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Cerebellar Hemangioblastoma

Cerebellar Haemangioblastoma


Presentation

  • A 72-year-old man presented with a gradual bilateral decrease in visual acuity. Imaging showed lesion in the sella turcica diagnosed as meningioma but proving secondarily to be a hemangioblastoma.[ncbi.nlm.nih.gov]
  • A 45-year-old male presented with sudden onset of severe headache, mild disorientation, and gait disturbance due to intracystic hemorrhage from a cerebellar hemangioblastoma.[ncbi.nlm.nih.gov]
  • An example of a form of cystic hemangioblastoma of the cerebellum in which the mural nodule itself showed a central cystic area is presented.[ncbi.nlm.nih.gov]
  • A 38-year-old woman with von Hippel-Lindau disease presented with a recurrent cerebellar hemangioblastoma despite two previous operations during which the mural nodule was removed but the cyst wall was not.[ncbi.nlm.nih.gov]
  • In the present case, we documented a distinctive histological appearance of perivascular pseudorosettes in hemangioblastoma and provided the evidence for stromal cells with glial differentiation.[ncbi.nlm.nih.gov]
Fever of Unknown Origin
  • A 51-year-old female, with a past medical history of resection of cerebellar hemangioblastoma, presented with an atrial tumor detected by sonography after undergoing a series of examinations for fever of unknown origin and subsequent symptoms of heart[ncbi.nlm.nih.gov]
Hiccup
  • The patient has been free of hiccups and has been neurologically intact since the day after total removal of the tumor. A review of the literature on medullary lesions presenting with intractable hiccups is provided.[ncbi.nlm.nih.gov]
Myopathy
  • […] of circular, laminated filaments, and longitudinally arranged filaments; and appear similar to the so-called "Hirano bodies", which have been previously found in normal and various pathologic conditions of the brain tissue, skeletal muscle cells in myopathy[ncbi.nlm.nih.gov]
Psychomotor Retardation
  • She presented dysmorphic facial features, growth retardation and severe psychomotor retardation associated with 3p deletion syndrome.[ncbi.nlm.nih.gov]
Ataxia
  • His symptoms included episodes of loss of consciousness, cerebellar ataxia, and postural vertigo. The clinical diagnosis presented considerable difficulties. The simultaneous occurrence of both tumors in one patient has not been reported previously.[ncbi.nlm.nih.gov]
  • A 28-year-old woman with ataxia and left-sided weakness was diagnosed with cerebellar hemangioblastoma in the second trimester of pregnancy following repeated hospital admissions for nausea and vomiting.[ncbi.nlm.nih.gov]
  • Hydrocephalus, gait or appendicular ataxia was the initial clinical presentation in these patients.[ncbi.nlm.nih.gov]
  • We report a 48-year-old man who was diagnosed with a large cerebellar mass after presenting with headaches and gait ataxia.[ncbi.nlm.nih.gov]
  • Relationship to von Hippel-Lindau disease 20% occur in patients with von Hippel-Lindau disease (multiple lesions). 35-60% of von Hippel-Lindau disease patients have hemangioblastomas von Hippel-Lindau disease Hemangioblastomas Clinical findings Headache Ataxia[learningradiology.com]
Cerebellar Ataxia
  • His symptoms included episodes of loss of consciousness, cerebellar ataxia, and postural vertigo. The clinical diagnosis presented considerable difficulties. The simultaneous occurrence of both tumors in one patient has not been reported previously.[ncbi.nlm.nih.gov]
  • "Riluzole in cerebellar ataxia: a randomized, double-blind, placebo-controlled pilot trial." Neurology 74(10): 839-845. Romano, S., et al. (2015).[dizziness-and-balance.com]
  • View/Print Table Summary Table Condition Characteristics Ataxia-telangiectasia Telangiectasia of skin and conjunctiva, cerebellar ataxia Sturge-Weber syndrome Congenital facial angioma, glaucoma, choroidal hemangioma, leptomeningeal hemangioma Tuberous[aafp.org]
Nystagmus
  • This patient was very unsteady and had strong positional nystagmus due to removal of her cerebellar nodulus.[dizziness-and-balance.com]
  • At the time of visit to our hospital, she presented with right dominant gaze rotatory nystagmus and right cerebellar hemisphere symptoms.[surgicalneurologyint.com]
Cerebellar Sign
  • The cerebellum demonstrates functional localization; cerebellar signs often correlate with the location of the cerebellar lesion. 2 The deep cerebellar nuclei have specific deficits when damaged.[clinicalgate.com]
  • More about cerebellar signs in the saccadic portions of the ENG/rotatory chair test is found here. Genetic testing in sporadic ataxia (i.e. with no family history) is very low yield.[dizziness-and-balance.com]
Tremor
  • Intention tremors may be present on an attempt to touch an object. A kinetic tremor may be present in motion. The finger-to-nose and heel-to-knee tests are classic tests of hemispheric cerebellar dysfunction.[dizziness-and-balance.com]
  • Patients may also present with ataxic tremor, dysarthria, postural instability, or gait disturbances.[clinicalgate.com]

