Lymphangioma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Pathogenesis
- Lymphangioma arises from lymphatic vessels, which are a vital part of the circulatory and immune system, where they are normally involved in re-circulation of excess body fluid back into the blood stream.
- Lymphangioma is a common benign tumor that often grows proportionally 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.
- The progression to lymphangioma usually involves the following growth factors:
- VEGF-C
- VEGFR-3
- Prox-1
- bFGF
- PEDF
- Thrombospondin
- Reelin
- cMAF
- Integrin-α1, -α9
- Lymphangiomas most commonly develop at the head and neck regions.
- However, lymphangioma may also develop in other anatomical sites such as:
- Breast
- Upper and lower limbs
- Internal visceral organs
Genetics
- Genetic mutations involved in the pathogenesis of lymphangioma include:
- Trisomy 13
- Tirsomy 18
- Trisomy 21
Associated Conditions
- Lymphangioma is associated with a number of conditions that include:
- Turner syndrome
- Noonan syndrome
- Down syndrome
Gross Pathology
- On gross pathology, characteristic findings of lymphangioma include:
- Grey-white mass
- Edematous appearance
- Variable size (may be masive)
- 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
