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1. Al-Za'abi AM, Ghazarian D, Greenberg GR, Shaw JC: Eruptive tufted angiomas in a patient with Crohn's disease. J Clin Pathol; 2005 Feb;58(2):214-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Eruptive tufted angiomas in a patient with Crohn's disease.
  • Angioblastoma is a rare, benign vascular tumour composed of undifferentiated mesenchymal cells with a tendency to form lumina.
  • This entity was first described by Nakagawa in 1949 as angioblastoma, and Wilson Jones was the first to use the term "tufted angioma" in 1976.
  • Tufted angiomas usually occur in infancy and spread slowly.
  • This report describes lesions from the right side of the forehead, forearms, and thighs of a 24 year old man with a four year history of Crohn's disease, who was receiving infliximab in addition to long standing azathioprine and ciprofloxacillin.
  • He developed numerous small itchy erythematous vascular appearing papules, which on histological examination resembled tufted angiomas, showing the classic "cannon ball" appearance.
  • This case may represent an eruptive acquired tufted angioma in which immunosuppression or drug induced modification of angiogenesis played a role in its development and regression.
  • One previous case of eruptive tufted angioma has been reported in an immunosuppressed patient.
  • [MeSH-major] Crohn Disease / pathology. Hemangioma / pathology. Skin Neoplasms / pathology

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  • (PMID = 15677546.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Gastrointestinal Agents; B72HH48FLU / Infliximab
  • [Other-IDs] NLM/ PMC1770572
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2. Rohana AG, Norazmi MK, Norlaila M: A rare case of Von Hippel Lindau disease. Med J Malaysia; 2006 Jun;61(2):254-7
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  • [Title] A rare case of Von Hippel Lindau disease.
  • It may occur sporadically or be associated as part of a tumour syndrome including Von Hippel Lindau (VHL), Multiple Endocrine Neoplasia (MEN) 2 and Neurofibromatosis Type 1.
  • VHL is associated with multi-organ involvement of benign and malignant tumours characterized by the presence of retinal angiomas, hemangioblastomas of the cerebellum and spinal cord, renal cell carcinomas, pheochromocytomas and other cystic lesions in the kidneys, pancreas, and epididymis.
  • It is a rare disorder with prevalence estimated at 2-3 per 100,000.
  • This case report describes a 37 years old Chinese gentleman who presented to our institution for further management of bilateral pheochromocytoma and retinal angioblastoma with problems of duodenal ulcer and anaemia.
  • With these features the criteria for the diagnosis of von Hippel Lindau disease was established.
  • [MeSH-major] von Hippel-Lindau Disease / diagnosis

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  • (PMID = 16898326.001).
  • [ISSN] 0300-5283
  • [Journal-full-title] The Medical journal of Malaysia
  • [ISO-abbreviation] Med. J. Malaysia
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Malaysia
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3. Almaraz RL, Gutiérrez JC, Bieler CB, Hernández AH, González ME, Villar GR: [Infantile vascular tumors]. An Pediatr (Barc); 2010 Feb;72(2):143.e1-143.e15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Transliterated title] Tumores vasculares en la infancia.
  • Hemangiomas of infancy are by far the most frequent, and other less common types are congenital hemangiomas (rapidly involuting or RICH and non-involuting or NICH), kaposiform hemangioendothelioma, angioblastoma or tufted angioma and pyogenic granuloma.

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  • (PMID = 20022827.001).
  • [ISSN] 1695-9531
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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4. Guillou L: [Mesenchymal tumors of the skin. Tufted angioma (angioblastoma)]. Ann Pathol; 2009 Oct;29(5):416-9
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  • [Title] [Mesenchymal tumors of the skin. Tufted angioma (angioblastoma)].
  • [Transliterated title] Tumeurs conjonctives de la peau. Cas n(o) 8. Hémangiome acquis en touffes (angioblastome de Nakagawa).
  • [MeSH-major] Hemangioma / pathology. Skin Neoplasms / pathology

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  • (PMID = 20004845.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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5. Ammerman J, Lonser RR, Oldfield EH: 844 Long-term Natural History of Hemangioblastomas in von Hippel-Lindau Disease: Implications for Treatment. Neurosurgery; 2005 Aug 01;57(2):410

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] 844 Long-term Natural History of Hemangioblastomas in von Hippel-Lindau Disease: Implications for Treatment.

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  • (PMID = 28184796.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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6. Bing F, Kremer S, Lamalle L, Chabardes S, Ashraf A, Pasquier B, Le Bas JF, Krainik A, Grand S: [Value of perfusion MRI in the study of pilocytic astrocytoma and hemangioblastoma: preliminary findings]. J Neuroradiol; 2009 May;36(2):82-7
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  • [Title] [Value of perfusion MRI in the study of pilocytic astrocytoma and hemangioblastoma: preliminary findings].
  • PURPOSE: Pilocytic astrocytomas (PA) and hemangioblastomas (HB) can present the same morphological characteristics on conventional MRI sequences, most usually in the form of a cerebellar cystic mass with a mural nodule that strongly enhances on post-contrast T1 images.
  • [MeSH-major] Astrocytoma / diagnosis. Blood Volume. Brain / blood supply. Brain Neoplasms / diagnosis. Cerebrovascular Circulation. Hemangioblastoma / diagnosis. Magnetic Resonance Imaging / methods


7. Samaila MO: Malignant tumours of childhood in Zaria. Afr J Paediatr Surg; 2009 Jan-Jun;6(1):19-23
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  • Germ cell tumours affected the ovary predominantly and two of the endodermal sinus tumour cases were seen in the testis of an eighteen month child and sacrococcygeum of a 5 year old girl, respectively.
  • The vascular tumours included epithelioid haemangioendothelioma, haemangioblastoma and Dabska tumour and they accounted for (5.8%) of all tumours seen.

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  • (PMID = 19661660.001).
  • [ISSN] 0974-5998
  • [Journal-full-title] African journal of paediatric surgery : AJPS
  • [ISO-abbreviation] Afr J Paediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Nigeria
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8. Lehman NL: The ubiquitin proteasome system in neuropathology. Acta Neuropathol; 2009 Sep;118(3):329-47
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  • In neuropathology, alteration of the UPS, or mutations in UPS target proteins may result in signaling abnormalities leading to the initiation or progression of tumors such as astrocytomas, hemangioblastomas, craniopharyngiomas, pituitary adenomas, and medulloblastomas.
  • In neurodegenerative diseases caused by the expression of mutant proteins, the cellular accumulation of these proteins may overload the UPS, indirectly contributing to the disease process, e.g., sporadic Parkinsonism and prion diseases.

