Psychosocial Consequences of the Screening of Von Hippel Lindau Diseases for Patients Operated for a hémangioblastoma of Nervous Centrasl System (PsychoVHL)
Von Hippel-Lindau (VHL) disease is a severe autosomal dominant genetic disorder (with almost complete penetrance) that predisposes to many tumors including some associated with a poorer outcome. Clear cell renal cell carcinoma (CCRCC) is the leading cause of mortality.
The diagnosis of VHL disease may be challenging because tumors have an asynchronous and multi-organ development and there is often no apparent hereditary context. As it is admitted that VHL disease is underdiagnosed, some countries have decided to recall patients presenting one of the potentially VHL disease-associated tumors to screen them for VHL mutation. Screening is currently recommended in guidelines but many patients may have not been previously screened.
Hemangioblastoma (HB) of the Central nervous system (CNS) is one of the typical VHL tumors and up to 20% of patients with HB show VHL mutation. VHL diagnosis in this population enables the diagnosis of other tumor types at an early stage of development since HB is chronologically the second tumor occurring during the VHL disease history.
But it raises critical problems and questions: difficult announcement of a potentially severe disease and psychosocial dimension related to inheritance of the disease.
|Hemangioblastoma (HB) of the Central Nervous System (CNS)||Other: evaluation of anxiety with psychosocial scales|
|Study Design:||Intervention Model: Single Group Assignment
Masking: None (Open Label)
- evaluate the impact on anxiety of VHL mutation screening of patients operated for HB [ Time Frame: two years ]The main objective of this study is to evaluate the impact on anxiety of VHL mutation screening of patients operated for HB
- assessment of the mood [ Time Frame: two years ]The evaluation of the mood will be made from Beck's scale. It is about a scale allowing to make a fast self-assessment of the depression
- assesment of quality of life [ Time Frame: two years ]The measure of the quality of life will be made by means of a generic questionnaire: the SF36. The SF36, used for the evaluation of the remote quality of life of the plan of announcement, is validated and available in French language
- assesment of the psychological consequences of the screening [ Time Frame: two years ]The psychological consequences of the screening will be estimated by means of Psychological Consequences Questionnaire . It is about a specific instrument allowing to estimate the psychosocial impact of the screening
|Study Start Date:||May 2014|
|Estimated Study Completion Date:||December 2016|
|Estimated Primary Completion Date:||September 2016 (Final data collection date for primary outcome measure)|
|Hemangioblastoma (HB) of the Central nervous system (CNS)||Other: evaluation of anxiety with psychosocial scales|
Please refer to this study by its ClinicalTrials.gov identifier: NCT02120040
|Assistance Publique Hopitaux de Marseille|
|Marseille, France, 13005|
|Principal Investigator:||Philippe Metellus, MD||Assistance Publique Hopitaux De Marseille|