China
Africa
Explore chapters and articles related to this topic
Pediatric Central Nervous System Tumors as Phenotypic Manifestation of Cancer Predisposition Syndromes
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Giorgio Perilongo, Irene Toldo, Stefano Sartori
The clinical history of hemangioblastoma is unpredictable. No clinical and/or biological markers are available to predict their behavior. They may remain dormant for long periods of time, but in some instances they may demonstrate growth capacity. A conservative neurosurgical approach should be the therapeutic concept guiding the treatment of hemangioblastomas, recognizing that surgery remains the cornerstone of treatment. Recently, radiosurgery has been used successfully for smaller lesions in critical locations. The National Institutes of Health (NIH) recommend that contrast-enhanced MRI of the brain and spinal cord should be performed for every patient with VHL every 2 years from the age of 11.72
Spinal Cord Angiography
Published in Milosh Perovitch, Radiological Evaluation of the Spinal Cord, 2019
Hemangioblastoma — This has been more often diagnosed by means of angiogra-phy, and its angiographic features are quite characteristic. This tumor is ususually well opacified, and it appears like a tumor stain in a form of larger or smaller nodules that represent clustered, sinusoidal, vascular channels with very thin walls. Sometimes, he-mangioblastoma may receive blood from different sources and will have an irregular arrangement of vessels on angiograms, di Chiro demonstrated on angiograms, the oc-currence of bleeding within a hemangioblastoma.90 Multiple hemangioblastomas of the spinal cord happen quite often, and for this reason an angiographic investigation of the entire spinal cord is indicated if one of these lesions is detected (Figure 9). Now and then, hemangioblastomas may be difficult to differentiate from arteriovenous mal-formations of the spinal cord. However, in hemangioblastomas, angiography demon-strates an early opacification of blood supply in form of a network of fine vessels. Later, the filling of characteristic vascular lakes takes place within the tumor. The capillary blush is well delineated and persistent, whereas draining veins appear on an-giograms later. 123, 125As hemangioblastomas were already discussed in the chapter deal ing with tumors of the spinal cord, they have been touched upon here only in conjunc tion with their angiographic appearance.
Brain
Published in Joseph Kovi, Hung Dinh Duong, Frozen Section In Surgical Pathology: An Atlas, 2019
Joseph Kovi, M.D. Hung Dinh Duong
Hemangioblastoma is a relatively uncommon intracranial neoplasm.151 It is recommended that in the assessment of a potential hemangioblastoma the frozen section be stained for neutral fat. The presence of large numbers of intracellular sudanophilic lipid droplets is virtually diagnostic of this tumor.151,159
Current concepts on ocular vascular abnormalities in the phakomatoses
Published in Seminars in Ophthalmology, 2021
Alessandro de Paula, Solmaz Abdolrahimzadeh, Serena Fragiotta, Mariachiara Di Pippo, Gianluca Scuderi
Hemangioblastoma in VHL can be treated with laser therapy,106–109 cryotherapy,100,110–113 plaque radiotherapy,114 external beam radiation, proton beam radiation, photodynamic therapy (PDT),115,116 transpupillary thermotherapy, and intravitreal anti-VEGF drugs.97 Vitrectomy can be considered in cases of tractional retinal detachment.90,117,118 Observation is suggested in the case of asymptomatic hemangioblastomas without signs of exudation.119 The anti-VEGF treatment showed conflicting results. In some reports, visual acuity was improved in patients treated with anti-VEGF alone120 or combined with other therapies.120,121 However, Dahr et al described the progression of the disease in 3 of 5 patients treated with pegaptanib,122 and Wong did not report success with ranibizumab.123 The contrasting results may be due to the variable size of lesions and the intrinsic characteristics of incessant VEGF production owing to the genetic nature of the condition.
A mobile hemangioblastoma of the cauda equina: Case report and review of the literature
Published in The Journal of Spinal Cord Medicine, 2020
Haitao Luo, Juexian Xiao, Shigang Lv, Xingen Zhu, Zujue Cheng
The phenomenon of migratory intradural extramedullary tumors has been reported in many countries since 1963.1 The most common migratory intradural extramedullary tumors of the spine are schwannomas, with some reported cases of neurenteric cysts2 and ependymomas.3-4 Hemangioblastoma is a highly vascularized benign tumor of the central nervous system, commonly found in the spinal cord, cerebellum, and brainstem.5 Spinal hemangioblastoma accounts for nearly 13–26% of all hemangioblastomas.6 Patients with spinal hemangioblastoma usually experience pain and exhibit signs of segmental and long-tract dysfunction, which are caused by syringomyelia associated with a mass effect due to increasing tumor size.7 About 30% of hemangioblastomas occur as a characteristic component of von Hippel Lindau syndrome (VHL), while about 70% occurs as sporadic lesion.8-10
Supratentorial hemangioblastomas in von Hippel–Lindau wild-type patients – case series and literature review
Published in International Journal of Neuroscience, 2018
Luís Rocha, Carolina Noronha, Ricardo Taipa, Joaquim Reis, Mário Gomes, Ernesto Carvalho
Hemangioblastoma of the central nervous system (CNS) are solid or cystic vascular-rich tumours, originated from mesoderm remnants incorporated into the brain during the third foetal month. It represents 1.1%–2.4% of all intracranial tumours, classically located in the cerebellum or spinal cord [1–3]. The entity was first described by von Hippel in 1895 [4] and Bielschowsky in 1902 [5], but the term hemangioblastoma (HBL) was proclaimed by Cushing and Bailey in 1928 [6,7]. In 1938, Gushing and Eisenhardt characterized 23 diverse vascular meningeal-based tumours into three groups: angioblastic, transitional angioblastic and angioblastomatous meningioma [8]. This classification was later revised in the 1989 by the neuropathologists Russell and Rubinstein, defining the last variant into hemangioblastoma [9]. Although this tumour is histologically benign, pathogenic action results from mass effect and peritumoural oedema [1]. Generally, it is considered to be a non-metastasizing tumour [10], and diagnosis, in most of the cases, is often delayed due to the absence of clinical signs. Due to its rarity, information regarding supratentorial HBL clinical characteristics, treatment and prognosis remains sparse. The available literature is mostly composed of case reports and small case series, mainly associated to von Hippel–Lindau disease (VHL) [3].