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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

Cerebellar hemangioblastoma clinical features.

Diagnosis

Cerebellar hemangioblastoma 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

see Cerebellar hemangioblastoma treatment.

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, 7) 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 8).


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 9).

Case reports

Cerebellar hemangioblastoma case reports.

Cerebellar hemangioblastoma associated with Von Hippel-Lindau disease

Cerebellar hemangioblastoma associated with Von Hippel-Lindau disease.

Unclassified

see Cerebellar hemangioblastoma unclassified

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)
Cushing H, Bailey P. Tumors arising from blood vessels in the brain: angiomatous malformations and hemangioblastomas. Springfield, IL: Charles C Thomas; 1928.
8)
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.
9)
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.
cerebellar_hemangioblastoma.txt · Last modified: 2020/03/29 13:57 by administrador