目的分析肥厚型葡萄酒色斑(Port-wine stains,PWS)的影像学和病理学特点,以期为改善该病的治疗效果提供依据。方法对32例接受过激光治疗的肥厚型PWS患者的面部MRI资料进行分析,对其增厚程度和血管畸形程度进行评价分级;对其中18例患者...目的分析肥厚型葡萄酒色斑(Port-wine stains,PWS)的影像学和病理学特点,以期为改善该病的治疗效果提供依据。方法对32例接受过激光治疗的肥厚型PWS患者的面部MRI资料进行分析,对其增厚程度和血管畸形程度进行评价分级;对其中18例患者进行组织学检查,并与15例平坦型患者进行对比分析,探讨两种类型的血管组织学结构差异。结果 MRI资料的相关性分析证实,软组织厚度随畸形血管分布增多而增加(P<0.001)。组织学显示,肥厚型相较于平坦型PWS患者,其面部脂肪层和肌肉层的血管口径更大(0.048 mm vs 0.020 mm,P=0.004;0.035 mm vs 0.017 mm,P=0.008),并且管壁更厚(0.014 mm vs 0.006 mm,P=0.003;0.010 mm vs 0.006 mm,P=0.020)。结论肥厚型PWS患者激光疗效较差,与患者深部畸形血管明显超出现有激光对血管热凝固的破坏深度相关。展开更多
BACKGROUND Hemangioma is a vascular benign tumour of endothelial origin.It appears commonly in the first decade of life with increases incidence in females.Hemangioma is not common to happen in the oral cavity and it ...BACKGROUND Hemangioma is a vascular benign tumour of endothelial origin.It appears commonly in the first decade of life with increases incidence in females.Hemangioma is not common to happen in the oral cavity and it is extremely rare to appear in the labial vestibule.CASE SUMMARY We present a case of an 11-year-old girl who complained of a painful,slowly growing mass which was consistent with the capillary hemangioma in the left mandibular vestibule.Vascular tumor such as hemangioma in the mandibular vestibule is extremely rare;hence,the clinical definitive diagnosis is very challenging.Therefore,radiographic imaging and histopathologic analysis are crucial to reach to the final diagnosis for proper management.CONCLUSION Comprehensive clinical evaluation,proper diagnostic imaging and microscopic analysis of the mass establish a precise diagnosis of the hemangioma for better management.展开更多
Background:Port-wine stain(PWS)has been classified not as the hyperplasia of cells,but rather,as an expansion of malformed vessels.However,previous studies have reported upregulated expression of proangiogenic factors...Background:Port-wine stain(PWS)has been classified not as the hyperplasia of cells,but rather,as an expansion of malformed vessels.However,previous studies have reported upregulated expression of proangiogenic factors in PWS.Several studies have indicated that the pathology exhibits proliferation of numerous endothelial cells in hypertrophic/nodular PWS.This study aimed to determine the expression of vascular epithelial growth factor(VEGF),matrix metalloproteinase-9(MMP-9),angiopoietin-2(ANG-2),and basic fibroblast growth factor(bFGF)in hypertrophic PWS.Methods:Immunohistochemistry was used to analyze skin samples from 33 patients with hypertrophic PWS.Expression levels of VEGF,MMP-9,ANG-2,and bFGF in hypertrophic PWS were determined by multiplying the intensity by the percentage of immunoreactive cells.Immunoreactivity scores were classified as follows:negative(0),low(1),moderate(2,3,and 4),or high(6).Results:Based on pathological characteristics,hypertrophic PWS was divided into vascular malformation and pyogenic granuloma(PG)types.VEGF,MMP-9,ANG-2,and bFGF were significantly activated in the blood vessels of PG-type PWS samples compared with their counterparts in blood vessels of vascular malformation-type PWS samples and controls.PG-type hypertrophic PWS,which exhibited proliferation of endothelial cells,showed the strongest activation.Conclusion:The exuberant proliferation of endothelial cells in PG-type hypertrophic PWS may be associated with the regulation of proangiogenic factors during development.These proangiogenic factors that function in the angiogenesis and proliferation of endothelial cells may play an important role in the pathogenesis and progression of PWS.Furthermore,these factors may be dynamic and behave differently in various types of hypertrophic PWS.展开更多
Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary malformations.SWS usually presents as a facial port-win...Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary malformations.SWS usually presents as a facial port-wine birthmark,with a varying presence of leptomeningeal capillary malformations and ocular vascular abnormalities.The latter may lead to significant neurological and ocular morbidity such as epilepsy and glaucoma.SWS is most often caused by a somatic mutation involving the G protein subunit alpha Q or G protein subunit alpha 11 gene causing various alterations in downstream signaling pathways.We specifically conducted a comprehensive review focusing on the current knowledge of clinical practices,the latest pathophysiological insights,and the potential novel therapeutic avenues they provide.Data sources A narrative,non-systematic review of the literature was conducted,combining expert opinion with a balanced review of the available literature.A search of PubMed,Google Scholar and Embase was conducted,using keywords“Sturge–Weber Syndrome”OR“SWS”,“Capillary malformations”,“G protein subunit alpha 11”OR“G protein subunit alpha Q”.Results One of the hallmark features of SWS is the presence of a port-wine birthmark at birth,and forehead involvement is most indicative for SWS.The most common ocular manifestations of SWS are glaucoma and choroidal hemangioma.Glaucoma presents in either in infancy(0–3 years of age)or later in life.Neurological complications are common in SWS,occurring in about 70%–80%of patients,with seizures being the most common one.SWS significantly impacts the quality of life for patients and their families,and requires a multidisciplinary approach for diagnosis and treatment.Currently,no disease-modifying therapies exist,and treatment is mostly focused on symptoms or complications as they arise.Conclusions SWS remains a complex and heterogeneous disorder.Further research is needed to optimize diagnostic and therapeutic strategies,and to translate insights from molecular pathogenesis to clinical practice.展开更多
文摘目的分析肥厚型葡萄酒色斑(Port-wine stains,PWS)的影像学和病理学特点,以期为改善该病的治疗效果提供依据。方法对32例接受过激光治疗的肥厚型PWS患者的面部MRI资料进行分析,对其增厚程度和血管畸形程度进行评价分级;对其中18例患者进行组织学检查,并与15例平坦型患者进行对比分析,探讨两种类型的血管组织学结构差异。结果 MRI资料的相关性分析证实,软组织厚度随畸形血管分布增多而增加(P<0.001)。组织学显示,肥厚型相较于平坦型PWS患者,其面部脂肪层和肌肉层的血管口径更大(0.048 mm vs 0.020 mm,P=0.004;0.035 mm vs 0.017 mm,P=0.008),并且管壁更厚(0.014 mm vs 0.006 mm,P=0.003;0.010 mm vs 0.006 mm,P=0.020)。结论肥厚型PWS患者激光疗效较差,与患者深部畸形血管明显超出现有激光对血管热凝固的破坏深度相关。
基金Supported by the Deanship of Scientific Research at Princess Nourah Bint Abdulrahman University Through the Fast-Track Research Funding Program.