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  • (PMID = 19597829.001).
  • [ISSN] 1432-0533
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / K08 NS045077
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Ubiquitin; EC 3.4.25.1 / Proteasome Endopeptidase Complex
  • [Number-of-references] 149
  • [Other-IDs] NLM/ PMC2716447
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9. Chen Y, Tachibana O, Hasegawa M, Xu R, Hamada JI, Yamashita J, Hashimoto N, Takahashi JA: Absence of Tight Junctions between Microvascular Endothelial Cells in Human Cerebellar Hemangioblastomas. Neurosurgery; 2006 Sep 01;59(3):660-670

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Absence of Tight Junctions between Microvascular Endothelial Cells in Human Cerebellar Hemangioblastomas.

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  • (PMID = 28180753.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Mao RJ, Li QM, Guo YM, Li WQ, Fan CS, Zhu XZ: [Clinicopathologic study of giant cell angioblastoma]. Zhonghua Bing Li Xue Za Zhi; 2010 Nov;39(11):752-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Clinicopathologic study of giant cell angioblastoma].
  • OBJECTIVE: to study the clinicopathological features, imaging characteristics, immunophenotypes and differential diagnosis of giant cell angioblastoma (GCAB).
  • A concentric arrangement of oval-to-spindle Cells around small-caliber vascular structures together with collagen fiber contributed to a so-called 'onion-skin' arrangement.
  • CONCLUSION: GCAB is a rare, locally infiltrative but slow growing neoplastic angiogenesis with unique morphological characteristics during infancy, which may occur not only in the skin, mucosa, subcutis and deep soft tissue but also in the bone.
  • [MeSH-major] Bone Neoplasms / pathology. Giant Cell Tumor of Bone / pathology. Hemangioblastoma / pathology. Tibia
  • [MeSH-minor] Actins / metabolism. Antigens, CD / metabolism. Antigens, CD31 / metabolism. Antigens, CD34 / metabolism. Antigens, Differentiation, Myelomonocytic / metabolism. Dermatofibrosarcoma / metabolism. Dermatofibrosarcoma / pathology. Diagnosis, Differential. Fibula. Hemangioendothelioma / metabolism. Hemangioendothelioma / pathology. Hemangioendothelioma, Epithelioid / metabolism. Hemangioendothelioma, Epithelioid / pathology. Hemangioma, Cavernous / metabolism. Hemangioma, Cavernous / pathology. Humans. Infant. Kasabach-Merritt Syndrome. Male. Sarcoma, Kaposi / metabolism. Sarcoma, Kaposi / pathology. Skin Neoplasms / metabolism. Skin Neoplasms / pathology. Thrombocytopenia / metabolism. Thrombocytopenia / pathology. Tomography, X-Ray Computed. Vascular Neoplasms / metabolism. Vascular Neoplasms / pathology. Vimentin / metabolism

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  • (PMID = 21215166.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / ACTA2 protein, human; 0 / Actins; 0 / Antigens, CD; 0 / Antigens, CD31; 0 / Antigens, CD34; 0 / Antigens, Differentiation, Myelomonocytic; 0 / CD68 antigen, human; 0 / Vimentin; Kaposiform Hemangioendothelioma
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11. Wang EM, Pan L, Wang BJ, Zhang N, Zhou LF, Dong YF, Dai JZ, Cai PW, Chen H: The long-term results of gamma knife radiosurgery for hemangioblastomas of the brain. J Neurosurg; 2005 Jan;102(s_supplement):225-229

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The long-term results of gamma knife radiosurgery for hemangioblastomas of the brain.
  • OBJECT: The authors assessed the long-term result of gamma knife surgery (GKS) for hemangioblastomas of the brain (HABs) and show histopathological findings after GKS.
  • Twenty-one patients had von Hippel-Lindau (VHL) disease.
  • Although GKS can treat multiple tumors in a single session, for HABs associated with VHL disease, GKS faces the dual problems of tumor recurrence or development of a new tumor.

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  • (PMID = 28306468.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; gamma knife surgery / hemangioblastoma / von Hippel—Lindau disease
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12. Tago M, Terahara A, Shin M, Maruyama K, Kurita H, Nakagawa K, Ohtomo K: Gamma knife surgery for hemangioblastomas. J Neurosurg; 2005 Jan;102(s_supplement):171-174

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gamma knife surgery for hemangioblastomas.
  • OBJECT: The authors reviewed their 14-year experience using stereotactic radiosurgery for the treatment of hemangioblastomas and define the role and the proper strategy for radiosurgery of this condition.
  • METHODS: This is a retrospective study of 38 hemangioblastomas in 13 patients.
  • Seven patients had von Hippel-Lindau disease.
  • CONCLUSIONS: Gamma knife surgery is a safe and effective method to control hemangioblastomas for as many as 10 years.

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  • (PMID = 28306464.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; gamma knife surgery / hemangioblastoma / stereotactic radiosurgery / von Hippel-Lindau disease
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13. Park YS, Chang JH, Chang JW, Chung SS, Park YG: Gamma knife surgery for multiple hemangioblastomas. J Neurosurg; 2005 Jan;102(s_supplement):97-101

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gamma knife surgery for multiple hemangioblastomas.
  • OBJECT: The authors describe their experience in treating patients with hemangioblastoma, especially multiple lesions, with gamma knife surgery (GKS).
  • METHODS: Nine patients with 84 hemangioblastomas underwent GKS between July 1992 and May 2003.
  • Of the six patients with multifocal tumors, a diagnosis of von Hippel-Lindau disease had been established in five.

