Abstract:
:Pediatric hepatic angiosarcoma is a very rare malignant vascular tumor. A few cases have shown pediatric hepatic angiosarcoma occurring on a background of preexisting vascular lesions. We report the case of a newborn girl who presented extensive limbs and upper trunk cutaneous mixed vascular malformations at birth. These malformations were associated with thrombocytopenia. Cutaneous biopsies revealed complex vascular malformations with a significant lymphatic component. Compressive body suit therapy led to regression of the limbs' cutaneous vascular malformations. At the age of 9 months, the patient presented multiple heterogeneous hepatosplenic nodules. Aggressive treatment with prednisone, vincristine, and hepatosplenic embolizations resulted in initial improvement of the hepatosplenic lesions for few months, followed by an increase of the lesions with failure of response to treatment despite adding alpha-interferon-2b to treatment. The patient died at the age of 19 months. The autopsy's pathological examination revealed a hepatic-based angiosarcoma with plurimetastatic dissemination to the spleen, lungs, peritoneum, pleura, mesenteric linings as well as the serosa of the stomach and small intestine. Multiple cutaneous and visceral complex capillaro-lymphatico-venous malformations were also identified. We hypothesize that these multiple extensive mixed vascular malformations were associated with chronic lymphedema which probably predisposed to the development of the angiosarcoma in our patient.
journal_name
Lymphat Res Bioljournal_title
Lymphatic research and biologyauthors
Al Dhaybi R,Agoumi M,Powell J,Dubois J,Kokta Vdoi
10.1089/lrb.2009.0034subject
Has Abstractpub_date
2010-09-01 00:00:00pages
175-9issue
3eissn
1539-6851issn
1557-8585journal_volume
8pub_type
杂志文章abstract::Hemangiomas of infancy are very common tumors, but they are heterogeneous in their behavior. A small, but significant, subset causes medical complications or permanent disfigurement, but due to their heterogeneity, there is no appropriate "one size fits all" approach to management. In addition, the rapid evolution of ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/153968503322758120
更新日期:2003-01-01 00:00:00
abstract::The knowledge of humoral and cellular factors retained in tissues affected by ineffective lymph transport is necessary to comprehend the pathological mechanisms of the evolving changes in the extremity. In this article, we report our observations on changes in the protein composition of lymph in obstructive lymphedema...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/153968503768330265
更新日期:2003-01-01 00:00:00
abstract:BACKGROUND:Primary idiopathic lymphedema is an uncommon condition that typically affects the lower extremities. Patients have a malformed lymphatic system that causes subcutaneous fluid and adipose deposition. Rarely, the disease also has been described in the upper extremities. The purpose of this study was to investi...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0085
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:Studies of lymphatic function often employ collecting lymphatic vessels that exhibit large-amplitude, spontaneous contractions. Data from such preparations have been analyzed using cardiac pump analogies that require accurate determination of vascular dimensions, including external (OD) and internal (ID) dia...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2006.4.3
更新日期:2006-04-01 00:00:00
abstract:: Background: The Lymphedema Functioning, Disability, and Health questionnaire for Upper Limb Lymphedema (Lymph-ICF-UL) is a health-related quality-of-life questionnaire for patients with breast cancer-related lymphedema. Previous testing of this questionnaire showed very go...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2019.0073
更新日期:2020-08-01 00:00:00
abstract:BACKGROUND:Previous studies suggest that rhythmic compression of the abdomen (abdominal lymphatic pump techniques, LPT) enhances immunity and resistance to infectious disease, but direct evidence of this has not been documented. In this study, the thoracic duct of eight anesthetized mongrel dogs was catheterized, so th...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2007.1001
更新日期:2007-01-01 00:00:00
abstract:BACKGROUND:Bioimpedance spectroscopy (BIS) is commonly used in the assessment and monitoring of lymphedema. This study investigated electrodes as a source of variability that could impact the accuracy of BIS in the clinic and determined if Ag/AgCl electrocardiograph (ECG) electrodes could be used as an alternative to i...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0002
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:Compared to volume/circumference measurements to diagnose lymphedema, thickening and disturbance of echogenicity of cutis and subcutis associated with the development of lymphedema is measured directly by ultrasonography. The aim of the present study was to investigate the evolution of thickness and echogeni...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2013.0028
更新日期:2014-03-01 00:00:00
abstract:BACKGROUND:Lymphangiomatosis is a rare disorder of the lymphatic system that can impact the dermis, soft tissue, bone, and viscera and can be characterized by lymphangiomas, swelling, and chylous discharge. Whether disordered lymphangiogenesis in lymphangiomatosis affects the function and anatomy of the entire systemic...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2015.0013
