Psychosocial Consequences of the Screening of Von Hippel Lindau Diseases for Patients Operated for a hémangioblastoma of Nervous Centrasl System

Study Overview
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Brief Summary
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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.

Conditions
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Hemangioblastoma (HB) of the Central Nervous System (CNS)

Intervention / Treatment
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* Other: evaluation of anxiety with psychosocial scales


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: age >=18; Surgery for a CNS HB in the department of Neurosurgery of la Timone university hospital since 1999 ; Absence of prior screening for VHL Exclusion Criteria: minor, incorrect French language

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Hemangioblastoma (HB) of the Central nervous system (CNS)<br> | Other: evaluation of anxiety with psychosocial scales<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| evaluate the impact on anxiety of VHL mutation screening of patients operated for HB | The main objective of this study is to evaluate the impact on anxiety of VHL mutation screening of patients operated for HB | two years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| assessment of the mood | 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 | two years |