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  • (PMID = 28306460.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; gamma knife surgery / hemangioblastoma / von Hippel—Lindau disease
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14. Na JI, Cho KH, Kim YG, Park KC: Angioblastoma showing aggravation after treatment with long-pulsed Nd:YAG laser (1064 nm). Pediatr Dermatol; 2007 Jul-Aug;24(4):397-400
MedlinePlus Health Information. consumer health - Skin Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Angioblastoma showing aggravation after treatment with long-pulsed Nd:YAG laser (1064 nm).
  • Angioblastoma usually develops in infancy or early childhood on the neck or upper trunk.
  • It is known to be slowly progressive and benign in nature, but treatment guidelines have not yet been established.
  • We report this occurrence to increase awareness of trauma-induced aggravation phenomena in angioblastoma.
  • [MeSH-major] Hemangioblastoma / pathology. Hemangioblastoma / radiotherapy. Lasers / adverse effects. Skin Neoplasms / pathology. Skin Neoplasms / radiotherapy

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  • (PMID = 17845165.001).
  • [ISSN] 1525-1470
  • [Journal-full-title] Pediatric dermatology
  • [ISO-abbreviation] Pediatr Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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15. Goh SG, Calonje E: Cutaneous vascular tumours: an update. Histopathology; 2008 May;52(6):661-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • This review evaluates changes in vascular nomenclature particularly in the category of vascular tumours of intermediate malignancy that includes the various haemangioendotheliomas, Kaposi's sarcoma and giant cell angioblastoma.
  • [MeSH-major] Neoplasms, Vascular Tissue / classification. Neoplasms, Vascular Tissue / pathology. Skin Neoplasms / classification. Skin Neoplasms / pathology
  • [MeSH-minor] Diagnosis, Differential. Hemangioendothelioma / diagnosis. Hemangioendothelioma / pathology. Hemangioma / diagnosis. Hemangioma / pathology. Humans. Lymphangioma / diagnosis. Lymphangioma / pathology. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / pathology

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  • (PMID = 18266723.001).
  • [ISSN] 1365-2559
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 69
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16. Browning J, Frieden I, Baselga E, Wagner A, Metry D: Congenital, self-regressing tufted angioma. Arch Dermatol; 2006 Jun;142(6):749-51
MedlinePlus Health Information. consumer health - Skin Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Congenital, self-regressing tufted angioma.
  • BACKGROUND: Tufted angioma (known in Japanese literature as angioblastoma of Nakagawa) is an uncommon, histologically benign, vascular tumor.
  • OBSERVATIONS: We present a series of 5 histopathologically confirmed cases of congenital tufted angioma that spontaneously regressed during infancy or early childhood.
  • CONCLUSION: We recommend that observation for potential regression be considered for otherwise uncomplicated congenital or early infantile cases of tufted angioma.
  • [MeSH-major] Hemangioma, Capillary / diagnosis. Neoplasm Regression, Spontaneous / pathology. Skin Neoplasms / diagnosis

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  • (PMID = 16785378.001).
  • [ISSN] 0003-987X
  • [Journal-full-title] Archives of dermatology
  • [ISO-abbreviation] Arch Dermatol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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17. Matin SF, McCutcheon IE, Gombos DS, Waguespack SG, Wen S, Smith LA, Zhang Y, Davis DW, Fuller G, Jonasch E: Treatment of VHL patients with sunitinib: Clinical observations and translational studies. J Clin Oncol; 2009 May 20;27(15_suppl):e22047

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • : e22047 Background: Von Hippel Lindau (VHL) disease induces vascular lesions in multiple organs.
  • Eligibility criteria included retinal angiomas, hemangioblastomas (HBs) measuring at least 5mm, renal cell carcinoma (RCC) 1 to 3 cm and pancreatic neuroendocrine tumors (NETs) 1 to 3 cm.

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  • (PMID = 27963229.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Barrey C, Kalamarides M, Polivka M, George B: Cervical Dumbbell Intra-extradural Hemangioblastoma: Total Removal through the Lateral Approach: Technical Case Report. Neurosurgery; 2005 Mar 01;56(3):E625

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cervical Dumbbell Intra-extradural Hemangioblastoma: Total Removal through the Lateral Approach: Technical Case Report.

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  • (PMID = 28184666.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. da Silva EB Jr, Leal AG, Milano JB, da Silva LF Jr, Clemente RS, Ramina R: Image-guided surgical planning using anatomical landmarks in the retrosigmoid approach. Acta Neurochir (Wien); 2010 May;152(5):905-10

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • RESULTS: There were 19 cases of vestibular schwannomas, 5 petroclival meningiomas, 3 trigeminal neuralgias, 1 angioblastoma, 1 epidermoid cyst and 1 hemifacial spasm.

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  • (PMID = 19902141.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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20. Drabko K, Choma M, Zaucha-Prazmo A, Wójcik B, Gorczyńska E, Kałwak K, Turkiewicz D, Słociak M, Ussowicz M, Dyla A, Chybicka A, Styczyński J, Debski R, Wysocki M, Goździk J, Ratajczak M, Kowalczyk JR: [Megachemotherapy and autologous hematopoietic stem cell transplantation in children with solid tumours excluding neuroblastoma--experience of Polish paediatric centres]. Med Wieku Rozwoj; 2006 Jul-Sep;10(3 Pt 1):785-92
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 25 children were treated for Ewing Sarcoma, 13 for rhabdomyosarcoma embryonale (RMS), 7 for germinal tumours, 6 for medulloblastoma, 4 for PNET, 4 for Wilm's tumours, 2 for glioblastoma and single patients with mesenchymoma, astrocytoma, ependymoma, angioblastoma, carcinoma ovarian and carcinoma embryonale glutei.
  • RESULTS: 30 children are alive (44%), 28 of them in complete remission of disease.
  • 39 patients relapsed at a median time 11 months after MCT and 37 of them subsequently died of disease at a median time of 16 months.
  • One toxic death was noted--it was a boy, transplanted with progressive disease.
  • [MeSH-minor] Academic Medical Centers. Adolescent. Antineoplastic Agents, Alkylating / administration & dosage. Bone Neoplasms / drug therapy. Bone Neoplasms / surgery. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Dose-Response Relationship, Drug. Ependymoma / drug therapy. Ependymoma / surgery. Female. Glioblastoma / drug therapy. Glioblastoma / surgery. Glioma / drug therapy. Glioma / surgery. Humans. Infant. Male. Meningeal Neoplasms / drug therapy. Meningeal Neoplasms / surgery. Meningioma / drug therapy. Meningioma / surgery. Neoplasm Staging. Oncology Service, Hospital. Poland. Remission Induction. Retrospective Studies. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / surgery. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 17317909.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating
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21. Henriquez F, Gallego R, Oliva E, Silva D, Vega N: Conversion to rapamycin in a renal transplant patient with von Hippel-Lindau disease: encouraging results-a case report. Transplant Proc; 2008 Nov;40(9):3115-6
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Conversion to rapamycin in a renal transplant patient with von Hippel-Lindau disease: encouraging results-a case report.
  • von Hippel-Lindau (VHL) disease is a genetic syndrome based on an abnormality of the VHL gene located on the short arm of chromosome 3.
  • Herein we have presented a case in which conversion to sirolimus improved graft function and also caused regression of retinal angioblastomas.
  • [MeSH-major] Kidney Transplantation / immunology. Sirolimus / therapeutic use. von Hippel-Lindau Disease / surgery

