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.

Spinal cord hemangioblastoma treatment

Spinal cord hemangioblastoma treatment

Although radiosurgery has been used to treat multiple hemangioblastoma, particularly in the cerebellum, complete microsurgical removal is the treatment of choice for spinal cord hemangioblastoma 1).

Partial resection or biopsy may cause postoperative bleeding and should therefore not be performed. Bleeding during dissection, due to the vascularity of HBs, increases the risk of adverse events.

minimally invasive approach for the resection of selected spinal hemangioblastomas is safe and allows complete tumor resection with good clinical results in experienced hands 2).


They are almost always associated with a syrinx or significant edema.

Cases associated with edema and syrinx are more space-occupying than those only with solid part of the tumor. Consequently, the mass effect producing neurological symptoms derives from the cyst rather than the tumor itself. On the removal of hemangioblastomas in association with a syrinx, the syrinx is spontaneously opened and always stops growing and usually regresses in size. Thus, the additional opening of the syrinx or surgical removal of the syrinx is not necessary 3).

Preceding Embolization

Although some investigators recommend preoperative embolization, 4) 5) in the series of Harati et al. it was usually not necessary to achieve complete resection 6). This is in concordance to several other series so that preoperative embolization is generally not recommended 7) 8) 9) 10) 11). To prevent intraoperative bleeding in selected cases, temporary artery occlusion was performed. This technique is described in detail by Clark et al.12).

Fluorescent dye

As vascular tumors, intramedullary hemangioblastomas are associated with significant intraoperative blood loss, making them particularly challenging clinical entities. The use of intraoperative indocyanine green or other fluorescent dyes has previously been described to avoid breaching the tumor capsule, but improved surgical outcomes may result from identifying and ligating the feeder arteries and arterialized draining veins.

Molina et al. presented a written and media illustration of a technique for intraoperative indocyanine green use in the en bloc resection of intramedullary hemangioblastoma 13).

Radiosurgery

Cyberknife radiosurgery has proven to be safe in the treatment of spinal HBs 14). However, as radiographic regression was achieved in only 22%, microsurgical resection remains the gold standard for spinal HBs that are clearly symptomatic or have developed radiographic progression in size, spinal cord edema, or syrinx 15) 16) 17).

References

1)

Samii M, Klekamp J. Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery. 1994 Nov;35(5):865-73; discussion 873. PubMed PMID: 7838335.
2)

Krüger MT, Steiert C, Gläsker S, Klingler JH. Minimally invasive resection of spinal hemangioblastoma: feasibility and clinical results in a series of 18 patients. J Neurosurg Spine. 2019 Aug 9:1-10. doi: 10.3171/2019.5.SPINE1975. [Epub ahead of print] PubMed PMID: 31398701.
3)

Na JH, Kim HS, Eoh W, Kim JH, Kim JS, Kim ES. Spinal cord hemangioblastoma : diagnosis and clinical outcome after surgical treatment. J Korean Neurosurg Soc. 2007 Dec;42(6):436-40. doi: 10.3340/jkns.2007.42.6.436. Epub 2007 Dec 20. PubMed PMID: 19096585; PubMed Central PMCID: PMC2588179.
4)

Montano N, Doglietto F, Pedicelli A, Albanese A, Lauretti L, Pallini R. Embolization of hemangioblastomas. J Neurosurg. 2008. 108: 1063-4
5)

Yang Y, Wang D, Jiang H, Sha C, Yuan Q, Liu J. [Treatment of spinal cord hemangioblastoma by microoperations combined with embolization]. Zhonghua Yi Xue Za Zhi. 2008. 88: 1309-12
6)

Harati A, Satopää J, Mahler L, Billon-Grand R, Elsharkawy A, Niemelä M, Hernesniemi J. Early microsurgical treatment for spinal hemangioblastomas improves outcome in patients with von Hippel-Lindau disease. Surg Neurol Int. 2012;3:6. doi: 10.4103/2152-7806.92170. Epub 2012 Jan 21. PubMed PMID: 22347675; PubMed Central PMCID: PMC3279991.
7)

Cornelius JF, Saint-Maurice JP, Bresson D, George B, Houdart E. Hemorrhage after particle embolization of hemangioblastomas: Comparison of outcomes in spinal and cerebellar lesions. J Neurosurg. 2007. 106: 994-8
8)

Mandigo CE, Ogden AT, Angevine PD, McCormick PC. Operative management of spinal hemangioblastoma. Neurosurgery. 2009. 65: 1166-77
9)

Mehta GU, Asthagiri AR, Bakhtian KD, Auh S, Oldfield EH, Lonser RR. Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine. 2010. 12: 233-42
10)

Oppenlander ME, Spetzler RF. Advances in spinal hemangioblastoma surgery. World Neurosurg. 2010. 74: 116-7
11)

Pietilä TA, Stendel R, Schilling A, Krznaric I, Brock M. Surgical treatment of spinal hemangioblastomas. Acta Neurochir (Wien). 2000. 142: 879-86
12)

Clark AJ, Lu DC, Richardson RM, Tihan T, Parsa AT, Chou D. Surgical technique of temporary arterial occlusion in the operative management of spinal hemangioblastomas. World Neurosurg. 2010. 74: 200-5
13)

Molina CA, Pennington Z, Ahmed AK, Westbroek E, Goodwin ML, Tamargo R, Sciubba DM. Use of Intraoperative Indocyanine Green Angiography for Feeder Vessel Ligation and En Bloc Resection of Intramedullary Hemangioblastoma. Oper Neurosurg (Hagerstown). 2019 Apr 1. pii: opz053. doi: 10.1093/ons/opz053. [Epub ahead of print] PubMed PMID: 31220325.
14)

Moss JM, Choi CY, Adler JR, Soltys SG, Gibbs IC, Chang SD. Stereotactic radiosurgical treatment of cranial and spinal hemangioblastomas. Neurosurgery. 2009. 65: 79-85
15)

Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: Implications for treatment. J Neurosurg. 2006. 105: 248-55
16)

Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery. 2001. 48: 55-62
17)

Samii M, Klekamp J. Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery. 1994. 35: 865-73
WhatsApp WhatsApp us
%d bloggers like this: