Hemangioblastoma MRI

The combination of a peripheral posterior fossa cyst with a mural nodule supplied by enlarged vessels may be pathognomonic 1).

Based on MRI findings, there are several known types of this tumor. The most common type consists of small nodular tumors on the side of a large cyst and the two rarer types comprise a solid mass, or a lesion with an enhanced cyst wall due to cystic nodules, which exhibits enhanced uneven walls on imaging 2).

Postcontrast MR of the head and spine is the best currently available means of detecting hemangioblastomas associated with VHL 3).

At present, no unified radiological classification system based on magnetic resonance imaging (MRI) findings exists for cerebellar hemangioblastoma, and this tumor type can be solid or cystic mass, according to the MRI findings. The most common presentation of cerebellar hemangioblastoma observed radiologically is a large sac with small nodules, where the wall of the large cyst is not enhanced. A tumor with enhanced large cysts and tumor nodules is extremely rare 4).

T1

hypointense to isointense mural nodule

CSF signal cyst content

T1 C+ (Gd)

mural nodule vividly enhances 5) cyst wall does not enhance 6).

T2

hyperintense mural nodule flow voids due to enlarged vessels may be evident especially at the periphery of the cyst, seen in 60-70% of cases 7) fluid filled cyst, similar to CSF

MR perfusion imaging:

high rCBV ratios

ADC and normalized ADC are valuable in the differential diagnosis of posterior fossa solid hemangioblastoma(PFSH) from other tumors with abundant blood supply 8).

1)
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.
2)
Aldape KD, Plate KH, Vortmeyer AO, et al. Haemangioblastoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Nervous System. IARC Press; Lyon: 2007.
3)
Filling-Katz MR, Choyke PL, Patronas NJ, Gorin MB, Barba D, Chang R, Doppman JL, Seizinger B, Oldfield EH. Radiologic screening for von Hippel-Lindau disease: the role of Gd-DTPA enhanced MR imaging of the CNS. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):743-55. PubMed PMID: 2778131.
4)
Sun Z, Yuan D, Sun Y, Yan P, Zuo H. Surgical resection of cerebellar hemangioblastoma with enhanced wall thickness: A report of two cases. Oncol Lett. 2015 Apr;9(4):1597-1599. Epub 2015 Feb 10. PubMed PMID: 25789007; PubMed Central PMCID: PMC4356399.
5)
Bonneville F, Sarrazin JL, Marsot-Dupuch K, Iffenecker C, Cordoliani YS, Doyon D, Bonneville JF. Unusual lesions of the cerebellopontine angle: a segmental approach. Radiographics. 2001 Mar-Apr;21(2):419-38. PubMed PMID: 11259705.
6) , 7)
Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ. Radiologic-pathologic correlation: hemangioblastoma. AJNR Am J Neuroradiol. 1992 Sep-Oct;13(5):1343-52. PubMed PMID: 1414827.
8)
Hao MZ, Zheng LM, Ma XX, Hu HC, Xing MJ, Zhu FH. [Value of Diffusion-weighted Imaging and Apparent Diffusion Coefficient in the Differential Diagnosis of Posterior Fossa Solid Hemangioblastoma]. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2019 Dec 30;41(6):756-760. doi: 10.3881/j.issn.1000-503X.11188. Chinese. PubMed PMID: 31907124.