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  • (PMID = 19010210.001).
  • [ISSN] 0041-1345
  • [Journal-full-title] Transplantation proceedings
  • [ISO-abbreviation] Transplant. Proc.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; W36ZG6FT64 / Sirolimus
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22. Fahrtash F, McCahon E, Arbuckle S: Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine. J Pediatr Hematol Oncol; 2010 Aug;32(6):506-10
Hazardous Substances Data Bank. VINCRISTINE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine.
  • BACKGROUND: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare, locally aggressive vascular tumors.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Hemangioendothelioma / drug therapy. Hemangioma / drug therapy. Vincristine / therapeutic use


23. Chiu CS, Yang LC, Hong HS, Kuan YZ: Treatment of a tufted angioma with intense pulsed light. J Dermatolog Treat; 2007;18(2):109-11
MedlinePlus Health Information. consumer health - Skin Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of a tufted angioma with intense pulsed light.
  • Tufted angioma is a rare cutaneous angiomatous proliferation named because of its characteristic histologic pattern of grouped dermal capillary tufts.
  • Although transformation to malignancy has not been described, tufted angiomas do not tend to regress.
  • We report an adult case of tufted angioma, with unusual presentation as annular plaques, which was alleviated after treatment with intense pulsed light in terms of both cosmetics and discomfort.
  • [MeSH-major] Hemangioma / diagnosis. Hemangioma / therapy. Skin Neoplasms / diagnosis. Skin Neoplasms / therapy

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  • (PMID = 17520468.001).
  • [ISSN] 0954-6634
  • [Journal-full-title] The Journal of dermatological treatment
  • [ISO-abbreviation] J Dermatolog Treat
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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24. Bienaimé A, Rojat-Habib MC, Hesse S, Pelissier JF, Bonerandi JJ: [Giant vascular tumour in an adult: tufted angioma or kaposiform hemangioendothelioma]. Ann Dermatol Venereol; 2006 Jun-Jul;133(6-7):553-6
MedlinePlus Health Information. consumer health - Skin Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Giant vascular tumour in an adult: tufted angioma or kaposiform hemangioendothelioma].
  • [Transliterated title] Tumeur vasculaire géante de l'adulte: angiome en touffe ou hémangioendothéliome kaposiforme.
  • INTRODUCTION: Tufted angioma and kaposiform hemangioendothelioma are two rare benign but aggressive vascular tumours that occur mainly in children.
  • On histological examination there were features of tufted angioma and kaposiform hemangioendothelioma.
  • The histological association of aspects which could correspond to tufted angioma and kaposiform hemangioendothelioma seems to confirm recent publications which support the hypothesis that these two tumours are two evolutive stages of one and only entity.
  • [MeSH-major] Hemangioendothelioma / pathology. Hemangioma / pathology. Skin Neoplasms / pathology


25. Ishikawa K, Hatano Y, Ichikawa H, Hashimoto H, Fujiwara S: The spontaneous regression of tufted angioma. A case of regression after two recurrences and a review of 27 cases reported in the literature. Dermatology; 2005;210(4):346-8
MedlinePlus Health Information. consumer health - Skin Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The spontaneous regression of tufted angioma. A case of regression after two recurrences and a review of 27 cases reported in the literature.
  • BACKGROUND: Tufted angioma, a peculiar angioma that is characterized by tufts of capillary-sized vessels scattered 'cannonball fashion' within the dermis, is known, on occasion, to regress spontaneously.
  • OBJECTIVE: To know the appropriate waiting period for spontaneous regression of tufted angioma.
  • METHODS: We report here a case of tufted angioma that regressed spontaneously after the lesions had recurred twice.
  • We also review previously reported cases of tufted angioma with spontaneous regression, including cases in the Japanese and non-Japanese literature.
  • [MeSH-major] Hemangioma, Capillary / pathology. Neoplasm Regression, Spontaneous / pathology. Skin Neoplasms / pathology

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  • [Copyright] 2005 S. Karger AG, Basel
  • (PMID = 15942226.001).
  • [ISSN] 1018-8665
  • [Journal-full-title] Dermatology (Basel, Switzerland)
  • [ISO-abbreviation] Dermatology (Basel)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 10
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26. Tuncer S, Araz B, Peksayar G, Buyukbabani N: Solitary lacrimal gland choristoma of the limbal conjunctiva. Ophthalmic Surg Lasers Imaging; 2010;41 Online:e1-2

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The authors report the clinical and histopathologic features of a conjunctival lacrimal gland choristoma.
  • Clinically, these tumors resemble conjunctival hemangioblastoma, lymphangioma, or a lymphoid infiltrate.

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  • [Copyright] Copyright 2010, SLACK Incorporated.
  • (PMID = 21053863.001).
  • [ISSN] 1938-2375
  • [Journal-full-title] Ophthalmic surgery, lasers & imaging : the official journal of the International Society for Imaging in the Eye
  • [ISO-abbreviation] Ophthalmic Surg Lasers Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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27. Peyre M, David P, Van Effenterre R, François P, Thys M, Emery E, Redondo A, Decq P, Aghakhani N, Parker F, Tadié M, Lacroix C, Bhangoo R, Giraud S, Richard S, French NCI Network VHL Disease and Inherited Predisposition to Kidney Cancer: Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease. Neurosurgery; 2010 Sep;67(3):577-87; discussion 587
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
  • BACKGROUND: Supratentorial hemangioblastomas are rare lesions, occurring either sporadically or in von Hippel-Lindau disease.
  • OBJECTIVE: Following recent advances in our understanding of the natural history of von Hippel-Lindau-associated cerebellar and spinal hemangioblastomas, we conducted a study of the natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
  • METHODS: We reviewed a series of 18 supratentorial hemangioblastomas in 13 patients with von Hippel-Lindau disease.
  • RESULTS: Hemangioblastomas were most commonly seen in the temporal lobe.
  • CONCLUSION: This series illustrates the wide variation in tumor locations, patterns of growth, and edema progression seen in supratentorial hemangioblastomas and adds to our knowledge of the natural history of hemangioblastomas.
  • [MeSH-major] Genetic Predisposition to Disease / genetics. Hemangioblastoma / genetics. Hemangioblastoma / pathology. von Hippel-Lindau Disease / genetics. von Hippel-Lindau Disease / pathology

