| disease | Cerebellar Hemangioblastoma |
Hemangioblastoma of the central nervous system is a tumor derived from immature vascular-forming tissues, with congenital characteristics, accounting for 1%-2% of intracranial tumors. The peak age of onset is 30-40 years, with a male-to-female ratio of approximately 2:1, following an autosomal dominant inheritance pattern. The vast majority are located below the tentorium, with only 12%-14% occurring above it. Clinical manifestations mainly include chronic intracranial hypertension, unilateral cerebellar dysfunction, or hemiplegia, and may be associated with retinal hemangiomas or visceral cysts. These tumors are benign and classified into cystic and solid types, with the cystic type being more common. Surgical resection can achieve a cure, and early diagnosis and treatment are crucial.
bubble_chart Clinical Manifestations
1. Headache, vomiting, papilledema. 2. Horizontal nystagmus, unsteady gait, limb coordination dysfunction or hemiplegia, hemisensory impairment, etc. 3. Retinal hemangioma or other congenital visceral diseases such as polycystic kidney, liver cysts, etc.
1. Chronic intracranial hypertension syndrome. 2. Unilateral cerebellar dysfunction or other localized neurological signs. 3. Family history. 4. Retinal hemangiomas may be present, or other congenital visceral abnormalities such as polycystic kidneys or associated polycythemia may occur. 5. Cerebral angiography during the arterial phase may reveal a rounded tumor nodule below (or above) the tentorium, or a relatively avascular area with fewer vessels, into which one (or more) well-defined and vascularized nodules protrude, forming a "ring-like" appearance. Head CT shows an isodense or heterogeneously dense area with clear borders and significant enhancement; another manifestation is a low-density cystic area, with a nodule visible after enhancement, possibly accompanied by obstructive hydrocephalus. Head MRI reveals that the cystic lesion appears hypointense on T1-weighted images and hyperintense on T2-weighted images, while the wall nodule and solid lesion appear isointense on T1-weighted images and hyperintense on T2-weighted images, also demonstrating the tumor's blood supply and draining vessels.
1. Surgical treatment: The preferred option is complete resection. For cystic cases, the entire wall nodule should be removed, while the cyst wall may not need excision. Avoid tumor puncture, biopsy, or premature piecemeal resection. If the tumor invades the brainstem and is confirmed unresectable, biopsy or partial resection should also be avoided. For multiple lesions, staged resection is recommended. 2. Radiotherapy: Only suitable for unresectable cases, with uncertain efficacy. 3. Stereotactic radiotherapy (gamma knife or X-knife). 4. Infection prevention and symptomatic treatment.
Expert advice: This tumor is benign, and early diagnosis and treatment yield good outcomes. Complete surgical resection can achieve a cure. If symptoms such as significant headache, vomiting, unsteady gait, or poor limb coordination occur, the possibility of this disease should be considered. A family history or other congenital visceral abnormalities further support the diagnosis. Consult a specialist for evaluation. Diagnosis can be confirmed via head CT, MRI, or cerebral angiography. For multiple lesions, staged surgery is recommended, with long-term postoperative follow-up. Recurrence can be treated with repeat surgery if the patient's condition permits. Radiotherapy has poor efficacy, and to date, no medication or dietary intervention has been found to shrink or eliminate the tumor.
1. Cure: The tumor is completely removed, symptoms of intracranial hypertension disappear, and cerebellar symptoms improve. 2. Improvement: The tumor is not removed, but simple cyst drainage or shunt surgery is performed, with symptoms of intracranial hypertension alleviated. 3. No recovery: The tumor is not removed, and symptoms show no improvement.
