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

Study Status: Active, not recruiting
Recruit Status: Active, not recruiting
Study Type: Interventional

Official Title:

Brief Summary:

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.


Detailed Summary:
Sponsor: Assistance Publique Hopitaux De Marseille

Current Primary Outcome: 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


Original Primary Outcome: Same as current

Current Secondary Outcome: 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


Original Secondary Outcome: Same as current

Information By: Assistance Publique Hopitaux De Marseille

Dates:
Date Received: April 16, 2014
Date Started: May 2014
Date Completion: December 2016
Last Updated: November 16, 2015
Last Verified: November 2015