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  • (PMID = 20647972.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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28. Rivera AL, Takei H, Zhai J, Shen SS, Ro JY, Powell SZ: Useful immunohistochemical markers in differentiating hemangioblastoma versus metastatic renal cell carcinoma. Neuropathology; 2010 Dec;30(6):580-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Useful immunohistochemical markers in differentiating hemangioblastoma versus metastatic renal cell carcinoma.
  • Hemangioblastomas (HBs) account for nearly a tenth of all posterior fossa neoplasms and can be the presenting finding in patients with von Hippel-Lindau (VHL) syndrome.
  • [MeSH-major] Biomarkers, Tumor / analysis. Carcinoma, Renal Cell / diagnosis. Cerebellar Neoplasms / diagnosis. Hemangioblastoma / diagnosis. Kidney Neoplasms / diagnosis

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  • [Copyright] © 2010 Japanese Society of Neuropathology.
  • (PMID = 20374497.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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29. Pavesi G, Berlucchi S, Munari M, Manara R, Scienza R, Opocher G: Clinical and surgical features of lower brain stem hemangioblastomas in von Hippel-Lindau disease. Acta Neurochir (Wien); 2010 Feb;152(2):287-92
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  • [Title] Clinical and surgical features of lower brain stem hemangioblastomas in von Hippel-Lindau disease.
  • BACKGROUND: In the context of von Hippel-Lindau disease (VHL), the medulla oblongata is a relatively frequent site of growth of hemangioblastomas, posing related clinical and surgical difficulties.
  • METHODS: Between 2001 and 2009, 14 patients (9 female and 5 male, mean age 34 years) underwent removal of 15 lower brain stem hemangioblastomas.
  • RESULTS: All the operated hemangioblastomas were located in the dorsal medulla oblongata, in the context of multiple lesions, cerebellar and/or spinal.
  • In ten patients hemangioblastomas were located in a median position at the obex area; in four cases a lateral location was observed.
  • CONCLUSIONS: Lower brain stem hemangioblastomas in the context of VHL show an often gradual onset of signs and symptoms except for patients who develop an obstructive hydrocephalus.
  • [MeSH-major] Brain Stem Neoplasms / pathology. Brain Stem Neoplasms / surgery. Hemangioblastoma / pathology. Hemangioblastoma / surgery. von Hippel-Lindau Disease / pathology. von Hippel-Lindau Disease / surgery

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  • (PMID = 19787293.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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30. Huang JS, Lin CM, Cheng YC, Hung KL, Chien CC, Chen SK, Chang CJ, Chen CW, Huang CJ: A vitronectin M381T polymorphism increases risk of hemangioblastoma in patients with VHL gene defect. J Mol Med (Berl); 2009 Jun;87(6):613-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A vitronectin M381T polymorphism increases risk of hemangioblastoma in patients with VHL gene defect.
  • Hemangioblastomas, highly vascular tumors, occur sporadically or associated with von Hippel-Lindau (VHL) disease.
  • Diverse mutations in the VHL gene inactivate the VHL protein and constitute the molecular etiology of the disease.
  • Adults simultaneously carrying a VHL defect and the T allele were 5.0-fold more likely to be affected by VHL disease than were methionine/methionine (M/M) homozygotes carrying a VHL defect.
  • Patients with sporadic hemangioblastoma, C-terminally truncated VHL protein or a large deletion in the VHL gene, and the T allele were 18.0-fold more likely to develop recurrent disease.
  • Taken together, individuals with mutated VHL are more likely to be affected by familial or recurrent sporadic hemangioblastoma when carrying the M/T or T/T genotype at codon 381 of vitronectin.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Hemangioblastoma / genetics. Polymorphism, Genetic. Vitronectin / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / complications. von Hippel-Lindau Disease / genetics

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  • (PMID = 19288063.001).
  • [ISSN] 1432-1440
  • [Journal-full-title] Journal of molecular medicine (Berlin, Germany)
  • [ISO-abbreviation] J. Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Vitronectin; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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31. Franke G, Bausch B, Hoffmann MM, Cybulla M, Wilhelm C, Kohlhase J, Scherer G, Neumann HP: Alu-Alu recombination underlies the vast majority of large VHL germline deletions: Molecular characterization and genotype-phenotype correlations in VHL patients. Hum Mutat; 2009 May;30(5):776-86
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Von Hippel-Lindau disease (VHL) is an autosomal dominant cancer syndrome.
  • In comparison to VHL patients in general, the 54 index cases and their affected relatives showed a higher occurrence of renal cell carcinomas (RCC) and of CNS hemangioblastomas.
  • We not only noted the association of RCC with retention of the HSPC300 gene, but also observed a significant correlation between retention of HSPC300 and the development of retinal angiomas (AR).
  • [MeSH-major] Alu Elements / genetics. Germ-Line Mutation / genetics. Recombination, Genetic / genetics. Sequence Deletion / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / genetics

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • [CommentIn] Hum Mutat. 2009 Sep;30(9):1365-6; author reply 1367 [19623534.001]
  • (PMID = 19280651.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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32. Kalof AN, Cooper K: D2-40 immunohistochemistry--so far! Adv Anat Pathol; 2009 Jan;16(1):62-4
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  • Since its introduction, D2-40 immunoexpression has been described in a variety of lymphovascular neoplasms including lymphangioma, Kaposi sarcoma, and hemangioendothelioma, as well as nonvascular neoplasms such as epithelioid mesothelioma, seminoma, and hemangioblastoma.
  • More recently, D2-40 immunoexpression has been reported in primary adrenal cortical tumors, schwannomas, and adnexal tumors of the skin.
  • [MeSH-minor] Adrenal Cortex Neoplasms / immunology. Adrenal Cortex Neoplasms / pathology. Antibodies, Monoclonal, Murine-Derived. Antigens, CD / analysis. Central Nervous System Neoplasms / immunology. Central Nervous System Neoplasms / pathology. Endothelium, Lymphatic / immunology. Endothelium, Lymphatic / pathology. Hemangioblastoma / immunology. Hemangioblastoma / pathology. Humans. Immunohistochemistry. Lymphangioma / immunology. Lymphangioma / pathology. Mesothelioma / immunology. Mesothelioma / pathology. Sarcoma, Kaposi / immunology. Sarcoma, Kaposi / pathology

