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Solid haemangioblastomas of the CNS: a review of 17 consecutive cases

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Abstract

A retrospective study on solid central nervous system haemangioblastomas was performed to characterize clinical features, treatment strategies and outcome in these rare lesions. Between 1993 and 2006 23, solid haemangioblastomas were surgically removed in 17 patients. Eight lesions were located within pons Varolii and medulla oblongata, six within the cerebellar hemispheres and three in the cerebellopontine angle. Three haemangioblastomas were located supratentorially and three within the spinal cord. All patients except two underwent pre-operative magnetic resonance imaging (MRI). Post-operative digital subtraction angiography and/or MRI was performed in all surviving patients. Except for spinal cord lesions, rather unsystematic clinical symptoms were observed. Twenty-two tumours could be resected completely. Two patients with brainstem lesions died within 10 weeks after surgery from infectious complications. Persistent new neurological deficits occurred in two patients. Three patients underwent radiosurgery prior to or following the surgical procedure. Solid haemangioblastomas represent a surgical challenge due to their arteriovenous malformation-like vascularisation and their frequent location in eloquent areas. Surgery is the therapy of choice. Circumferential dissection with devascularization and en bloc removal yields good functional results. A location within the brainstem carries the most unfavourable prognosis.

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Authors and Affiliations

  1. Department of Neurosurgery, University of Halle-Wittenberg, Ernst-Grube-Strasse 40, 06097, Halle, Germany

    Jens Rachinger, Julian Prell & Christian Strauss

  2. Department of Neuropathology, University of Erlangen-Nuremberg, Erlangen, Germany

    Rolf Buslei

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Comments

Kartik G. Krishnan, Gabriele Schackert, Dresden, Germany

In this report on solid hemangioblastomas of the central nervous system, Rachinger et al. analyze the outcomes after the surgical treatment of the condition in 17 patients (in all, 23 hemangioblastomas). The majority of the lesions (17 of 23) were found in the posterior cranial fossa, whereas three were located supratentorially and the rest three within the spinal cord.

Although hemangioblastomas are benign lesions, as per the histological description, major factors influencing outcomes of treatment are size and localization of the lesions. Hemangioblastomas preferentially tend to occur in the posterior cranial fossa. Here, as in the case of any other lesion, pons Varolii and brainstem are vulnerable regions.

Whereas small tumors in resectable locations can be completely excised, large tumors require a combination treatment strategy of interventional embolization and surgery. Pre-operative embolization of large hemangioblastomas of the posterior fossa helps to minimize intraoperative blood loss by virtue of reducing the vascularity of the tumors. Having said the above, even super-selective embolization of large hemangioblastomas of the craniocervical junction is not without infarction hazards, which may eventually turn life threatening. Thus, pre-operative embolization of large tumors that draw their vascularity from the magistrals of the posterior circulation is not always redeemable. Surgical resection is nevertheless indicated, owing to the space-occupying nature of the lesions, before bleeding or decompensation of the brain stem can occur. A favorable prognosis may be expected in these cases in the long run.

Uwe Spetzger, Karlsruhe, Germany

The authors present an interesting retrospective study to evaluate the clinical features and the therapeutic strategies in 17 patients, totally harbouring 23 solid haemangioblastomas of the CNS. Usually, haemangioblastomas appear cystic, solid with cystic components or solid. This study indicates that there might be differences in clinical manifestation between cystic and solid lesions. The mechanism for neurological deterioration is the growing of the lesion, especially the expansion of the cyst with focal compression of the surrounding tissue. Another potential problem could be a bleeding from a haemangioblastoma; this rare event increase morbidity and mortality significantly. However, this is a very unlikely event and is associated mainly with bigger lesions. There are no figures from the literature if solid haemangioblastomas have a higher rate of spontaneous bleeding, which is also not answered by this report. Considerably, small solid haemangioblastomas are in an early stage of their evolution, which eventually may transform into cystic ones. However, due to our own experience, we have also seen the transformation of cystic lesions into solid tumours.

The typical infratentorial location of haemangioblastomas within the brainstem or in the spinal cord is a challenge even for an experienced neurosurgeon. There is evidence that operative treatment of solid haemangioblastomas is more complex than that of cystic ones because the amount of the tissue and especially the vascular architecture and the blood supply of a solid component are much more complex to handle as the simple microsurgical removal of a cystic compartment.

For my experience, mainly the size and the location, respectively the access and the optimal visualization of a haemangioblastoma, are the decisive features to predict the surgical risk. The adequate pre-operative imaging allowing the exact localization of the arterial feeders and the venous drainage, as well as the intraoperative discovery and confirmation of those vessels, are the essentials for a well-ordered microsurgical removal, especially in huge tumours. Therefore, the whole armamentarium of vascular microsurgery including intraoperative blood-flow measurement, neuronavigation, electrophysiological monitoring and an experienced neuroanaesthesiologist are mandatory to reduce surgical morbidity.

The meticulously recorded results of the authors correspond to our own experiences; if the size of the haemangiobalstoma and the approach to the lesion allow an en bloc removal, usually, the post-operative results are favourable. These results are the basis to discuss the indication of non-surgical treatment modalities, especially in the evolving field of stereotactic radiosurgery.

Yoko Kato, Toyoake, Japan

The removal of a solid haemangioblastoma in an eloquent location remains one of the most formidable tasks even for an experienced neurosurgeon; even in the best hands, some of these lesions are to be left for other modalities of treatment. The meticulously recorded experience of the authors is valuable for decision making in these difficult-to-treat tumors; however, the variability of locations (spinal cord, brain stem, cerebellar hemispheres and supratentorial lesions), makes it difficult to draw conclusive evidence.

For haemangioblastoma being solid is considered to be an early stage in their evolution, which eventually may transform into a cystic one, and that is true particularly for the small ones. If they are multiple (vHL disease), it is difficult to decide which one is symptomatic, and the expected morbidity if removed is the most important limiting factor in the decision.

In our experience, all brain stem lesions potentially may show post-operative lower cranial nerve deficits and have to be taken out of ventillatory support carefully, over the next 2–3 days, irrespective of the uncomplicated surgery.

A problematic event is a bleeding haemangioblastoma, an event that significantly increases morbidity, and life-saving emergency surgery should be considered; fortunately, the review of statistics showed that hemorrhage is a very unlikely event and is associated mainly with bigger lesions.

Removal “in toto” has been also our strategy, and we have abstained from embolizing brain stem lesions, avoiding the risk of complications.

With the increasing experience in stereotactic radiosurgery, we expect that the dividing line of indications between modalities will move towards less aggressive surgery.

Many important questions remain unanswered, particularly regarding the effect of cerebral blood flow changes in critical zones at the time of removal. The proper preservation of important arterial supply and venous drainage, the use of combined modalities of treatment and certainly a broader accumulation and evaluation of data is very important.

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Rachinger, J., Buslei, R., Prell, J. et al. Solid haemangioblastomas of the CNS: a review of 17 consecutive cases. Neurosurg Rev 32, 37–48 (2009). https://doi.org/10.1007/s10143-008-0166-0

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