LAUSR

BACKGROUND Despite being previously considered as congenital lesions, recent studies agree to classify Cerebral Cavernous Malformations (CCM) as acquired forms with clear correlations with other pathological affections of the Central Nervous System (CNS). In addition, a special subgroup, notably known as "de Novo" CCMs (dnCCM), are associated in a significant number of cases with Developmental Venous Anomalies (DVAs) and, in other cases, with Radiotherapy treatments. METHODS A mini-series of 4 patients with clinical history characterized by developing dnCCM is reported. In three patients, the dnCCM was associated with the presence of an isolated DVA. In one case, no DVA was detected, but the patient underwent brain radiotherapy. In three cases, the dnCCM was clinically symptomatic, and the patients were submitted to a surgical procedure for lesion removal. In one case, the dnCCM was detected during MRI follow-up. RESULTS Adding a review of the literature, we describe 47 patients who presented dnCCMs. The most common presentation is a sporadic CCM with a DVA, and the onset presentation was bleeding in 4 out of 47 cases (8,5%). Bleeding of dnCCM was observed in 9 out of 47 cases (19 %), and the choice treatment was surgical in 24 out of 47 cases (51%). DISCUSSIONS We present our series with a review of the recent literature and discuss the "de novo" cavernous malformation pathogenesis. A throughout review of recent literature is reported to clarify the predisposing factors that may lead to dnCCM development in patients carrying specific genetic and molecular features. Considering the high risk of bleeding, strict follow-up and aggressive treatment should be evaluated in dnCCM management.