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  • (PMID = 19098468.001).
  • [ISSN] 1533-4031
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / monoclonal antibody D2-40
  • [Number-of-references] 14
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33. Yang YM, Wang DM, Jiang HZ, Sha C, Yuan QG, Liu JC: [Treatment of spinal cord hemangioblastoma by microoperations combined with embolization]. Zhonghua Yi Xue Za Zhi; 2008 May 20;88(19):1309-12
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  • [Title] [Treatment of spinal cord hemangioblastoma by microoperations combined with embolization].
  • OBJECTIVE: To investigate the effects of microoperations combined with embolization in treatment of spinal cord hemangioblastoma.
  • METHODS: Thirty-six patients with spinal cord hemangioblastoma, 21 males and 15 females, aged 24.2 (12-48), underwent MRI and digital abstraction angiography.
  • CONCLUSION: Embolization lowers the blood supply of the spinal cord hemangioblastoma, elevates the cure rate, and enlarges the indication of spinal cord hemangioblastoma operation.
  • [MeSH-major] Embolization, Therapeutic / methods. Hemangioblastoma / therapy. Microsurgery / methods. Spinal Cord Neoplasms / therapy

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  • (PMID = 18956698.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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34. Moch H: [Von-Hippel-Lindau (VHL) protein function by initiation and progression of renal cancer]. Pathologe; 2008 Nov;29 Suppl 2:149-52
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Von-Hippel-Lindau (VHL) protein function by initiation and progression of renal cancer].
  • Germ line inactivation of the von-Hippel-Lindau (VHL) tumor suppressor gene causes von Hippel-Lindau hereditary cancer syndrome, and somatic mutations of this gene have been linked to the development of sporadic hemangioblastomas and clear cell renal carcinomas.
  • [MeSH-major] Alleles. Carcinoma, Renal Cell / genetics. Kidney Neoplasms / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / genetics

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  • (PMID = 18751708.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
  • [Number-of-references] 32
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35. Shin DA, Kim SH, Kim KN, Shin HC, Yoon DH: Surgical management of spinal cord haemangioblastoma. Acta Neurochir (Wien); 2008 Mar;150(3):215-20; discussion 220

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  • [Title] Surgical management of spinal cord haemangioblastoma.
  • BACKGROUND: The surgical management of spinal cord haemangioblastomas is distinct from that of other benign spinal cord tumours and optimal surgical strategy is still being determined because of the rarity of the condition.
  • PATIENTS AND METHODS: We retrospectively analysed 24 operations for symptomatic spinal cord haemangioblastomas in 20 patients.
  • CONCLUSION: The cystic component of spinal cord haemangioblastomas is responsible for symptom generation and is helpful for dissecting tumours.
  • [MeSH-major] Hemangioblastoma / surgery. Neurosurgical Procedures / methods. Spinal Cord / surgery. Spinal Cord Neoplasms / surgery

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  • (PMID = 18253694.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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36. Leung RS, Biswas SV, Duncan M, Rankin S: Imaging features of von Hippel-Lindau disease. Radiographics; 2008 Jan-Feb;28(1):65-79; quiz 323
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imaging features of von Hippel-Lindau disease.
  • von Hippel-Lindau (VHL) disease is a rare, autosomal dominantly inherited multisystem disorder characterized by development of a variety of benign and malignant tumors.
  • The spectrum of clinical manifestations of the disease is broad and includes retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renal cysts and tumors, pancreatic cysts and tumors, pheochromocytomas, and epididymal cystadenomas.
  • The most common causes of death in VHL disease patients are renal cell carcinoma and neurologic complications from cerebellar hemangioblastomas.
  • Screening is important because the lesions in VHL disease are treatable; thus, early detection allows use of more conservative therapy and may enhance the patient's length and quality of life.
  • A multidisciplinary team approach is important in screening for VHL disease.
  • [MeSH-major] Diagnostic Imaging / methods. Image Enhancement / methods. Neoplasms / diagnosis. von Hippel-Lindau Disease / diagnosis

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  • (PMID = 18203931.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 28
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37. Kaelin WG Jr: The von hippel-lindau tumor suppressor protein: an update. Methods Enzymol; 2007;435:371-83
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  • [Title] The von hippel-lindau tumor suppressor protein: an update.
  • Inactivation of the von Hippel-Lindau (VHL) tumor suppressor has been linked to a variety of tumors, including clear cell renal carcinoma, retinal and cerebellar hemangioblastoma, and pheochromocytoma.
  • [MeSH-major] Cell Transformation, Neoplastic / genetics. Von Hippel-Lindau Tumor Suppressor Protein / metabolism. von Hippel-Lindau Disease / genetics


38. Schlesinger T, Appukuttan B, Hwang T, Atchaneeyakasul LO, Chan CC, Zhuang Z, Stout JT, Wilson DJ: Internal en bloc resection and genetic analysis of retinal capillary hemangioblastoma. Arch Ophthalmol; 2007 Sep;125(9):1189-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Internal en bloc resection and genetic analysis of retinal capillary hemangioblastoma.
  • OBJECTIVE: To report the clinical outcomes and molecular genetics of nongermline retinal hemangioblastoma managed by surgical resection.
  • Tumors 7 to 9 mm in diameter were removed from 3 consecutive eyes (in 3 patients) via internal en bloc surgical resection using a bimanual technique.
  • Samples of DNA from 2 of 3 tumors were tested for von Hippel-Lindau gene (VHL) mutations as a clue to the molecular basis for spontaneously occurring hemangioblastoma.
  • Histopathological findings were typical of retinal hemangioblastoma in all cases.
  • CONCLUSIONS: Our patients' favorable outcomes suggest that surgical resection is an option for patients with large retinal hemangioblastomas.
  • In addition, ours may be the first report of retinal hemangioblastoma unassociated with a VHL mutation.
  • [MeSH-major] Hemangioblastoma / genetics. Hemangioblastoma / surgery. Loss of Heterozygosity. Ophthalmologic Surgical Procedures. Retinal Neoplasms / genetics. Retinal Neoplasms / surgery. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 17846357.001).
  • [ISSN] 0003-9950
  • [Journal-full-title] Archives of ophthalmology (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch. Ophthalmol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000222-22
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
  • [Other-IDs] NLM/ NIHMS31544; NLM/ PMC2140243
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39. Ciappetta P, Occhiogrosso G, Domenicucci M, D'Andrea G, Bastianello S, Frati A: Hemangioblastoma of the filum terminale. Case report and review of the literature. J Exp Clin Cancer Res; 2007 Jun;26(2):281-5
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  • [Title] Hemangioblastoma of the filum terminale. Case report and review of the literature.
  • Hemangioblastomas of the filum terminale are particularly rare tumors.
  • The authors present the case of a 62-year-old woman with a 6 month history of low-back-pain, who underwent surgery for the removal of an hemangioblastoma affecting the cauda at L2-L3 level.
  • Histologically it consisted in vascular structures interposed to a network of capillary-like vessels, surrounded by stromal cells.
  • [MeSH-major] Cauda Equina. Hemangioblastoma / diagnosis. Peripheral Nervous System Neoplasms / diagnosis

