Cerebellar hemangioblastoma

Cerebellar hemangioblastoma

Cerebellar hemangioblastoma is a vascular posterior fossa tumor with a clear border that develops intramedullary to extramedullary.

Classification

Histologically 1) and radiologically 2) , cerebellar HBs are traditionally described as four types:

Type 1 (5% of posterior fossa HBs) is a simple cyst without a macroscopic nodule.

Type 2 is a cyst with a mural nodule (60%).

Type 3, or solid tumors (26%).

Type 4, or solid tumors with small internal cysts (9%), are also seen in the cerebellum and predominate in the spinal cord.

Some authors have stated that type 1 is actually rare.

Clinical features

Diagnosis

Differential diagnosis

Several primary pathologic entities in diverse anatomic locations have the potential to simulate metastatic neoplasms histologically. Their misinterpretation as such may result in needless and extensive clinical evaluations that are intended to detect a presumed malignancy at another site. More importantly, mistakes of this type can deprive patients of surgical excisions that could be curative 3).

In adults with only cerebellar masses, cerebellar hemangioblastoma and cerebellar metastases are the 2 most important differential diagnoses.

High b value DWI reflects diffusion more accurately than does regular b value. Results showed that ADC calculation by high b value (b = 4000) DWI at 3-T magnetic resonance imaging is clinically useful for differentiating hemangioblastomas from brain metastases 4).

Arterial spin labelled imaging can aid in distinguishing hemangioblastoma from metastasis in patients with only cerebellar masses 5).


Coexistence of hemangioblastomas and AVMs is extremely rare, and only 3 cases have been reported previously in the literature 6).

Treatment

Radiation treatment

Effectiveness is dubious. May be useful to reduce tumor size or to retard growth, e.g. in patients who are not surgical candidates, for multiple small deep lesions, or for inoperable brainstem HGB. Does not prevent regrowth following subtotal excision.

Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas.

A retrospective chart review revealed 12 patients with a total of 20 intracranial hemangioblastomas treated with GKRS from May 1998 until December 2014. Kaplan-Meier plots were used to calculate the actuarial local tumor control rates and rate of recurrence following GKRS. Univariate analysis, including log rank test and Wilcoxon test were used on the Kaplan-Meier plots to evaluate the predictors of tumor progression. Two-tailed p value of <0.05 was considered as significant. Median follow-up was 64months (2-184). Median tumor volume pre-GKRS was 946mm3 (79-15970), while median tumor volume post-GKRS was 356mm3 (30-5404). Complications were seen in two patients. Tumor control rates were 100% at 1year, 90% at 3years, and 85% at 5years, using the Kaplan-Meier method. There were no statistically significant univariate predictors of progression identified, although there was a trend towards successful tumor control in solid tumors (p=0.07). GKRS is an effective and safe option for treating intracranial hemangioblastoma with favorable tumor control rates 7).


Suzuki et al. emphasize the usefulness of embolization with N-butyl cyanoacrylate for hemangioblastoma with ruptured feeder aneurysm, by which the aneurysm and the feeder could be simultaneously embolized 8).

Outcome

Surgical treatment may be curative in cases of sporadic HGB, not in VHL.

Solitary hemangioblastomas are for the most part considered benign, curable by total resection, except in those cases associated with von Hippel Lindau disease.

Despite extensive literature describing the diagnosis, treatment, and prognosis of these lesions, 9) individual cases still present a surgical quandary given their frequently eloquent location and high degree of vascularity.

Case series

Bründl et al. retrospectively analyzed the clinical, radiological, surgical, and histopathologic records of 24 consecutive patients (11 men, 13 women; mean age 51.3 years) with HBL of the posterior cranial fossa, who had been treated between 2001 and 2012.

Mean time to diagnosis was 14 weeks. The extent of resection (EOR) was total in 20 and near total in 4 patients. Four patients required revision within 24 h because of relevant postoperative bleeding. One patient died within 14 days. One patient required permanent shunting. At discharge, 75% of patients [n = 18, modified Rankin scale (mRS) 0-1] showed no or at least resolved symptoms. Mean follow-up was 21 months. Two recurrences were detected during follow-up.

In comparison to other benign entities of the posterior fossa, time to diagnosis was significantly shorter for HBL. This finding indicates the rather aggressive biological behavior of these excessively vascularized tumors. In this series, however, the rate of complete resection was high, and morbidity and mortality rates were within the reported range 10).


