Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Tuesday, February 26, 2008

Overexpression of VEGF-C causes transient lymphatic hyperplasia but not increased lymphangiogenesis in regenerating skin

Overexpression of VEGF-C causes transient lymphatic hyperplasia (lymphedema) but not increased lymphangiogenesis in regenerating skin.

Goldman J, Le TX, Skobe M, Swartz MA. Biomedical Engineering Department, Northwestern University, Evanston, Ill, USA.


Correspondence to Dr Melody A. Swartz, Assistant Professor, Institute for Biological Engineering and Biotechnology, School of Life Sciences, Swiss Federal Institute of Technology Lausanne (EPFL), 1015 Lausanne, Switzerland. E-mail melody.swartz@epfl.ch

Vascular endothelial growth factor (VEGF)-C is necessary for lymphangiogenesis and holds potential for lymphangiogenic therapy in diseases lacking adequate lymphatic drainage. However, the ability of VEGF-C to enhance sustainable, functional lymphatic growth in adult tissues remains unclear. To address this, we evaluated VEGF-C overexpression in adult lymphangiogenesis in regenerating skin. We used a model of mouse tail skin regeneration incorporating a suspension of either VEGF-C overexpressing tumor cells, which provide a continuous supplement of excess VEGF-C to the natural regenerating environment for more than 25 days, or otherwise identical control- transfected tumor cells. We found that excess VEGF-C did not enhance the rate of lymphatic endothelial cell (LEC) migration, the density of lymphatic vessels, or the rate of functionality -- even though lymphatic hyperplasia was present early on. Furthermore, the hyperplasia disappeared when VEGF-C levels diminished, which occurred after 25 days, rendering the lymphatics indistinguishable from those in control groups. In vitro, we showed that whereas cell-derived VEGF- C could induce chemoattraction of LECs across a membrane (which involves amoeboid-like transmigration), it did not increase LEC chemoinvasion within a 3-dimensional fibrin matrix (which requires proteolytic migration). These results suggest that whereas excess VEGF- C may enhance early LEC proliferation and cause lymphatic vessel hyperplasia, it does not augment the physiological rate of migration or functionality, and by itself cannot sustain any lasting effects on lymphatic size, density, or organization in regenerating adult skin.

AHA Journals

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Saturday, February 23, 2008

Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities

Definitive percutaneous treatment of lymphatic malformations of the trunk and extremities

J Pediatr Surg. 2008 Jan

Shiels WE 2nd, Kenney BD, Caniano DA, Besner GE.
Department of Radiology, Nationwide Children's Hospital, The Ohio State University Medical Center, Columbus, OH 43205, USA.
william.shiels@nationwidechildrens.org

PURPOSE: The aim of this study is to investigate a new treatment regimen for macrocystic and microcystic lymphatic malformations (LMs) of the trunk and extremities.

METHODS: Sixteen patients (aged 2 months-22 years) underwent percutaneous treatment for LM of the trunk and extremities from 2002 to 2007. The LM involved the arm, leg, axilla, chest, abdomen, scrotum, and penis. Eleven patients underwent primary treatment of LM; 5 were treated for recurrence after prior operative resection. Macrocysts (>or=1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline was used for microcysts. Macrocysts and microcysts were treated after complete cyst aspiration using sonographic guidance. Twenty-four macrocysts and 103 microcysts were treated. The goal of treatment was complete cyst ablation documented by sonography or magnetic resonance imaging.

RESULTS: The mean number of treatments was 1.7 per patient; the mean number of treatments for macrocysts was 1.3 and for microcysts was 1.7. Ablation efficacy was 100% (127/127 cysts). Treatments included massive intraperitoneal cysts and cysts surrounding the adventitia of the brachial artery and brachial nerve. Infection occurred in 2 (13%) of 16 patients. No patient experienced postprocedural pain, skin necrosis, neuropathy, bowel obstruction, skin retraction, or myoglobinuria.

CONCLUSIONS: Percutaneous sclerotherapy provides effective treatment for macrocystic and microcystic LM as primary treatment or for recurrence after surgical resection.

