Medical management of CNS hemangioblastoma in pediatric patients with von Hippel-Lindau (VHL) disease

• Conditions: D48.1; Q85.8; Connective and other soft tissue; Other phakomatoses, not elsewhere classified

• Registration Number: DRKS00029553
• Lead Sponsor: niversitätsklinikum Freiburg

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2022-08-16
• Study Completion: 2023-06-23
• Last Update Posted: 19 August 2024

Recruitment & Eligibility

• Status: Complete
• Sex: All
• Target Recruitment: 99

Inclusion Criteria

Pediatric patients diagnosed with VHL disease and available MRI examinations of the CNS during the period 01/2000 - 07/2022

Exclusion Criteria

Presence of sporadic CNS hemangioblastoma (thus excluding VHL disease).
Presence of MRI examinations exclusively in adulthood.

Study & Design

• Study Type: observational
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Clinical neurological status (modified Rankin Scale and modified McCormick Scale) of VHL patients at entry into adulthood in terms of the number of a) detected CNS hemangioblastomas as well as b) microsurgical removals of CNS hemangioblastomas in childhood/adolescence.

Secondary Outcome Measures

Name	Time	Method
- Comparison of clinical neurological status (mRS and modified McCormick Scale) postoperatively to preoperatively<br>- Survey of complications and reasons of possible worsening of clinical outcome due to microsurgical therapy of CNS hemangioblastomas<br>- To evaluate the indication for microsurgical removal of CNS hemangioblastomas in children/adolescents (asymptomatic/symptomatic)<br>- Survey of clinical neurological symptoms that led to MRI examination of the CNS outside the screening intervals (unscheduled)<br>- Survey of the number of tumor recurrences in childhood/adolescence<br>- Age distribution of performed surgeries on CNS hemangioblastomas in VHL patients in childhood/adolescence<br>- Survey of the number and distribution of CNS hemangioblastomas at the time of first MRI examination of the CNS<br>- To survey the age in childhood/adolescence with first detection of CNS hemangioblastoma