Cerebrospinal fluid dissemination of cerebellar hemangioblastoma was found dominantly in non-Von Hippel Lindau disease patients. The diagnosis was made 10 years after the initial surgery. Irradiation therapy was performed, but the patients died about 2 years after the diagnosis was given. Molecular targeted therapies including vascular proliferation suppression have been attempted lately, but no effective therapy has been established. Early diagnosis of dissemination as well as combination of aggressive excision and stereotactic radiosurgery are considered to be appropriate for current interventions 11).

Case reports

Cerebellar hemangioblastoma in von Hippel-Lindau disease

References

1)

Richard S, Campello C, Taillandier L, Parker F, Resche F. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. French VHL Study Group. J Intern Med. 1998 Jun;243(6):547-53. Review. PubMed PMID: 9681857.
2)

Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton TH. Posterior fossa hemangioblastomas: MR imaging. Radiology. 1989 May;171(2):463-8. PubMed PMID: 2704812.
3)

Wick MR. Primary lesions that may imitate metastatic tumors histologically: A selective review. Semin Diagn Pathol. 2017 Nov 17. pii: S0740-2570(17)30137-5. doi: 10.1053/j.semdp.2017.11.010. [Epub ahead of print] Review. PubMed PMID: 29174934.
4)

Onishi S, Hirose T, Takayasu T, Nosaka R, Kolakshyapati M, Saito T, Akiyama Y, Sugiyama K, Kurisu K, Yamasaki F. Advantage of High b Value Diffusion-Weighted Imaging for Differentiation of Hemangioblastoma from Brain Metastases in Posterior Fossa. World Neurosurg. 2017 May;101:643-650. doi: 10.1016/j.wneu.2017.01.100. Epub 2017 Feb 4. PubMed PMID: 28179177.
5)

Kang KM, Sohn CH, You SH, Nam JG, Choi SH, Yun TJ, Yoo RE, Kim JH. Added Value of Arterial Spin-Labeling MR Imaging for the Differentiation of Cerebellar Hemangioblastoma from Metastasis. AJNR Am J Neuroradiol. 2017 Nov;38(11):2052-2058. doi: 10.3174/ajnr.A5363. Epub 2017 Sep 14. PubMed PMID: 28912280.
6)

Monserrate Marrero JA, Monserrate Marrero AE, Pérez Berenguer JL, Álvarez EL, Corona JM, Feliciano C. Cerebellar Arteriovenous Malformation with Coexistent Hemangioblastoma. World Neurosurg. 2019 Nov 9;134:495-500. doi: 10.1016/j.wneu.2019.10.197. [Epub ahead of print] PubMed PMID: 31712111.
7)

Silva D, Grabowski MM, Juthani R, Sharma M, Angelov L, Vogelbaum MA, Chao S, Suh J, Mohammadi A, Barnett GH. Gamma Knife radiosurgery for intracranial hemangioblastoma. J Clin Neurosci. 2016 Jul 12. pii: S0967-5868(16)30013-3. doi: 10.1016/j.jocn.2016.03.008. [Epub ahead of print] PubMed PMID: 27422585.
8)

Suzuki M, Umeoka K, Kominami S, Morita A. Successful treatment of a ruptured flow-related aneurysm in a patient with hemangioblastoma: Case report and review of literature. Surg Neurol Int. 2014 Sep 26;5(Suppl 9):S430-3. doi: 10.4103/2152-7806.141887. eCollection 2014. PubMed PMID: 25324977; PubMed Central PMCID: PMC4199150.
9)

Cushing H, Bailey P. Tumors arising from blood vessels in the brain: angiomatous malformations and hemangioblastomas. Springfield, IL: Charles C Thomas; 1928.
10)

Bründl E, Schödel P, Ullrich OW, Brawanski A, Schebesch KM. Surgical resection of sporadic and hereditary hemangioblastoma: Our 10-year experience and a literature review. Surg Neurol Int. 2014 Sep 22;5:138. doi: 10.4103/2152-7806.141469. eCollection 2014. Review. PubMed PMID: 25317353; PubMed Central PMCID: PMC4192902.
11)

Akimoto J, Fukuhara H, Suda T, Nagai K, Hashimoto R, Michihiro K. Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease. Surg Neurol Int. 2014 Oct 7;5:145. doi: 10.4103/2152-7806.142321. eCollection 2014. PubMed PMID: 25324974; PubMed Central PMCID: PMC4199185.