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  • (PMID = 17725110.001).
  • [ISSN] 0392-9078
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 26
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40. Stanojevic BR, Lohse P, Neskovic GG, Damjanovic SM, Novkovic TB, Jovanovic-Cupic SP, Dimitrijević BB: Germline VHL gene mutations in three Serbian families with von Hippel-Lindau disease. Neoplasma; 2007;54(5):402-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Germline VHL gene mutations in three Serbian families with von Hippel-Lindau disease.
  • Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited cancer predisposition syndrome due to germline mutations in the VHL tumor suppressor gene which is associated with virtually complete penetrance.
  • The VHL syndrome has a highly variable phenotypic expressivity including retinal and CNS haemangioblastomas, pheochromocytomas, renal clear cell carcinomas, and multifocal cysts.
  • In order to establish VHL gene testing, we analyzed three families affected by VHL disease, using SSCP mutation screening and DNA sequencing.
  • Affected members of the third family with a VHL type 1 disease carried a c.475T>C/p.Trp88Arg exchange.
  • We observed also intrafamiliar variation in time of onset and severity of the disease.
  • [MeSH-major] Mutation. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / genetics

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  • (PMID = 17688370.001).
  • [ISSN] 0028-2685
  • [Journal-full-title] Neoplasma
  • [ISO-abbreviation] Neoplasma
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Slovakia
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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41. Hes FJ, van der Luijt RB, Janssen AL, Zewald RA, de Jong GJ, Lenders JW, Links TP, Luyten GP, Sijmons RH, Eussen HJ, Halley DJ, Lips CJ, Pearson PL, van den Ouweland AM, Majoor-Krakauer DF: Frequency of Von Hippel-Lindau germline mutations in classic and non-classic Von Hippel-Lindau disease identified by DNA sequencing, Southern blot analysis and multiplex ligation-dependent probe amplification. Clin Genet; 2007 Aug;72(2):122-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Frequency of Von Hippel-Lindau germline mutations in classic and non-classic Von Hippel-Lindau disease identified by DNA sequencing, Southern blot analysis and multiplex ligation-dependent probe amplification.
  • The current clinical diagnosis of Von Hippel-Lindau (VHL) disease demands at least one specific [corrected] VHL manifestation in a patient with familial VHL disease, or, in a [corrected] sporadic patient, at least two or more hemangioblastomas or a single hemangioblastoma in combination with a typical visceral lesion.
  • [MeSH-major] Gene Frequency. Germ-Line Mutation. von Hippel-Lindau Disease / diagnosis. von Hippel-Lindau Disease / genetics

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  • [ErratumIn] Clin Genet. 2008 Apr;73(4):399
  • (PMID = 17661816.001).
  • [ISSN] 0009-9163
  • [Journal-full-title] Clinical genetics
  • [ISO-abbreviation] Clin. Genet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Denmark
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42. Pipp I, Wagner L, Rössler K, Budka H, Preusser M: Secretagogin expression in tumours of the human brain and its coverings. APMIS; 2007 Apr;115(4):319-26
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  • We found focal or widespread secretagogin expression in tumour cells in 1/18 oligoastrocytomas, 1/19 oligodendrogliomas, 2/20 anaplastic oligodendrogliomas, 2/9 ependymomas, 2/11 anaplastic ependymomas, 2/10 glioblastomas, 3/11 gangliogliomas and 1/2 anaplastic gangliogliomas, 10/10 central neurocytomas, 5/10 classic medulloblastomas, 4/5 desmoplastic medulloblastomas, 3/5 large cell/anaplastic medulloblastomas, 3/5 neuroblastomas, 3/10 meningiomas, 2/10 haemangioblastomas, and 13/19 pituitary adenomas.
  • Further, we observed secretagogin expression in endothelial cells in 5/10 meningiomas, 2/5 haemangiopericytomas, and 2/10 haemangioblastomas.

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  • (PMID = 17504298.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Calcium-Binding Proteins; 0 / SCGN protein, human; 0 / Secretagogins
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43. Junker B, Schmidt D, Agostini HT: [Retinal angiomatosis. Ocular manifestation of von Hippel-Lindau disease]. Ophthalmologe; 2007 Feb;104(2):107-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Retinal angiomatosis. Ocular manifestation of von Hippel-Lindau disease].
  • [Transliterated title] Angiomatosis retinae. Okuläre Manifestation des Von-Hippel-Lindau-Syndroms.
  • Von Hippel-Lindau disease (VHL disease) is a rare multisystem disorder of autosomal dominant inheritance with high penetrance.
  • Retinal capillary hemangioblastoma is one of the earliest manifestations of VHL disease.
  • It increases to 60% if there is already retinal capillary hemangioblastoma.
  • If VHL disease is suspected, a careful ophthalmological examination should be included in the clinical screening program.
  • Therapeutic options for small to medium sized peripheral tumors are laser or cryocoagulation; larger- hemangioblastomas can be treated by brachytherapy using ruthenium plaques, while asymptomatic juxtapapillary tumors can be observed at regular intervals.
  • [MeSH-major] Hemangioblastoma. Hemangioma, Capillary. Retinal Neoplasms. von Hippel-Lindau Disease

