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.

Study Overview

• Status: Completed
• Conditions: Hemangioblastoma (HB) of the Central Nervous System (CNS)
• Intervention / Treatment: Other: evaluation of anxiety with psychosocial scales

• Study Type: Interventional
• Enrollment (Anticipated): 42
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Marseille, France, 13005
    • Assistance Publique Hopitaux de Marseille

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 80 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Other
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

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

What is the study measuring?

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

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
assesment of quality of life	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	two years
assesment of the psychological consequences of the screening	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	two years

Collaborators and Investigators

• Sponsor: Assistance Publique Hopitaux De Marseille
• Investigators: Principal Investigator: Philippe Metellus, MD, Assistance Publique Hopitaux de Marseille

Study record dates

Study Major Dates

• Study Start (Actual): May 2, 2014
• Primary Completion (Actual): October 9, 2015
• Study Completion (Actual): March 8, 2023

Study Registration Dates

• First Submitted: April 16, 2014
• First Submitted That Met QC Criteria: April 18, 2014
• First Posted (Estimate): April 22, 2014

Study Record Updates

• Last Update Posted (Actual): April 6, 2023
• Last Update Submitted That Met QC Criteria: April 5, 2023
• Last Verified: April 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Neoplasms by Histologic Type; Neoplasms; Hemangioma; Neoplasms, Vascular Tissue; Hemangioma, Capillary; Hemangioblastoma
• Other Study ID Numbers: 2014-01