Lymphangioma pathophysiology
|
Lymphangioma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Lymphangioma pathophysiology On the Web |
|
American Roentgen Ray Society Images of Lymphangioma pathophysiology |
|
Risk calculators and risk factors for Lymphangioma pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Lymphangioma arises from lymph vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream. The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels. Lymphangiomas most commonly develop at the head and neck regions. Lymphangioma is associated with a number of conditions that include Turner syndrome and Down syndrome. On gross pathology, characteristic findings of lymphangioma include a grey-white, well circumscribed, edematous mass with a variable size and consistency. On microscopic histopathological analysis, characteristic findings of lymphangioma include thin walled endothelial lining, intraluminal accumulation of eosinophilic deposits, and clusters of intraluminal lymphocytes.
Pathogenesis
Lymphangioma arises from lymph vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream.[5] The exact mechanism of development of abnormal malformations is not known. It is suggested that lymphangioma is formed as a result of one of four mechanisms: Sequestration of lymph tissue Abnormal budding of lymph vessels Lack of fusion with the venous system Obstruction of lymph vessels
Whimster describes pathogenesis of lymphangioma as the collection of lymphatic cisterns in the deep subcutaneous plane which get detached from normal network, however they keep connected to superficial lymph vessels through short and dilated vessels.[6]
The cisterns originally derive from primitive lymph sac, which fail to connect to normal circulation. These are covered with thick muscular coat, which on contraction increase intramural pressure. Ultimately leads to dilated sacs which are visible on skin as out-pouchings.[7] Whimster's description is proved with radio-graphic evidence as well.[8] Which showed large multilobulated cisterns extend deep in the dermis and laterally beyond the clinical lesions. These deep lymphangiomas had no connections with the adjacent normal lymphatics. Some lymphangiomas may represent vascular malformations during embryonic development rather than as true neoplasms. For more information on Vascular anamolies. Click here. Vascular endothelial growth factor (VEGF)–C and VEGF receptor-3 play a key role in development of lymphangiomas.[9]
- The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels.
- The progression to lymphangioma usually involves the following growth factors:
- VEGF-C
- VEGFR-3
- Prox-1
- bFGF
- PEDF
- Thrombospondin
- Reelin
- cMAF
- Integrin-α1
- Integrin-α9
Acquired lymphangiomas [10] Acquired lymphangioms arise as a cosequence of any interruption of previously normal lymphatic drainage such as surgery[11], trauma, Infection, malignancy, and radiation therapy.[10]
- Lymphangioma arises from lymphatic vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream.
- Lymphangioma is a common benign tumor that often grows in proportion to the patients body growth rate.
- The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels.
Genetics
- Genetic mutations involved in the pathogenesis of lymphangioma include:
Associated Conditions
- Lymphangioma is associated with a number of conditions that include:
Gross Pathology
- On gross pathology, characteristic findings of lymphangioma include:
- Grey-white mass
- Well circumscribed
- Edematous appearance
- Variable size (may be massive)
- Filled with serous fluid
- Smooth inner lining
Microscopic Pathology
- On microscopic histopathological analysis, characteristic findings of lymphangioma include:
- Thin walled channels lined by endothelium
- Intraluminal accumulation of eosinophilic deposits
- Clusters of intraluminal lymphocytes
- On immunohistochemistry, characteristic findings of lymphangioma include:
- D2-40 +ve
