Artikel
Large hemangioblastomata of the posterior fossa: report of three cases
Hämangioblastom der hinteren Schädelgrube: drei klinische Berichte
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Autoren
Veröffentlicht: | 4. Mai 2005 |
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Gliederung
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Objective
Haemangioblastoma (HBs) of the central nervous system may occur sporadically, or in association with von Hippel-Lindau (vHL) disease. Not seldom are hemangioblastomata seen in the posterior cranial fossa. However, large lesions of the brainstem form a rarity.
Case Reports
Three patients (all males; 38, 68 and 36 years) presented with a history of chronic headache and subtle caudal cranial nerve deficiencies (spontaneous nystagmus, facial hypesthesia, deglutition problems). Diagnostic imaging showed large vascular lesions in the region of the IV ventricle, compressing the brainstem. The lesions were 5x6 cm, 4x5 cm and 5x6 cm respectively. There was no family history, or other concomitant findings associated with vHL disease. One patient required an external ventricle drainage due to occlusive hydrocephalus, before definitive the treatment of the tumor could be started.
Treatment
Staged treatment was administered. Interventional endovascular embolization was performed as the first phase. One patient showed a PICA occlusion and associated cerebellar infarction after embolization. In the second phase, the lesions were removed via the midline suboccipital approach (cerebellar interhemispheric approach). Complete removal was possible in all three cases, as documented by means of postoperative MRI.
Postoperative course and follow-up
The caudal cranial nerve deficiencies deteriorated soon after surgery in all the three patients. The two young patients (38 & 36 yrs) recovered from these deficiencies within three months. In one elderly patient tracheotomy was required. In two patients ventriculo-peritoneal shunts were implanted. All three underwent rehabilitation procedures, clinical and MRI follow-up every three months after surgery. The patient with the tracheostoma could be weaned successfully during the rehab. Follow-up accounts to four years in one patient and three years in two. There was no recurrence of the hemangioblastomata in MRI control studies performed at the latest follow-up. The Karnofsky Index at the last follow-up accounts to 60, 70 and 80 in the three patients aged 68, 36 and 38 respectively.
Conclusions
We have presented three rare cases of large hemangioblatomata of the brainstem region. The treatment of such cases mandates a combined approach, e.g., endovascular and surgical. The outcomes might be associated with the age of the patient and the initial functional deficiencies before treatment.