disease | Hemangioblastoma |
alias | Angioreticuloma, Hemangioblastoma |
Angioreticuloma (also known as hemangioblastoma) is a benign tumor, accounting for 1.3% to 2.4% of intracranial tumors. It is more common in males than females, with a ratio of approximately 2:1, and predominantly occurs in young adults. The disease has genetic factors; Huashan Hospital in Shanghai once documented a family with 11 affected individuals across 4 generations out of 122 members, 6 of which were confirmed cases.
bubble_chart Clinical Manifestations
The time from symptom onset to medical consultation varies from weeks to several years, with most cases occurring within one year. Cerebellar tumors, due to their tendency to compress and obstruct the fourth ventricle, generally have a shorter disease course. In rare cases where the tumor bleeds, symptoms may suddenly worsen.
Approximately 90% of patients experience symptoms of increased intracranial pressure such as headache, vomiting, and papilledema. Prolonged papilledema often leads to visual impairment. Most cerebellar tumors present with nystagmus and ataxia, unsteady gait, and frequently dizziness and diplopia. A minority of cases may exhibit forced head posture, hearing loss, cranial nerve palsies (9th–11th), mild hemiparesis, and unilateral sensory disturbances. Tumors in the cerebral hemispheres may cause varying degrees of hemiparesis, unilateral sensory disturbances, hemianopia, etc., depending on their location.
10–15% of patients exhibit polycythemia, with a corresponding increase in hemoglobin. Peripheral blood counts may show red blood cells reaching 6×10/L and hemoglobin up to 220g/L. After tumor resection, polycythemia gradually returns to normal within 2 weeks to 1 month.
bubble_chart DiagnosisIn adults presenting with symptoms of cerebellar tumors, besides common gliomas, the possibility of this disease should be considered. If concurrent retinal {|###|} hemangiomas, visceral cysts or hemangiomas, polycythemia, or a family history are found, the diagnosis can be essentially confirmed. However, most patients require CT scanning to assist in diagnosis and localization.
CT scans of solid tumors typically show round or oval high-density images with uneven density, while cystic lesions appear as low-density areas, though generally higher in density than simple cysts, with poorly defined margins. Sometimes, high-density patches protruding into the cystic cavity can be observed. Post-contrast scans show image enhancement. A low-density edema zone may surround the tumor.
bubble_chart Treatment Measures
Surgical removal of the tumor can be curative. For cystic tumors, after confirmation by exploration and puncture, the cystic cavity is first opened to aspirate the cystic fluid. The tumor nodules protruding into the cyst from the cyst wall are then dissected along their periphery and completely excised. For tumor nodules that are not visibly protruding but hidden within the cyst wall, a thorough search should be conducted. Areas with abnormal color or thickness should be explored and dissected to locate and remove the tumor nodules. Simple drainage of the cyst only provides temporary symptom relief, with symptoms often recurring within a few years. Generally, cystic tumors are easier to completely remove and can be cured, with a low mortality rate of less than 5%. Solid tumors are inherently difficult to completely excise and may be treated with radiotherapy, though the efficacy is relatively poor.