Cervical hemangioblastoma (Haemangioblastoma)
Spinal cord hemangioblastoma is characterized by prominent hypervascularity and specific tomographic alterations.
Sagittal T1- and T2-weighted MRI visualize diffuse thickening of the spinal cord. This tumor is typically characterized by extensive edema and the formation of multiple cysts that may extend cranially up to the level of the obex (obex).
Administration of gadolinium contrast (on T1-weighted imaging) reveals the following features (demonstrated on a C7 segment lesion):
- The tumor tissue intensely enhances with the contrast agent.
- Areas of signal loss—the so-called "flow voids" (flow voids)—are identified within the lesion, caused by very rapid blood flow through the vessels.
- Dilated veins that also enhance with contrast are well-delineated along the posterior surface of the spinal cord.
Digital subtraction angiography (DSA) is used for a detailed assessment of the blood supply. It confirms hypervascularity and allows identification of feeding afferents, such as dilated arterial branches from the right vertebral artery (a. vertebralis dextra) and the right thyrocervical trunk (truncus thyrocervicalis dexter).
Intramedullary cavernous malformations
Cavernous malformations (cavernomas) have an extremely specific appearance on MRI. The classic pattern is described as a "mulberry" or "popcorn" appearance. This effect is created by the alternation of areas of high and low signal intensity.
- On T1WI: the lesion is spontaneously partially hyperintense, with only moderate enhancement observed after contrast administration.
- On T2*-weighted gradient-echo: this sequence is optimal for visualizing signs of chronic hemorrhage (hemoglobin breakdown products), which is especially important for detecting small cavernomas.
- Perifocal edema around an intact cavernoma is absent.
Important clinical rule: when an intramedullary cavernoma is detected, the patient is always recommended to undergo a brain MRI. This is due to the frequent association of the pathology with familial cavernous angiomatosis. Brain susceptibility-weighted imaging (SWI) in such cases reveals multiple cavernomas, confirming the familial nature of the disease.
Ganglioglioma and extramedullary tumors
Ganglioglioma (Ganglioglioma) is frequently encountered in early childhood and involves extensive segments (e.g., 5–6 thoracic segments). On MRI (T1 and T2), it appears as a heterogeneous, solid, infiltrating tumor. Its structure reveals small intratumoral cysts surrounded by moderately pronounced peritumoral edema. Upon gadolinium enhancement, contrast uptake is diffuse, heterogeneous, and patchy in nature.
Intradural extramedullary tumors originate from the meninges (meninges) or spinal nerve roots. In most cases, they are benign.
- MRI appearance: the tumor leads to displacement of the spinal cord to the contralateral side and widening of the ipsilateral subarachnoid (cerebrospinal fluid) space.
- Clinical presentation: manifested by progressive myelopathy, muscle weakness, as well as diffuse or radicular pain.
Differential Diagnosis on MRI
When identifying lesions in the spinal cord, it is important to differentiate vascular tumors from other pathologies:
- Spinal cord metastases. T2-weighted imaging shows isolated hyperintense areas centrally within the spinal cord (e.g., at Th8, Th11), but obvious edema is absent. On contrast-enhanced T1WI, the lesions actively take up the contrast agent. They are frequently associated with brain metastases (e.g., in breast cancer).
- Neuromyelitis optica (Devic's disease). T2-weighted imaging reveals a poorly marginated area of hyperintense signal extending longitudinally across 3–4 segments, with moderate spinal cord thickening. Contrast enhancement is uneven. The diagnosis is confirmed clinically (optic neuritis) and by a normal brain MRI.
Briefly: answers to topical questions
Which genetic syndrome is most commonly associated with spinal cord hemangioblastomas?
Spinal cord hemangioblastomas are associated with von Hippel–Lindau syndrome (VHLs). This is an autosomal dominant disorder in which the lifetime risk of developing spinal hemangioblastomas reaches 88%.
Features of tumors in this syndrome compared to sporadic cases:
| Characteristic | Von Hippel–Lindau syndrome | Sporadic cases |
|---|---|---|
| Number and localization | Multiple lesions at all levels of the spinal cord | Usually a solitary lesion at the cervical or thoracic level |
| Age of detection | Diagnosed on average 10 years earlier | Peak incidence in the 4th decade of life |
| Symptoms | Less pronounced neurological symptoms | More pronounced |
Patients with this syndrome are indicated for spinal MRI screening; up to 1/3 of patients develop new lesions every 2 years.
Which arteries act as afferents for cervical hemangioblastoma according to angiography?
According to digital subtraction angiography, the afferents of cervical hemangioblastoma are dilated arterial branches. The angiographic study demonstrates the hypervascularity typical of this tumor.
Blood supply is provided by the following vessels:
- Right vertebral artery (a. vertebralis dextra) — gives off feeding branches to the vascular lesion.
- Right thyrocervical trunk (truncus thyrocervicalis dexter) — also participates in forming the afferent bed of the tumor.
To recognize a cavernoma on MRI, think of popcorn or a mulberry—this is precisely what the variegated mixture of blood breakdown products of varying ages looks like.
Class and exam questions
What is the "flow void effect" on MRI?
These are areas of signal loss within the enhancing tumor. They occur due to very rapid blood flow through pathological vessels, which prevents the scanner from capturing the signal from magnetized protons (characteristic of hemangioblastomas).
Why is a brain scan mandatory in intramedullary cavernoma?
Spinal cavernomas are often a manifestation of familial cavernous angiomatosis. Brain scanning (especially in SWI sequence) helps detect multiple asymptomatic cavernomas and confirm the genetic nature of the disease.
How to differentiate spinal cord metastases from primary hemangioblastoma?
Metastases present as isolated central lesions without obvious extensive edema and without the formation of cranially extending cysts. Metastases actively enhance with contrast, but lack large feeding vessels and the "flow void" effect.
What else to study on this topic
- Clinical significance of spinal cysts extending to the level of the obex.
- MRI physics: why the T2* gradient-echo sequence shows hemoglobin breakdown products better than others.
- The role of digital subtraction angiography in planning surgical resection of vascular tumors.
- Pathogenesis of muscle weakness and radicular pain in intradural extramedullary tumors.
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