Elsevier

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Monday, February 18, 2008

Congenital vascular malformations: general diagnostic principles.

Congenital vascular malformations: general diagnostic principles.
Phlebology. 2007

Lee BB, Laredo J, Lee SJ, Huh SH, Joe JH, Neville R.
Department of Surgery, Georgetown University, Washington, DC, USA.
bblee38@comcast.net


Venous malformation (VM) is the most common congenital vascular malformation (CVM), which usually presents as a single lesion in the majority of cases. It also presents as a mixed lesion combined with other CVMs (e.g. lymphatic malformation and arteriovenous malformation [AVM]). Therefore, the diagnosis of VM should include an appropriate work-up, to not only confirm and characterize the VM as either extratruncular or truncular but also to diagnose or exclude the presence of other CVMs. The diagnosis of VM can be made safely using non-invasive to minimally invasive studies, which can also distinguish VM from infantile haemangioma. Invasive studies, such as venography and arteriography, are generally reserved for therapeutic planning and diagnosis of more virulent CVMs (e.g. AVM). The work-up of VM should include a complete assessment of the extent and severity of the primary VM lesion. In addition, its embryologic origin, as well as its haemodynamic characteristics and secondary effects should also be determined.


PMID: 18274332 [PubMed - in process]

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Saturday, February 16, 2008

Laser excision of multiple esophageal lymphangiomas: A case report and review of the literature.

Laser excision of multiple esophageal lymphangiomas: A case report and review of the literature.
Auris Nasus Larynx. 2008 Feb 1

Best SR, Coelho DH, Ahrens WA, Atez G, Sasaki CT.
Yale University School of Medicine Division of Otolaryngology, Head & Neck Surgery, New Haven, CT, USA.


Lymphangiomas of the gastrointestinal tract are rare and benign submucosal tumors. We present the first case of multiple lymphangiomas in the cervical esophagus and only the 15th case of an esophageal lymphangioma. A 63-year-old male presented to our clinic with two years of dysphagia and an esophageal mass noted on MRI. A barium swallow confirmed a polypoid mass of the upper esophagus with operative findings of two smooth pedunculated lymphangiomas occupying the entire lumen of the esophagus. Previous reports have documented successful surgical intervention through open or endoscopic techniques, whereas the favorable location of the tumors in this case allowed for the first successful excision utilizing a CO(2) laser. We demonstrate here that esophageal laser surgery can provide an accurate, complete, and minimally invasive excision technique for lymphangiomas of the upper digestive tract with minimal morbidity for the patient. A complete review of all published cases is presented, with a focus on the clinical presentation and surgical treatment of this disease.


PMID: 18243618 [PubMed - as supplied by publisher]

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Sunday, February 03, 2008

Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer.

Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer.

World J Surg. 2007 Oct

Machens A, Hauptmann S, Dralle H.
Department of General, Visceral and Vascular Surgery, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str 40, D-06097, Halle/Saale, Germany.
AndreasMachens@aol.com

(1)
Department of General, Visceral and Vascular Surgery, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, D-06097 Halle/Saale, Germany
(2)
Department of Pathology, Martin-Luther-University Halle-Wittenberg, Magdeburger Str. 14, D-06097 Halle/Saale, Germany

BACKGROUND: In sporadic and hereditary medullary thyroid cancer, tumor multifocality may constitute an independent risk factor of lymph node metastasis on top of primary tumor size when the diameter of the largest primary tumor is the same. METHODS: Included in this institutional cohort study were 232 consecutive patients operated on at our institution for hitherto untreated medullary thyroid cancer. Associations of clinicopathologic variables with lymph node metastasis were investigated simultaneously using multivariate Cox regression analysis.

RESULTS: On univariate analysis, multifocal cancers developed lymph node metastases significantly more often (p < or =" 0.005)" p =" 0.01)." or =" 3.1;">

CONCLUSION: The excess risk of lymph node metastasis of 34%-49% in multifocal medullary thyroid cancer seems to be caused by concurrent smaller thyroid cancers. A diagnosis of more than one medullary thyroid cancer signifies a higher risk of lymph node metastasis, warranting systematic lymph node dissection.

Springer Link

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