Hemangioma, PEComa
Conditions
Keywords
propranolol, betablocker, angiogenesis
Brief summary
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
Interventions
Sponsors
Study design
Eligibility
Inclusion criteria
* patients who will have biopsy or surgery for PEComa or vascular pediatric tumor (RICH, NICH, hemangioma or pyogenic granuloma) * A free, informed and written consent will be established
Exclusion criteria
* none
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| YAP cellular expression by immunohistochemistry study on collected tissues | At inclusion (day 0)] |
Secondary
| Measure | Time frame |
|---|---|
| Study of Hippo/YAP pathway expression by western-blot, and correlation with propranolol efficacy | At inclusion (day 0)] |
| Beta adrenergic receptors expression by immunohistochemistry study on collected tissues | At inclusion (day 0)] |
| Study of MAP/kinases pathway expression by western-blot, and correlation with propranolol efficacy | At inclusion (day 0)] |
| Study of PKA/AMPc pathway expression by western-blot, and correlation with propranolol efficacy | At inclusion (day 0)] |
Countries
France