title: [Intraspinal hemangioblastomas].
abstract: 138 cases of spinal haemangioblastomas have been reviewed (30 original cases). The first part of this study is devoted to a general survey concerning history, pathological anatomy, genetics and nosology of haemangioblastomas of the central nervous system. Microscopic anatomy of these tumors has been a much debated question and is responsible for the numerous denominations proposed: angiomatosis, capillary angiomas, angioreticulomas, haemangiomas, haemangioendotheliomas, haemangiopericytomas, angioblastic meningiomas; haemangioblastoma seems the most correct. Recent studies in optical and mainly electron microscopy do not allow exact typing of tumoral cells and histogenesis remains uncertain. It is generally accepted that the tumor arises in vascular nesenchyme as a result of dygenetic abnormality. This theory provides a rational explanation for visceral localizations and classification of Lindau disease among phakomatoses. An hereditary factor is present in 25 percent of cases, mainly in multifocal localizations. In the second part, interest is focused on spinal haemangioblastomas, 17 extradural, 121 subdural haemangioblastomas are studied and listed. Extradural tumors are always solitary, mostly in the dorsal area. Subdural tumors may be out of the cord (10 tumors), on the posterior roots (28 tumors), or within the cord. The lesions are situated mainly in the cervicodorsal and dorsolumbar regions. Intramedullary haemangioblastomas are situated in nearly all cases in the dorsal part of the cord behind the central canal. They are close to the dorsal surface of the cord (60 percent) or partly out of the cord (6 percent) or totally intramedullary (30 percent). Cysts close to the tumor or at a distance are present in nearly 50 percent of cases and their origin is still questionnable. Clinical patterns have no special peculiarities, syrinomyelic symptomatology occurs in only 25 percent of cases. Neuroradiology and mainly spinal angiography are a very important step. Angiography was performed in 23 cases (36 tumors). Feeding vessels arise from the posterior system (33 cases) and the anterior system (19 cases). A dark blush indicates the tumor, disappears within 16 to 35 seconds, and can be easily differentiated from arterio venous malformations. The opportunity for spinal angiography depends on clinical and radiological criteria as well as the natural history of the disease. Total removal is the only effective treatment of haemangioblastomas of the cor. Accurate localization of feeding arteries and venous drainage through spinal angiography, preoperative embolization if possible and microsugical techniques applied to cord lesions allow thie ideal treatment. But a lot of problems remain unsolved.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000792:Angiography; D002648:Child; D005260:Female; D006394:Hemangiosarcoma / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D006801:Humans; D007796:Laminectomy; D008297:Male; D008875:Middle Aged; D009192:Myelography; D010375:Pedigree; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D014711:Vertebral Artery / Q000000981:diagnostic imaging
vernacular_title: Les hémangioblastomes intrarachidiens