Hemangioblastoma Diagnosis
The diagnosis of hemangioblastoma may include inquire about your symptoms and medical history, physical, and can be confirm diagnosed by perform certain test include neurological examination, computed tomography (CT) or magnetic resonance imaging (MRI) scan or ultrasound, and cerebral angiography.
Perform blood tests to help reveal associated lesions that may be a part of the VHL disease complex. When a family history of von Hippel Lindau is present, there is a strong likelihood that other directly descended family members will be afflicted by this disorder.
Hemangioblastomas are best diagnosed with magnetic resonance imaging (MRI). MRI visualize hemangioblastomas because it is highly sensitive and provides better anatomic definition than computed tomography (CT). Further, the multiplanar capabilities of MRI are ideally suited to provide maximal anatomic definition of these lesions. An additional advantage to MRI is that it is better suited for imaging lesions in the posterior fossa where hemangioblastomas have a predilection to occur. Cerebellar and supratentorial hemangioblastomas typically demonstrate a sharply marginated smooth cyst border and an enhancing tumor nodule. Prior to the administration of a gadolinium-containing contrast material, the mural module is hypointense to isointense on T1-weighted and is hyperintense on T2-weighted images. Administration of an MR contrast agent typically demonstrates intense enhancement of the mural nodule. Non-cystic hemangioblastomas enhance uniformly following the administration of the contrast agent. Spinal cord hemangioblastomas may be characterized by an intramedullary vascular nodule, enlarged draining veins, diffuse enlargement of the cord, or an intramedullary cyst.
On computed tomography (CT), hemangioblastomas typically enhance intensely after the injection of a contrast agent, with either a homogeneous or a mottled appearance; in some cases there may be a high-density rim with central lucency from an intratumoral cyst. Dilated vessels may be seen in the vicinity of the tumor. The cystic tumors appear as sharply-defined, low-density lesions with or without a mural nodule. The attenuation values of the cyst may be the same as or slightly higher than those of cerebrospinal fluid. Bony artefact may be problematic in imaging the posterior fossa. The mural nodule, if present, enhances intensely on contrast injection, but the cyst margin itself generally does not enhance. If a cyst is seen alone, without a nodule, in a patient with known VHL complex, one has to conclude that the nodule is too small to be defined by CT. In such instances, angiography is mandatory to locate the mural nodule, because the cyst is bound to recur unless the mural nodule is removed. Typically, solid tumors are uniform, isodense or hyperdense, and enhance with contrast. Important CT characteristics include marked contrast enhancement of the nodule, proximity of the mural nodule to the pial surface, large size of the cystic component of the tumor compared with the mural nodule, and isodensity of the nodule on preinfusion CT.
Cerebral angiography reveals a highly vascular tumor blush, and this diagnostic modality may be extremely useful for assessing the vascular supply to the tumor. This is often used to determine the degree of vascularity.
In patients with hemangioblastomas, complete neural axis imaging usually is recommended in order to rule out multiple lesions, especially in those cases in which VHL syndrome is either diagnosed or clinically suspected.