Contents
Benign hamartomatous tumors of the lymphatic channels which present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels.
- Represent 4% of all vascular tumors and ~25% of all benign pediatric vascular tumor
Classification
Primary lymphangioma:
Congenital forms typically occurs before the age of 5 years and is due to improper connection of lymphatic channels to the main lymphatic drainage duct.
- Cavernous lymphangiomas (deep)
- Cystic hygromas (deep)
- Lymphangioma circumscriptum (superficial): Lymphatic channel dilation due to a congenital malformation of the lymphatic system
Secondary lymphangioma:
Acquired lymphangiomas occur as a sequela of any interruption of previously normal lymphatic drainage such as surgery, trauma, malignancy, and radiation therapy.
- Lymphangiectasia, or acquired lymphangioma: Dilated lymphatic channels of previously normal lymphatics that have become obstructed by an external cause
Histopathologically classification:
- Lymphangioma simplex or capillary lymphangioma Consisting of small, thin-walled capillary-sized lymphatics
- Cavernous lymphangioma: Comprising large, dilated lymphatics with surrounding adventitia
- Cystic lymphangioma/hygrmoa: Huge, macroscopic lymphatic spaces surrounded by fibrovascular tissue, and smooth muscle
- Benign lymphangioendothelioma: Lymphatic channels appears to be dissecting through dense collagenic bundles
Clinical features
Lymphangioma circumscriptum:
Multiple, grouped or scattered, translucent or hemorrhagic vesicular papules that resemble frog-spawn. Because the lesions consist of a combination of blood and lymph elements, purple areas can be seen scattered within the vesicle-like papules. In the genital area, the surface can be verrucous, and the lesions may be mistaken for warts.
Lymphangioma circumscriptum: Clusters of vesicles of varying size with clear to hemorrhagic fluid are seen over the right lower abdomen and buttock | Kudur, M. H., & Hulmani, M. (2013). Extensive and invasive lymphangioma circumscriptum in a young female: A rare case report and review of the literature. Indian dermatology online journal, 4(3), 199–201. https://doi.org/10.4103/2229-5178.115516 
Tiny vesicles on the left side of the scrotum | Omprakash Hm, & Rajendran Sc (2008). Lymphangioma circumscriptum (microcystic lymphatic malformation): palliative coagulation using radiofrequency current. Journal of cutaneous and aesthetic surgery, 1(2), 85–88. https://doi.org/10.4103/0974-2077.44165
Lymphangiectasia, or acquired lymphangioma:
Most often found in the axilla, inguinal, and genital areas and there is often coexisting lymphedema. Associated symptoms may include pruritus, pain, burning, lymphatic drainage, infection, and aesthetic concerns.
Cavernous lymphangioma:
Typically presents during infancy as a painless, ill-defined subcutaneous swelling with no changes of the overlying skin that can be several centimeters in size.
Cystic hygromas:
Lymphatic malformations that are clinically more circumscribed than cavernous lymphangioma and typically occur on the neck, axilla, or groin. On physical examination, they are soft, with varying sizes and shapes, and will typically grow if not surgically excised.
Management
- Surgical excision: Preferred treatment when vital structures are not involved
- Other procedures: Laser therapy, sclerotherapy, radiation therapy, electrocautery, cryotherapy, ligation, embolization and steroid