更新日期:2015-09-01 00:00:00
abstract:BACKGROUND:Tissue dielectric constant (TDC) and spot bioimpedance measurement (BIA) have a role in the assessment of tissue composition changes in breast cancer-related lymphedema (BCRL). Our aims were to determine whether TDC and spot BIA measures could detect inter-limb differences in BCRL, explore the relationship b...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2014.0032
更新日期:2015-03-01 00:00:00
abstract::Exciting studies involving the molecular regulation of lymphangiogenesis in lymphatic-associated disorders (e.g., wound healing, lymphedema and tumor metastasis) have focused renewed attention on the intrinsic relationship between lymphatic endothelial cells (LECs) and extracellular matrix (ECM) microenvironment. ECM ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/lrb.2006.4.83
更新日期:2006-01-01 00:00:00
abstract:: Background: Bioimpedance spectroscopy detects unilateral lymphedema if the ratio of extracellular fluid (ECF) between arms or between legs is outside three standard deviations (SDs) of the normative mean. Detection of bilateral lymphedema, common after bilateral breast or ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0082
更新日期:2018-12-01 00:00:00
abstract::The present report describes a case with the triad of yellow nail syndrome (YNS) and the use of low-pressure compression pump as treatment of lymphedema in YNS. A 71-year-old woman presented with bilateral lower extremity lymphedema, yellow nails, and recurrent bilateral pleural effusion. In this case, we specifically...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0012
更新日期:2012-03-01 00:00:00
abstract:BACKGROUND:Analysis of the innervation pattern of the thoracic duct in young and elderly human subjects has been performed. The subdivision of the vessels in cervical and lumbar region were taken in consideration. METHODS AND RESULTS:Immunostaining for general nerve fibers with a PGP 9.5 marker disclosed a diffuse inn...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0014
更新日期:2012-06-01 00:00:00
abstract:BACKGROUND:Lymph flow depends on both the rate of lymph production by tissues and the extent of passive and active pumping. Here we aim to characterize the passive mechanical properties of a lymphangion in both mid-lymphangion and valve segments to assess regional differences along a lymphangion, as well as evaluating ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0015
更新日期:2012-12-01 00:00:00
abstract::Despite the fact that the central nervous system parenchyma does not contain lymphatics, extracranial lymphatic vessels play a very important role in volumetric cerebrospinal fluid (CSF) transport. The most important extracranial location at which lymphatics gain access to CSF is in the nasal submucosa after CSF conve...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/15396850360495682
更新日期:2003-01-01 00:00:00
abstract:BACKGROUND:Changes in arm soft tissue composition, especially increased adipose tissue, has been found in advanced, non-pitting breast cancer-related lymphedema (BCRL). The aim of this study was to examine whether these changes were localized to any particular region of the arm and whether they occurred in lymphedema w...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2014.0033
更新日期:2015-03-01 00:00:00
abstract:BACKGROUND:Corneal lymphangiogenesis (LG) and hemangiogenesis (HG) accompany many diseases after inflammatory, infectious, traumatic or chemical insults. They also contribute to transplant rejection. It is known that corneal transplants in infants or children have a higher rejection rate than in adults. However, it has...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2011.0005
更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:Lymphedema leads to adipose tissue deposition. Water-fat magnetic resonance imaging (MRI) can quantify and localize fat and water. The presence of excess fat and excess water/muscle in the subfascial compartment of the lymphedematous limb has not been investigated before. The aim of this study was to investi...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0042
更新日期:2018-04-01 00:00:00
abstract:OBJECTIVE:The aim of the article was to assess risk factors and to analyze methods applied in the prevention and treatment of lower limb edema in pregnant women with a particular focus on compression therapy and exercise. MATERIALS AND METHODS:Fifty-four women during the early 24-hour period following delivery were as...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2016.0038
更新日期:2017-06-01 00:00:00
abstract:: Background: The use of bioimpedance spectroscopy (BIS) for the detection of unilateral upper limb lymphedema is growing. Currently, normatively determined diagnostic thresholds using bioimpedance are available for females only. It is unclear if they are suitable for males ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0051
更新日期:2020-02-01 00:00:00
abstract:BACKGROUND:The purpose of this study is to investigate the effects of the aqua-lymphatic therapy (ALT) on unilateral lower extremity lymphedema in the maintenance phase. MATERIALS AND METHODS:This is a randomized controlled trial with a blinded assessor. The study was completed with 30 ALT and 27 control group partici...