Workup

  • Such a workup should include imaging of the chest (via chest x-ray and/or CT scan of the chest) and also CT of the abdomen and pelvis.[clinicalgate.com]

Treatment

  • Thorough coverage for each neurological disease clearly defines age at onset, course of illness, clinical features, and treatment options. Differential diagnosis tables and treatment algorithms expedite clinical decision making.[books.google.de]
  • Massive hemorrhage from hemangioblastoma is rare but needs prompt treatment because of the relatively high mortality.[ncbi.nlm.nih.gov]
  • Treatment was performed in two stages, and he was discharged with remaining slight ataxic gait. The diagnoses were cerebellar hemangioblastoma and pituitary null cell adenoma.[ncbi.nlm.nih.gov]
  • The treatment is surgical excision of the tumor, as in this case. When cerebellar symptoms occur or a cerebellar mass lesion is detected in an organ recipient, HB should be considered in the differential diagnosis.[ncbi.nlm.nih.gov]

Prognosis

  • However, cerebellar hemangioblastoma occurs in younger patients, is often multiple and recurrent, and has a poorer prognosis than sporadic cases.[jkns.or.kr]
  • These tumors generally have a good prognosis after surgical resection with an 85% to 100% 5-year survival rate. 1, 9, 10 The favorable prognosis in these patients has been attributed to the low-grade nature of cerebellar pilocytic astrocytomas and the[clinicalgate.com]
  • Prognosis [ edit ] The outcome for hemangioblastoma is very good, if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed[en.wikipedia.org]
  • Prognosis Good. he·man·gi·o·blas·to·ma ( hē-man'jē-ō-blas-tō'mă ) A benign cerebellar neoplasm composed of capillary vessel-forming endothelial cells and stromal cells; a slowly growing tumor that affects, primarily, middle-aged individuals; increased[medical-dictionary.thefreedictionary.com]
  • ., argon laser photocoagulation, partial nephrectomy, and neurosurgical excision in non-stable lesions Prognosis, Prevention, and Complications Complications includes renal cell carcinoma and hemangioblastoma Please rate topic.[medbullets.com]

Etiology

  • Although the precise etiology of the cystic component within hemangioblastoma tumor nodules is not known, it probably results from tumor degeneration.[ncbi.nlm.nih.gov]
  • Abstract quote Central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord.[thedoctorsdoctor.com]
  • Etiology of the hemangioblastoma is obscure, but its presence in various clinical syndromes may suggest an underlying genetic abnormality.[emedicine.medscape.com]

Epidemiology

  • "Hemangioblastomas in the elderly: epidemiology and clinical characteristics.". J Clin Neurosci 21 (7): 1205-8. doi : 10.1016/j.jocn.2013.10.023. PMID 24629394. URL: . Accessed on: 9 December 2010. URL: . Accessed on: 23 June 2010.[librepathology.org]
  • Clin Neuroradiol 20:5–24 Google Scholar Laakso A, Hernesniemi J (2012) Arteriovenous malformations: epidemiology and clinical presentation.[link.springer.com]
  • Hemangioblastomas in the elderly: epidemiology and clinical characteristics. J Clin Neurosci. 2014 Jul. 21 (7):1205-8. [Medline]. Yoon JY, Gao A, Das S, Munoz DG.[emedicine.medscape.com]
  • Persons with VHL syndrome have a bleaker prognosis than those who have sporadic tumors since those with VHL syndrome usually have more than one lesion. [2] Epidemiology [ edit ] Hemangioblastoma are among the rarest central nervous system tumors, accounting[en.wikipedia.org]
  • Central nervous system hemangioblastomas: Epidemiology, pathology and clinical spectrum in a tertiary care centre. Astrocyte [serial online] 2014 [cited 2019 May 13];1:186-9.[astrocyte.in]
Sex distribution
Age distribution

Pathophysiology

  • Various theories have been proposed to explain the rapid neurologic deterioration of hemangioblastoma patients during pregnancy; however, the pathophysiologic behavior and histogenesis of this vascular tumor are still not well understood.[ncbi.nlm.nih.gov]

Prevention

  • […] wall enhancement demonstrates a correlation between enhancement on magnetic resonance imaging and presence of neoplasia within the cyst wall, as well as the importance of considering complete resection of these areas, including the cyst wall, for the prevention[ncbi.nlm.nih.gov]
  • […] while a complete surgical resection usually provides a definitive cure in sporadic hemangioblastomas. 3,4 Due to the autosomal dominant nature of VHL disease, the patient’s relatives should be screened to detect the manifestations at an early stage and prevent[consultant360.com]
  • Therefore, interventions such as early detection of dissemination, surgical removal of the local tumor, and stereotactic radiation may prevent widespread dissemination.[surgicalneurologyint.com]
  • However, XRT does not prevent regrowth following surgery. Outcome Hemangioblastomas are potentially curable lesions, although they are the cause of death in 82 percent of patients with von Hippel-Lindau syndrome.[neurosurgery.ucla.edu]
  • ., argon laser photocoagulation, partial nephrectomy, and neurosurgical excision in non-stable lesions Prognosis, Prevention, and Complications Complications includes renal cell carcinoma and hemangioblastoma Please rate topic.[medbullets.com]

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