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

Public Assistance-Hospitals of Marseille (AP-HM)

Status: Completed

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

Treatments: Other: evaluation of anxiety with psychosocial scales

Study type: Interventional

Funder types: Other

Identifiers

NCT02120040

2014-01

Details and patient eligibility

About

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.

Enrollment: 42 estimated patients

Sex: All

Ages: 18 to 80 years old

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Other

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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42 participants in 1 patient group

Hemangioblastoma (HB) of the Central nervous system (CNS)

Other group

Treatment:

Other: evaluation of anxiety with psychosocial scales