journal_title:Lymphatic research and biology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1089/lrb.2017.0017
更新日期:2017-09-01 00:00:00
abstract:BACKGROUND:Lymphedema of limbs affects a large mass of tissues. Pathological changes develop in skin and subcutaneous tissue. Bacterial retention in edema fluid is followed by chronic inflammatory reaction. The question arises whether the chronic processes affecting a large mass of limb tissues are reflected in the ser...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2017.0003
更新日期:2017-09-01 00:00:00
abstract:BACKGROUND:In arm lymphedema secondary to axillary surgery and radiotherapy (breast cancer-related lymphedema), the swelling is largely epifascial and lymph flow per unit epifascial volume is impaired. The subfascial muscle compartment is not measurably swollen despite the iatrogenic damage to its axillary drainage pat...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/153968503321642615
更新日期:2003-01-01 00:00:00
abstract:: Background: Conservative nonsurgical treatment has traditionally been applied to lymphedema. Early surgical procedures were invasive and disfiguring and their long-term success was often limited. In contrast, contemporary surgical techniques are much less invasive and have...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2018.0038
更新日期:2019-08-01 00:00:00
abstract:: Background: Surgical excision and/or radiation targeting of regional lymph nodes are an essential component in the clinical management of cancer. Importantly, a more accurate understanding of lymphatic anatomy could enable refinement of present treatment strategies. Given ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2019.0007
更新日期:2020-04-01 00:00:00
abstract:BACKGROUND:Near-infrared fluorescence (NIRF) imaging is a new imaging technique that is used to visualize lymphatic vessels in humans. It has a high spatial and temporal resolution, allowing real-time visualization of lymphatic flow. METHODS AND RESULTS:The current study investigated the intra- and inter-individual va...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2016.0061
更新日期:2017-09-01 00:00:00
abstract::The vasculature of the central nervous system (CNS) is highly specialized with a blood-brain-barrier, reciprocal neuroepithelial-endothelial cell interactions and extensive pericyte coverage. Developmentally, numerous important signaling pathways participate in CNS angiogenesis to orchestrate the precise timing and sp...
journal_title:Lymphatic research and biology
pub_type: 杂志文章,评审
doi:10.1089/lrb.2008.1014
更新日期:2008-01-01 00:00:00
abstract::Abstracts Background: Arm lymphedema after breast cancer surgery affects women both from physical and psychological points of view. Lymphedema leads to adipose tissue deposition. Liposuction and controlled compression therapy (CCT) reduces the lymphedema completely. METHODS AND RESULTS:Sixty female patients with arm ...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2016.0035
更新日期:2017-03-01 00:00:00
abstract:BACKGROUND:We performed experiments to test whether isolated lymphatic vessels would remain viable after overnight long-distance shipment. METHODS AND RESULTS:Freshly isolated segments of rat mesenteric lymphatic vessels were placed into tubes filled by chilled D-MEM/F12 supplemented with antibiotics and shipped via o...
journal_title:Lymphatic research and biology
pub_type: 杂志文章
doi:10.1089/lrb.2010.0007
更新日期:2010-12-01 00:00:00