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Related Concept Videos

Lymphatic Vessels and Lymph Transport01:16

Lymphatic Vessels and Lymph Transport

Lymphatic vessels, known as lymphatics, are crucial in transporting lymph from peripheral tissues to our venous system. This process begins with lymph entering through tiny capillaries that branch through tissues. These capillaries have unique features such as larger diameters, thinner walls, and a distinctive one-way valve system formed by overlapping endothelial cells.
This one-way system allows fluids, solutes, and even pathogens to enter but prevents their return to the intercellular spaces.
Development of the Lymphatic System01:15

Development of the Lymphatic System

The development of lymphatic tissues and vessels in embryonic life begins around the fifth week. These structures originate from the mesoderm layer, with lymph sacs emerging from developing veins.
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...

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Related Experiment Video

Updated: May 10, 2026

Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
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Lymphatic Malformation: Classification, Pathogenesis, and Therapeutic Strategies.

Chaonan Zhang1, Jing Liu2, Yao Wang2

  • 1School of Clinical Medicine, Shandong Second Medical University, Weifang, Shandong, P.R. China; Laboratory of Translational Medicine in Microvascular Regulation, Medical Research Center, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, Shandong, P.R. China; Shandong Provincial Key Medical and Health Laboratory of Translational Medicine in Microvascular Aging, Jinan, Shandong, P.R. China; Laboratory of Future Industry of Gene Editing in Vascular Endothelial Cells of Universities in Shandong Province, Jinan, Shandong, P.R. China.

Annals of Vascular Surgery
|May 25, 2025
PubMed
Summary
This summary is machine-generated.

Lymphatic malformations (LM) are lymphatic system tumors driven by genetic mutations affecting key signaling pathways like PI3K/AKT/mTOR and VEGF. Current treatments include mTOR inhibitors and VEGFR-3 inhibitors, offering new hope for patients.

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Area of Science:

  • Vascular Biology
  • Developmental Biology
  • Oncology

Background:

  • Lymphatic malformation (LM) is a congenital lymphatic system tumor arising from abnormal lymphatic endothelial cell (LEC) development.
  • LMs manifest as structural defects, abnormal proliferation, and cystic expansion of LECs, often in the head, neck, and axilla.
  • Understanding the molecular pathogenesis is crucial for developing targeted therapies.

Purpose of the Study:

  • To review the latest advancements in clinical manifestations, molecular pathogenesis, and treatment strategies for lymphatic malformations.
  • To consolidate current knowledge on genetic mutations and signaling pathways implicated in LM development.
  • To highlight emerging therapeutic targets and their clinical efficacy.

Main Methods:

  • Literature review of recent studies on lymphatic malformations.
  • Analysis of molecular mechanisms including genetic mutations and signaling pathway dysregulation (PI3K/AKT/mTOR, VEGF, Wnt/β-catenin).
  • Synthesis of information on clinical presentations and therapeutic interventions.

Main Results:

  • Mutations in PI3K subunits activate the PI3K/AKT/mTOR pathway, promoting LM LEC proliferation and lymphangiogenesis.
  • Vascular Endothelial Growth Factor C (VEGF-C) and its receptor VEGFR-3 play critical roles in LEC survival, proliferation, and migration.
  • Specific genetic alterations in Wnt/β-catenin pathway and asparaginyl-tRNA synthetase are linked to LM subtypes.
  • mTOR inhibitors (e.g., sirolimus) and VEGFR-3 inhibitors show promise in LM treatment.

Conclusions:

  • Lymphatic malformations result from complex molecular events involving dysregulated signaling pathways.
  • Targeted therapies, particularly mTOR and VEGFR-3 inhibitors, represent significant progress in LM management.
  • Further research into the precise molecular mechanisms will enable more personalized and effective treatment strategies.