Anterior inferior cerebellar artery aneurysm treatment

Anterior inferior cerebellar artery aneurysm treatment

The management strategies for treatment differ according to the location and configuration of the aneurysm. The existing body of neurosurgical literature contains articles published on aneurysms arising from the AICA near the basilar artery (BA), intracanalicular/meatal aneurysms, and distal AICA. Several therapeutic options exist, encompassing microsurgical and endovascular techniques.

Anterior inferior cerebellar artery aneurysms are rare lesions with a predisposition for distal location and non-saccular morphology. These aneurysms are less amenable to clipping and may instead require aneurysm trapping with bypass.

Anterior inferior cerebellar artery aneurysm endovascular treatment

Anterior inferior cerebellar artery aneurysm surgery

Cerebellar hemorrhage surgery

Cerebellar hemorrhage surgery

In 1906, Ballance first reported a surgical approach to treatment of cerebellar hemorrhage1) 2).

Since then, surgical treatment has become the general option for treatment 3).

Recommendations from Kobayashi et al in 1994 4)

1. patients with a Glasgow Coma Scale (GCS) score ≥14 and hematoma <4 cm diameter: treat conservatively

2. patients with GCS≤13 or with a hematoma ≥4 cm: surgical evacuation.

3. patients with absent brain stem reflexes and flaccid quadriplegia: intensive therapy is not indi- cated. Note: some authors contend that the loss of brain stem reflexes from direct compression may not be irreversible, 5) and that cerebellar hemorrhage represents a surgical emergency (and that the above criteria would thus deny potentially helpful surgery to some, see discussion of cerebellar infarction and decompression.

4. patients with hydrocephalus: ventricular catheter (if no coagulopathy). Caution: do not overdrain to avoid upward cerebellar herniation. Most cases with hydrocephalus also require evacuation of the clot

Criteria

Surgical treatment of cerebellar ICH can be life-saving but often leads to a poor functional outcome. New studies are needed on long-term functional outcome after a cerebellar ICH 6).

Since the 1970s, there has been a wide mutual consensus in the neurological and neurosurgical community that cerebellar ICHs should be operated on. However, the scientific proof is mainly based on small retrospective series with conflicting results 7).

To relieve brainstem compression and hydrocephalus, surgeons tend to favor occipital craniectomy or occipital craniotomy with hematoma evacuation in patients with a declining level of consciousness 8). Some regard this counterintuitive as long-term outcomes after surgical treatment of cerebellar ICH are generally pessimistic 9).


Since the report by Little et al., 10) the hematoma diameter has been considered a significant factor in the decision-making process for optimal treatment.

The criteria for surgery remain controversial, and many researchers have determined that a hematoma larger than 3 cm, obstruction of the quadrigeminal cistern, and compression of the fourth ventricle are surgical criteria 11) 12) 13).

Cohen et al. 14) used a maximal hematoma diameter greater than 3 cm as the surgical criterion, however, some patients with a hematoma larger than 3 cm who underwent conservative treatment had a good prognosis as well. In addition, a hematoma volume greater than 15 mL, being equivalent with a hematoma with a maximal diameter greater than 3 cm, has also been used as a criterion in some cases 15).


The criteria of Kobayashi et al., are as follows:

1) patients with Glasgow Coma Scale scores of 14 or 15 and with a hematoma of less than 40 mm in maximum diameter are treated conservatively

2) for the patients with Glasgow Coma Scale scores of 13 or less at admission or with a hematoma measuring 40 mm or more, hematoma evacuation with decompressive suboccipital craniectomy should be a treatment of choice

3) for the patient whose brain stem reflexes are entirely lost with flaccid tetraplegia or whose general condition is poor, intensive therapy is not indicated. The validity of these criteria was tested and confirmed in 49 cases 16).

Technique

Position

Lateral oblique position with the involved side up.

If rapidity is crucial a suboccipital midline skin incision is preferred because it can be taken down quickly with little fear of encountering a vertebral artery.

Suboccipital craniectomy is preferred over suboccipital craniotomy to accomodate postoperative swelling.

A prophylactically ventriculostomy at Frazier’s point is recommended to allow rapid treatment of postoperative hydrocephalus or intracranial pressure monitoring.

In cases where there has been rupture into the ventricular system, the surgical microscope should be used to follow the clot to the fourth ventriclewhich is then cleared of clot.

External ventricular drainage (EVD) combined with intraventricular thrombolysis (IVF) is rarely used in severe spontaneous cerebellar hemorrhage(SCH) with intraventricular hemorrhage (IVH).

