal end of the lesions. Fascial tail sign was found at the periphery of the lesions in 26 cases, of which 23 cases were located at the upper and lower ends of the lesions.Conclusion:FAVA is a complex vascular malformation with unclear pathological classification. MRI usually shows a solid mass in the superficial muscle layer of the lower limbs parallel to the long axis of the muscle, with cloud-like, band-like, and patchy hyperintensity on the background of high signal on T1WI, and dendritic, band-like, and cloud-like hypointensity on the background of high signal on T2WI-FS, which is helpful for the diagnosis of FAVA. Combined with the data of fascial tail sign, draining vein and clinical manifestations, the diagnosis of FAVA can be confirmed to a certain extent, which can provide reference and basis for clinical decision making.

Kaposiform lymphangiomatosis(KLA)is an aggressive subtype of generalized lymphatic anomaly. It is difficult to diagnose because of the lack of specificity of early symptoms. Moreover, there is still a lack of effective treatment, so the prognosis of this disease is generally poor, with a 5-year survival rate of 51% and an overall survival rate of only 34%. This article reviews the progress of the genetic studies related to the development of KLA and summarizes potential drug targets. A review of the literature found that the mutations potentially associated with KLA pathogenesis included NRAS gene mutations and CBL gene mutations. The signaling pathways involved were the PI3K-AKT-mTOR pathway and the RAS-MAPK-ERK pathway. Therefore, the key molecules PI3K/AKT/mTOR and RAS/MEK in the above signaling pathway may be used as targets for KLA-targeted therapy and develop specific treatments.

Objective:To investigate the differential performance of ultrasound between fibro-adipose vascular anomaly (FAVA) and venous malformations(VMs).Methods:From January 2015 to December 2020, the patients diagnosed with lower extremity FAVA by pathology in Henan Provincial People’s Hospital were enrolled as FAVA group. The patients diagnosed with lower extremity VMs by pathology were enrolled as the control group. The clinical and ultrasound imaging data were retrospectively analyzed. Through the single factor analysis of the two groups’data, the ultrasonic imaging indicators which may be valuable for distinguishing FAVA from VMs were screened. Further, the indicators valuable for differential diagnosis were determined by multi-factor Logistic regression analysis, and a multi-factor joint diagnosis model was constructed. The diagnostic efficiency of the joint diagnosis model was evaluated by the receiver operator characteristic curve (ROC curve), sensitivity, and specificity of the subjects.Results:A total of 20 patients with FAVA were involved, including 11 males and 9 females. The mean age was (18.1±12.2) years. Forty-six patients with VMs were involved, including 20 males and 26 females. The mean age was (19.9±13.6) years. Results of the single-factor analysis were differences in the lesion echo, fascial tail, blood flow, extrusion test, and posterior echo enhance characteristics between groups ( P<0.05). Multivariate analysis showed significant differences between groups in three aspects: fascial tail, extrusion test, and posterior echo enhancement ( P=0.001, 0.008, 0.007). The sensitivity and specificity of the multi-factor combined diagnosis model were 90.0% (95% CI: 68.3%-98.8%) and 93.5%(95% CI: 82.1%-98.6%), indicating high diagnostic efficiency. The ROC(AUC) area was 0.964(95% CI: 0.886-0.994), indicating high diagnostic efficiency. Conclusions:The ultrasonic imaging features of FAVA and VMs were different. The combined diagnosis of the fascial tail, compression test, and posterior echo enhancement has a higher auxiliary diagnostic value.

Objective:This study aims to discuss the clinical effect of surgical treatment of vascular anomalies in the sciatic nerve region.Methods:Retrospective analysis of clinical data was performed on patients with pain and dysfunction in the sciatic nerve region in the Department of Hemangioma, Henan Provincial People’s Hospital, between July 2013 and December 2018. The operation time, intraoperative blood loss, hospitalization time, postoperative complications, and wound healing were recorded. Postoperative follow-up included physical and imaging examination. Visual analogue scale (VAS) was used to evaluate the pain intensity before operation and at discharge. Oswestry dysfunction index (ODI) was used to evaluate the pain and limb function before operation and during follow-up.Results:This study enrolled 76 cases (32 males, aged from 2 to 55 years ), including 48 cases of venous malformation (VM), 9 cases of arteriovenous malformation (AVM), 6 cases of lymphatic malformation (LM), and 13 cases of fibro-adipose vascular anomaly (FAVA). Thirteen patients had been treated with local sclerotherapy, and four patients had been treated with interventional embolization for AVM without improvement or exacerbation. The operative time, intraoperative blood loss, and hospitalization time of FAVA patients were (113.33±30.11) min, (81.67±22.29) ml, and (3.83±0.98) d respectively, which were less than those of the other three vascular anomalies with the lest surgical damage and quickest recovery. The operative time, intraoperative blood loss, and hospitalization time of AVM patients were (288.33±33.71) min, (981.67±164.85) ml, and (11.17±1.47) d respectively, which were more than those of the other three vascular anomalies. The operation time of VM and LM cases was close, but the amount of blood loss [(396.67±85.71) ml] in VM cases varies greatly, with a minimum of 150 ml and a maximum of 1 100 ml. The vital signs of all patients were stable during and after the operation, without injury of main vessels and nerves. Seventy-one patients had grade A wound healing, four patients had grade B wound healing, and one patient had grade C wound healing. The postoperative follow-up was 6-36 months (mean 13 months), and the pain was completely relieved in 73 cases and partially relieved in 3 cases at the last follow-up. Seventy-two cases returned to normal, and 4 cases improved with lower limb dysfunction. Imaging examination showed that lesions of 70 cases disappeared and of 6 cases were residual. Preoperative VAS scores of four groups were 2.00 (1.00, 3.75), 2.00 (1.00, 3.50), 4.00 (0.75, 6.25), 4.00 (1.50, 6.00) respectively, and the postoperative VAS scores were all 0.00 (0.00, 0.00). The preoperative ODI scores of four groups were 17.78% (15.56%, 22.22%), 17.78% (13.33%, 35.56%), 50.00% (31.67%, 84.44%), 42.22% (31.11%, 56.67%). Apart from LM [4.44% (0.00%, 22.22%)], the postoperative ODI of the others was all 0.00% (0.00%, 0.00%). VAS and ODI were statistically significant differences before and after surgical treatment among all four groups ( P<0.05). Conclusions:Surgical treatment is safe and effective for vascular anomalies in the sciatic nerve region, which can significantly improve pain and joint mobility disorders.