Lymphangioma circumscriptum

The clinical manifestations of congenital and acquired lymphangiomas are not different. They appear as small (from a few millimeters to 1-2 centimeters) raised cystic formations with the color of normal skin or reddish-brown (due to the presence of blood), grouped or arranged linearly, often against a slightly firm edematous background. The localization can be anywhere, but more commonly affects proximal parts of the limbs, neck, less commonly the genitalia, oral mucosa and periorbital area. Cases with zosteriform arrangement of elements, unilateral involvement of the abdomen, buttocks and thighs have been described. Classical lymphangiomas are usually not associated with subjective sensations.

Congenital malformations tend to progress slowly, especially during the first 2-5 years of life, with stabilization of growth occurring in the post-pubertal period. This tendency is also observed in adults with secondary lymphangiomas. Spontaneous regression is extremely rare.

Vulvar lymphangioma circumscriptum

Vulvar lymphangioma circumscriptum have distinct characteristics. They often appear as a solid lesion on both labia majora or on one side, or as separate grouped elements. Their surface is often verrucous with outflow of lymphatic fluid. Sometimes these elements are interspersed with papulovesicular ones. It's not uncommon to experience itching, pain, swelling, cellulitis and sexual dysfunction.

Oral Lymphangioma circumscriptum

In the oral cavity, capillary lymphangiomas most frequently appear on the dorsal surface of the tongue, less commonly on the buccal mucosa, soft palate, lips, gums, and under the tongue. They are translucent, filled with yellowish-red fluid, and often associated with unilateral facial swelling. Tongue lymphangiomas are the most common cause of macroglossia (enlarged tongue) in children. They can lead to difficulties in chewing, swallowing, and speech.

Cavernous Lymphangioma

Large solitary or multiple tumors on a swollen, firm base. Papillomatosis and hyperkeratosis may develop on the surface. They may occur anywhere on the body. Diffuse involvement of the lower extremities is also possible.

Cystic Lymphangioma

Present as nodules over 2 cm in diameter, sometimes reaching gigantic sizes up to 12-20 cm, elevated above the surface, single or multiple. The nodules are soft in consistency, fluctuant, not adherent to deep tissues, sometimes painful, with a color matching the surrounding skin, occasionally showing a bluish tint due to fluid shining through thin tissues. They are most commonly located in the head and neck area, less commonly in the axillary folds, mediastinum, groin, and lumbosacral regions. Occurrence on the extremities is very rare. When localized in the neck, they can displace and compress the carotid arteries and jugular veins, and when localized in the mediastinum, they can compress the esophagus and trachea. They can be complicated by infection, spontaneous hemorrhage, and rupture leading to fistula formation.

Diagnosis is based on clinical presentation and results of histological examination.

• Lymphangiosarcoma
• Kaposi Sarcoma
• Angiokeratoma
• Herpes Zoster
• Morphea
• Congenital elephantiasis
• Leiomyoma
• Angiofibroma
• Warts
• Pachydermia
• Lipoma
• Infantile Hemangioma

Capillary and Cavernous Lymphangiomas

• Surgical excision to the deep fascia if a deep component is present. Recurrence occurs in 25-50% of patients within 6-80 months.
• Cryotherapy is not very effective.
• Sclerotherapy provides short-term effects and various complications.
• Laser therapy (CO2, ND:YAG)
• Radiofrequency ablation
• Oral administration of sildenafil (selective phosphodiesterase-5 inhibitor)

Cystic Lymphangiomas

• For single-chamber cysts, periodic aspiration of their contents is recommended.
• Sclerosing therapy using bleomycin, sodium tetradecyl sulfate, OK-432 (picibanil) is effective in 87-95% of cases.
• With surgical treatment, recurrences occur in 10-27% of cases with complete excision and 88% with partial removal.