[Intramedullary hemangioblastomas]
P David1, M Messerer2, N Aghakhani2
1Service de neurochirurgie, hôpital Bicêtre, AP-HP, 78, rue du General-Leclerc, 94275 Le Kremlin-Bicêtre cedex, France; Centre expert national cancers rares PREDIR, hôpital Bicêtre, AP-HP, 78, rue du General-Leclerc, 94275 Le Kremlin-Bicêtre, France.
Surgical management of intramedullary hemangioblastomas, rare spinal tumors often linked to Von Hippel Lindau disease, yields positive outcomes. Complete resection is achievable in most cases, leading to stability or improvement for the majority of patients.
Area of Science:
- Neurosurgery
- Oncology
- Genetics
Background:
- Intramedullary hemangioblastomas are rare spinal tumors, accounting for 1-5% of all spinal neoplasms.
- These lesions are frequently associated with Von Hippel Lindau disease, a genetic disorder.
- Surgical intervention is the primary treatment modality for these tumors.
Purpose of the Study:
- To review the surgical management experience with intramedullary hemangioblastomas.
- To analyze clinical presentation, radiological findings, surgical procedures, and outcomes.
- To evaluate the association with Von Hippel Lindau disease.
Main Methods:
- Retrospective analysis of 59 patients with intramedullary hemangioblastomas operated between 1993 and 2011.
- Screening for Von Hippel Lindau disease in all patients.
- Minimum follow-up of 3 years, recording clinical, radiological, and surgical data.
Main Results:
- A total of 65 tumors were analyzed in 59 patients (mean age 38 years).
- Von Hippel Lindau disease was present in 72.5% of patients.
- Complete resection was achieved in 95% of cases, with 86% experiencing stability and 12% clinical improvement.
Conclusions:
- Screening for Von Hippel Lindau disease is crucial for all patients with intramedullary hemangioblastomas.
- Close monitoring is recommended for patients diagnosed with Von Hippel Lindau disease.
- Surgical removal is strongly advised for tumors causing neurological deficits or showing radiological progression.
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