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Tumours of Vascular Origin

Revised: June 2014

The most common CNS tumors of vascular origin are hemangioblastomas and hemangiopericytomas. Other, more rare tumours, include hemangioendothelioma and angiosarcoma.

Hemangioblastomas represent the most common primary intra-axial tumor in the posterior fossa in the adult. They can, however, be found virtually anywhere in the CNS as well as elsewhere in the body. Hemangioblastomas can occur sporadically or, in 20% of cases, in association with Von Hippel Lindau syndrome. The latter is an autosomal dominant, multiple organ neoplastic syndrome with high penetrance. In addition to the CNS hemangioblastomas, patients can also harbor retinal angiomas, renal cell carcinoma, cysts of abdominal organs and suffer from polycythemia. Classically the cerebellar lesions are seen as cystic masses with an enhancing nodule. However solid tumor masses are also seen. Predictably the tumor is highly vascular and is histologically benign. As such ideal treatment is surgical resection. The resection may be rendered more facile by pre-operative embolization of the tumor. The tumors can be multiple, asymptomatic and/or reside within eloquent regions of the brain. For any or all of these reasons surgical resection may not be feasible. In this instance radiation therapy can be considered. There are variable reports of success using stereotactic radiosurgery as a means of treatment. At the present time its role is not clear, but remains a potential alternative.

Hemangiopericytomas are mesenchymal tumors arising from the meninges. Originally classified as angioblastic meningiomas, they are now recognized as a meningeal form of solitary fibrous tumor. They are considered highly aggressive, if not malignant neoplasms, which distinguishes them from the mostly benign meningiomas. They present clinically, and are seen on imaging studies, very much like meningiomas. Primary treatment is an attempt at complete surgical resection. Surgery is often hampered by the vascular nature of the tumor and its propensity to bleed. Because of this, pre-operative angiography and embolization is ideal. Tumor recurrence and progression of residual tumor is common. Metastases have also frequently been recorded. Subtotal removal generally mandates a course of post-operative radiation therapy. Stereotactic radiosurgery has been used with success, but its definitive role is yet to be determined.

SOURCE: Tumours of Vascular Origin ( )
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