PEComa is rare tumor affecting particularly patients with Tuberous Sclerosis. Biological similarities were seen between PEComa and infantile hemangioma. Propranolol is highly efficient to treat infantile hemangioma and we believe that this drug can also be useful for the treatment of PEComa. Purpose : to understand the mechanism of action of propranolol in PEComas and related pediatric vascular lesions and to select possible novel targets of betablockers in vascular tumors related to PEComas by using YAP oncogene activation as a molecular marker
Study Type
OBSERVATIONAL
Enrollment
13
CHU de Bordeaux
Bordeaux, France
YAP cellular expression by immunohistochemistry study on collected tissues
Time frame: At inclusion (day 0)]
Beta adrenergic receptors expression by immunohistochemistry study on collected tissues
Time frame: At inclusion (day 0)]
Study of Hippo/YAP pathway expression by western-blot, and correlation with propranolol efficacy
Time frame: At inclusion (day 0)]
Study of MAP/kinases pathway expression by western-blot, and correlation with propranolol efficacy
Time frame: At inclusion (day 0)]
Study of PKA/AMPc pathway expression by western-blot, and correlation with propranolol efficacy
Time frame: At inclusion (day 0)]
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