Cavernous Hemangioma

Cavernous Hemangioma (cavernous hemangioma) is a low-flow vascular malformation present at birth, also known as venous malformation. Most venous malformations are cavernous, hence the name. There is ongoing debate about the nature of cavernous hemangiomas, but recent research increasingly suggests they are congenital vascular anomalies.

Etiology and Pathogenesis

1. Genetic Factors: Familial cavernous hemangiomas are common in Hispanic individuals and are autosomal dominant. The mutated gene is located on chromosome 7q (q11-q22).
2. Abnormal Vascular Development: During embryonic development, vascular tissues may develop abnormally, particularly during the vascular network stage, forming clusters that merge into cavernous hemangiomas, usually visible at birth or shortly after.
3. Induced Factors: Routine treatments, viral infections, trauma, and surgical bleeding can induce local necrosis and dilatation of blood vessels, forming cavernous hemangiomas. Increased tumor size may result from repeated minor bleeding and thrombosis within the lesion.
4. Endocrine Changes: Hormonal changes, such as pregnancy or oral estrogen use, can cause rapid growth and symptoms of hemangiomas.

Clinical Manifestations

Cavernous hemangiomas appear at birth or shortly after, affecting males and females equally, commonly on the head, face, neck, limbs, and trunk. They can involve skin, subcutaneous tissue, mucous membranes, muscles, bones, or internal organs. Skin lesions are large, irregular, soft subcutaneous masses, bluish or light purple, with visible varicose veins. Deeper lesions cause localized swelling without significant skin color changes. Hemangiomas in muscles, bones, or organs can cause functional impairment or deformities. Symptoms include localized pain, swelling, or ulceration, with potential bleeding risks from trauma or infection. Severe types like Kasabach-Merritt syndrome and conditions like Macci syndrome and blue rubber bleb nevus can accompany multiple hemangiomas and other congenital abnormalities.

Pathological Features

Tumors are located in the deep dermis or subcutaneous tissue, consisting of large, irregular cavities resembling venous sinuses, lined with a single layer of endothelial cells, with fibrous tissue of varying thickness surrounding them. Cavities contain red blood cells and fibrin, with occasional thrombus or calcification.

Diagnosis and Differential Diagnosis

Diagnosis is based on the presence at birth, growth with age, bluish or purple skin elevation, and compression shrinkage. It is differentiated from strawberry hemangiomas, which appear weeks after birth, grow rapidly, and regress by age 1-2.

Treatment

1. General Treatment: Compression therapy with elastic bandages can delay further expansion and alleviate symptoms for extensive limb involvement.
2. Medication: Sclerotherapy involves injecting sclerosing agents (e.g., sodium morrhuate, urea) into the tumor to induce inflammation and occlusion, effective for small to medium-sized hemangiomas.
3. Surgical Treatment: Surgical excision is feasible for localized hemangiomas, with preoperative imaging to assess blood flow. Techniques like copper needle placement to induce thrombosis can reduce tumor size before surgery.
4. Laser Treatment: Effective for superficial lesions with limited depth penetration, lasers like long-pulse 1064nm Ndand dual-wavelength lasers are used. Post-laser care follows protocols similar to port wine stain treatment.

Source: Cavernous Hemangioma