Spinal haemangioblastoma

Clinical History

A patient with a history of chronic cervical pain was referred with gradually increasing quadriparesis over the previous 2 months.

Imaging Findings

A patient with a history of chronic cervical pain was referred with gradually increasing quadriparesis over the previous 2 months. Cranial and cervical MRI studies were performed on a 1.5T MRI scanner, with SE T1-weighted, FSE T2-weighted, FLAIR, and post-contrast SE T1-weighted sequences in the sagittal, axial and coronal planes. An additional dynamic contrast-enhanced MR angiographic study was also performed.

A well-circumscribed intradural-extramedullary mass lesion was detected at the C1-C2 level; the mass caused serious compression on the spinal cord. The mass was isointense to grey matter on T1-weighted images, and hypointense to grey matter with a stippled appearance on T2-weighted images. An enlarged signal-void vascular structure was detected at the upper pole of lesion. Following IV gadolinium administration, the mass revealed intense contrast enhancement with a large venous structure at its upper pole. The dynamic MRA study demonstrated the hypervascular characteristics of the mass with a large drainage vein at the upper pole of the mass. The radiological diagnosis of haemangioblastoma was confirmed on surgery.

Discussion

Haemangioblastomas are uncommon benign vascular neoplasms of the central nervous system. They constitute 2% of all spinal tumours. The tumours usually develop in adulthood, with a mild male predominance. The most common locations for spinal haemangioblastomas are intramedullary (75%), radicular (20%), and extramedullary (5%) sites. 90% of the tumours are solitary, and they are commonly found in the cervico-dorsal region. Almost one third of the tumours are associated with von Hippel-Lindau disease. Almost 60% of spinal haemangioblastomas are cystic with a mural nodule, and 40% are solid in gross pathological sections. Haemorrhage, calcification, and necrosis are rare in haemangioblastomas. Histopathologically, they are heterogeneous tumours with endothelial pericytic and interstitial stromal cells.

Computed tomography (CT) reveals a cystic mass which is isodense with cerebrospinal fluid (CSF), and containing a strongly contrast-enhancing peripheral mural nodule in most cases. Magnetic resonance imaging (MRI) reveals the cystic component of the lesion as hyperintense to CSF in all spin-echo sequences. The solid component has variable signal intensity characteristics, but it is usually hypointense on all sequences and strongly enhances with contrast material. Large draining veins are detected at the poles of the mass as signal-void curvilinear structures. Syringomyelia and medullary oedema may develop with haemangioblastomas. Angiography shows a densely and prolonged stained tumour nidus within an avascular cyst in addition to the large drainage veins. Although haemangioblastomas are potentially curable lesions, almost 25% of the tumours recur following surgical resection. Spinal haemorrhage (both intramedullary and subarachnoid) may develop as a complication of haemangioblastomas. Differential diagnosis involves cystic astrocytoma, and cystic metastases. Cystic astrocytomas have frequent calcifications within larger nodules, the cyst wall is very thick, and angiographic blushing of the mural nodule is absent. Metastases are very rare and can be ruled out by clinical history and the presence of extensive surrounding oedema.

Differential Diagnosis List

Final Diagnosis

Spinal haemangioblastoma

URL:	https://www.eurorad.org/case/1766
DOI:	10.1594/EURORAD/CASE.1766
ISSN:	1563-4086