Angiochondromatosis Congenital

Congenital angiochondromatosis is a rare disease characterized by the development of benign tumors of cartilage tissue in combination with damage to blood vessels.

Causes

The exact cause of congenital angiochondromatosis is unknown. It is assumed that the disease is caused by genetic defects leading to disturbances in the development of cartilage and vascular tissue.

Symptoms

The main manifestations of congenital angiochondromatosis:

  1. The appearance of multiple subcutaneous nodules and tumors consisting of cartilage tissue. The limbs, face, and torso are most often affected.

  2. Involvement of blood vessels in the pathological process, which leads to their deformation and expansion (aneurysm).

  3. Possible damage to internal organs and bone tissue.

  4. Pain syndrome when nerves are compressed by tumors.

Diagnosis is based on the clinical picture and data from imaging examinations (X-ray, MRI, angiography).

Treatment consists of removing tumors and correcting vascular disorders. The prognosis is generally favorable, but relapses and progression of the process are possible.