Figure 5. Progression of CCM Lesion Pathogenesis.
The pathogenesis of CCMs begins with an inherited or somatic mutation, followed by somatic mutations resulting in lesion genesis and growth. The natural history of CCM disease is thought to lead to five clinically relevant outcomes (1) lesion stabilization, (2) lesion regression, (3) increased lesion burden, (4) symptomatic hemorrhage (SH), and/or (5) lesional growth (shown from top to bottom). CASH, Cavernous Angioma with Symptomatic Hemorrhage.