It is a treatment option for elderly patients with severe SCH + IVH 17).

Video

References

1) , 15)

Cho SM, Hu C, Pyen JS, Whang K, Kim HJ, Han YP, et al. Predictors of outcome of spontaneous cerebellar hemorrhage. J Korean Neurosurg Soc. 1997 Oct;26(10):1395–1400.
2) , 3)

Dahdaleh NS, Dlouhy BJ, Viljoen SV, Capuano AW, Kung DK, Torner JC, Hasan DM, Howard MA 3rd. Clinical and radiographic predictors of neurological outcome following posterior fossa decompression for spontaneous cerebellar hemorrhage. J Clin Neurosci. 2012 Sep;19(9):1236-41. doi: 10.1016/j.jocn.2011.11.025. Epub 2012 Jun 20. PubMed PMID: 22721890.
4)

Kobayashi S, Sato A, Kageyama Y, et al. Treatment of Hypertensive Cerebellar Hemorrhage – Surgical or Conservative Management. Neurosurgery. 1994; 34:246–251
5)

Heros RC. Surgical Treatment of Cerebellar Infarc- tion. Stroke. 1992; 23:937–938
6)

Satopää J, Meretoja A, Koivunen RJ, Mustanoja S, Putaala J, Kaste M, Strbian D, Tatlisumak T, Niemelä MR. Treatment of intracerebellar haemorrhage: Poor outcome and high long-term mortality. Surg Neurol Int. 2017 Nov 9;8:272. doi: 10.4103/sni.sni_168_17. eCollection 2017. PubMed PMID: 29204307; PubMed Central PMCID: PMC5691556.
7)

Witsch J, Neugebauer H, Zweckberger K, Jüttler E. Primary cerebellar haemorrhage: complications, treatment and outcome. Clin Neurol Neurosurg. 2013 Jul;115(7):863-9. doi: 10.1016/j.clineuro.2013.04.009. Epub 2013 May 6. Review. PubMed PMID: 23659765.
8)

Wijdicks EF, St Louis EK, Atkinson JD, Li H. Clinician’s biases toward surgery in cerebellar hematomas: an analysis of decision-making in 94 patients. Cerebrovasc Dis. 2000 Mar-Apr;10(2):93-6. PubMed PMID: 10686446.
9)

Luney MS, English SW, Longworth A, Simpson J, Gudibande S, Matta B, Burnstein RM, Veenith T. Acute Posterior Cranial Fossa Hemorrhage-Is Surgical Decompression Better than Expectant Medical Management? Neurocrit Care. 2016 Dec;25(3):365-370. PubMed PMID: 27071924; PubMed Central PMCID: PMC5138260.
10)

Little JR, Tubman DE, Ethier R. Cerebellar hemorrhage in adults. Diagnosis by computerized tomography. J Neurosurg. 1978 Apr;48(4):575-9. PubMed PMID: 632882.
11) , 14)

Cohen ZR, Ram Z, Knoller N, Peles E, Hadani M. Management and outcome of non-traumatic cerebellar haemorrhage. Cerebrovasc Dis. 2002;14(3-4):207-13. PubMed PMID: 12403953.
12)

Kirollos RW, Tyagi AK, Ross SA, van Hille PT, Marks PV. Management of spontaneous cerebellar hematomas: a prospective treatment protocol. Neurosurgery. 2001 Dec;49(6):1378-86; discussion 1386-7. PubMed PMID: 11846937.
13)

Salvati M, Cervoni L, Raco A, Delfini R. Spontaneous cerebellar hemorrhage: clinical remarks on 50 cases. Surg Neurol. 2001 Mar;55(3):156-61; discussion 161. PubMed PMID: 11311913.
16)

Kobayashi S, Sato A, Kageyama Y, Nakamura H, Watanabe Y, Yamaura A. Treatment of hypertensive cerebellar hemorrhage–surgical or conservative management? Neurosurgery. 1994 Feb;34(2):246-50; discussion 250-1. PubMed PMID: 8177384.
17)

Zhang J, Wang L, Xiong Z, Han Q, Du Q, Sun S, Wang Y, You C, Chen J. A treatment option for severe cerebellar hemorrhage with ventricular extension in elderly patients: intraventricular fibrinolysis. J Neurol. 2014 Feb;261(2):324-9. doi: 10.1007/s00415-013-7198-2. Epub 2013 Dec 3. PubMed PMID: 24297364.
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