文摘BACKGROUND Hemangioma is a vascular benign tumour of endothelial origin.It appears commonly in the first decade of life with increases incidence in females.Hemangioma is not common to happen in the oral cavity and it is extremely rare to appear in the labial vestibule.CASE SUMMARY We present a case of an 11-year-old girl who complained of a painful,slowly growing mass which was consistent with the capillary hemangioma in the left mandibular vestibule.Vascular tumor such as hemangioma in the mandibular vestibule is extremely rare;hence,the clinical definitive diagnosis is very challenging.Therefore,radiographic imaging and histopathologic analysis are crucial to reach to the final diagnosis for proper management.CONCLUSION Comprehensive clinical evaluation,proper diagnostic imaging and microscopic analysis of the mass establish a precise diagnosis of the hemangioma for better management.
基金the National Natural Science Foundation of China(grant no.81602777).
文摘Background:Port-wine stain(PWS)has been classified not as the hyperplasia of cells,but rather,as an expansion of malformed vessels.However,previous studies have reported upregulated expression of proangiogenic factors in PWS.Several studies have indicated that the pathology exhibits proliferation of numerous endothelial cells in hypertrophic/nodular PWS.This study aimed to determine the expression of vascular epithelial growth factor(VEGF),matrix metalloproteinase-9(MMP-9),angiopoietin-2(ANG-2),and basic fibroblast growth factor(bFGF)in hypertrophic PWS.Methods:Immunohistochemistry was used to analyze skin samples from 33 patients with hypertrophic PWS.Expression levels of VEGF,MMP-9,ANG-2,and bFGF in hypertrophic PWS were determined by multiplying the intensity by the percentage of immunoreactive cells.Immunoreactivity scores were classified as follows:negative(0),low(1),moderate(2,3,and 4),or high(6).Results:Based on pathological characteristics,hypertrophic PWS was divided into vascular malformation and pyogenic granuloma(PG)types.VEGF,MMP-9,ANG-2,and bFGF were significantly activated in the blood vessels of PG-type PWS samples compared with their counterparts in blood vessels of vascular malformation-type PWS samples and controls.PG-type hypertrophic PWS,which exhibited proliferation of endothelial cells,showed the strongest activation.Conclusion:The exuberant proliferation of endothelial cells in PG-type hypertrophic PWS may be associated with the regulation of proangiogenic factors during development.These proangiogenic factors that function in the angiogenesis and proliferation of endothelial cells may play an important role in the pathogenesis and progression of PWS.Furthermore,these factors may be dynamic and behave differently in various types of hypertrophic PWS.
文摘Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary malformations.SWS usually presents as a facial port-wine birthmark,with a varying presence of leptomeningeal capillary malformations and ocular vascular abnormalities.The latter may lead to significant neurological and ocular morbidity such as epilepsy and glaucoma.SWS is most often caused by a somatic mutation involving the G protein subunit alpha Q or G protein subunit alpha 11 gene causing various alterations in downstream signaling pathways.We specifically conducted a comprehensive review focusing on the current knowledge of clinical practices,the latest pathophysiological insights,and the potential novel therapeutic avenues they provide.Data sources A narrative,non-systematic review of the literature was conducted,combining expert opinion with a balanced review of the available literature.A search of PubMed,Google Scholar and Embase was conducted,using keywords“Sturge–Weber Syndrome”OR“SWS”,“Capillary malformations”,“G protein subunit alpha 11”OR“G protein subunit alpha Q”.Results One of the hallmark features of SWS is the presence of a port-wine birthmark at birth,and forehead involvement is most indicative for SWS.The most common ocular manifestations of SWS are glaucoma and choroidal hemangioma.Glaucoma presents in either in infancy(0–3 years of age)or later in life.Neurological complications are common in SWS,occurring in about 70%–80%of patients,with seizures being the most common one.SWS significantly impacts the quality of life for patients and their families,and requires a multidisciplinary approach for diagnosis and treatment.Currently,no disease-modifying therapies exist,and treatment is mostly focused on symptoms or complications as they arise.Conclusions SWS remains a complex and heterogeneous disorder.Further research is needed to optimize diagnostic and therapeutic strategies,and to translate insights from molecular pathogenesis to clinical practice.