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  • (PMID = 17219178.001).
  • [ISSN] 0941-293X
  • [Journal-full-title] Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
  • [ISO-abbreviation] Ophthalmologe
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 7UI0TKC3U5 / Ruthenium
  • [Number-of-references] 46
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44. Hong SB, Furihata M, Baba M, Zbar B, Schmidt LS: Vascular defects and liver damage by the acute inactivation of the VHL gene during mouse embryogenesis. Lab Invest; 2006 Jul;86(7):664-75
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  • Inactivation of the von Hippel-Lindau (VHL) tumor suppressor gene leads to the development of central nervous system hemangioblastomas, pheochromocytomas and renal cell carcinomas.
  • [MeSH-major] Blood Vessels / abnormalities. Embryonic Development. Genes, Tumor Suppressor. Liver Diseases / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 16652107.001).
  • [ISSN] 0023-6837
  • [Journal-full-title] Laboratory investigation; a journal of technical methods and pathology
  • [ISO-abbreviation] Lab. Invest.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CO / N01-CO-12400; United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Culture Media; 0 / Viral Proteins; 33X04XA5AT / Lactic Acid; EC 2.7.7.- / Cre recombinase; EC 2.7.7.- / Integrases; EC 6.3.2.19 / Vhlh protein, mouse; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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45. Rasmussen A, Nava-Salazar S, Yescas P, Alonso E, Revuelta R, Ortiz I, Canizales-Quinteros S, Tusié-Luna MT, López-López M: Von Hippel-Lindau disease germline mutations in Mexican patients with cerebellar hemangioblastoma. J Neurosurg; 2006 Mar;104(3):389-94
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  • [Title] Von Hippel-Lindau disease germline mutations in Mexican patients with cerebellar hemangioblastoma.
  • OBJECT: Central nervous system (CNS) hemangioblastomas are benign vascular tumors arising either sporadically or as a manifestation of von Hippel-Lindau (VHL) disease, a hereditary cancer syndrome.
  • The authors studied a series of patients with CNS hemangioblastomas and their families to identify germline mutations in the VHL tumor suppressor gene and to establish a predictive testing and screening protocol.
  • METHODS: Patients admitted between 2002 and 2004 to the Instituto Nacional de Neurología y Neurocirugía for hemangioblastoma were prospectively enrolled, together with their at-risk family members.
  • Seven of the families had definite clinical criteria of VHL disease, five had sporadic hemangioblastoma, and four had CNS hemangioblastoma combined with minor visceral signs.
  • Molecular genetic testing identified five germline mutations in six of the definite VHL families (sensitivity 85%), but none in the possible VHL and sporadic hemangioblastoma cases; four of these mutations had been previously described and one is a novel mutation present in two unrelated families.
  • CONCLUSIONS: Genetic testing for mutations in the VHL gene is crucial in patients with CNS hemangioblastoma.
  • [MeSH-major] Germ-Line Mutation. Hemangioblastoma / etiology. Hemangioblastoma / genetics. von Hippel-Lindau Disease / complications. von Hippel-Lindau Disease / genetics

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  • (PMID = 16572651.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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46. Sharma MS, Jha AN: Ruptured intracranial aneurysm associated with von Hippel-Lindau syndrome: a molecular link? J Neurosurg; 2006 Feb;104(2 Suppl):90-3
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  • [Title] Ruptured intracranial aneurysm associated with von Hippel-Lindau syndrome: a molecular link?
  • The authors describe the case of aneurysmal rupture in a patient with von Hippel-Lindau (VHL) syndrome and explore a possible molecular link.
  • Six years later, she developed spinocerebellar hemangioblastomas.
  • [MeSH-major] Aneurysm, Ruptured / etiology. Aneurysm, Ruptured / genetics. Chromosomes, Human, Pair 3. Intracranial Aneurysm / etiology. Intracranial Aneurysm / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / complications. von Hippel-Lindau Disease / genetics

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  • (PMID = 16506495.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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47. Toledo M, Pujadas F, Purroy F, Alvarez-Sabin J: [Polycythaemia as a ready factor of transitory global amnesia]. Neurologia; 2005 Jul-Aug;20(6):317-20

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  • On the other hand polycythaemia is classified like a prothrombotic disorder that can produce manifold manifestations in the central nervous system, from strokes to psychiatric disturbances of anxious type, and all related to the secondary cerebral hipoperfusión due to hyperviscosity.
  • Two patients, who had a previous diagnostic of polycythaemia suffered an episode of TGA, in addition in one of the two cases a cerebellar hemangioblastoma was found.

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  • (PMID = 16007516.001).
  • [ISSN] 0213-4853
  • [Journal-full-title] Neurología (Barcelona, Spain)
  • [ISO-abbreviation] Neurologia
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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48. Le Huu AR, Jokinen CH, Rubin BP, Mihm MC, Weiss SW, North PE, Dadras SS: Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. Am J Surg Pathol; 2010 Nov;34(11):1563-73
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  • [Title] Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma.
  • Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare tumors mainly occurring in early childhood.
  • For this purpose, we examined 75 vascular lesions: KHE (n=18), TA (n=13), infantile hemangioma (n=13), pyogenic granuloma (n=18), and granulation tissue (n=13).
  • Overall, our results show, for the first time, that Prox1 is an immunohistochemical biomarker helpful in confirming the diagnosis of KHE/TA and in distinguishing it from infantile hemangioma and pyogenic granuloma.
  • [MeSH-major] Biomarkers, Tumor / analysis. Hemangioendothelioma / chemistry. Hemangioma / chemistry. Homeodomain Proteins / analysis. Skin Neoplasms / chemistry. Soft Tissue Neoplasms / chemistry. Tumor Suppressor Proteins / analysis

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  • [ErratumIn] Am J Surg Pathol. 2011 Feb;35(2):314. Ruben, Brian P [corrected to Rubin, Brian P]
  • (PMID = 20975337.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Antigens, CD34; 0 / Biomarkers, Tumor; 0 / Homeodomain Proteins; 0 / LYVE1 protein, human; 0 / Membrane Glycoproteins; 0 / PDPN protein, human; 0 / Tumor Suppressor Proteins; 0 / Vesicular Transport Proteins; 0 / prospero-related homeobox 1 protein
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49. Rodriguez V, Lee A, Witman PM, Anderson PA: Kasabach-merritt phenomenon: case series and retrospective review of the mayo clinic experience. J Pediatr Hematol Oncol; 2009 Jul;31(7):522-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Kasabach-Merritt phenomenon (KMP) is a rare thrombocytopenic consumption coagulopathy associated with an enlarging tufted angioma or kaposiform hemangioendothelioma.
  • [MeSH-major] Disseminated Intravascular Coagulation / complications. Disseminated Intravascular Coagulation / physiopathology. Hemangioendothelioma / complications. Hemangioma / complications. Skin Neoplasms / complications

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  • (PMID = 19564750.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Inflammatory Agents; 0 / Antineoplastic Agents
  • [Number-of-references] 10
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50. Lee B, Chiu M, Soriano T, Craft N: Adult-onset tufted angioma: a case report and review of the literature. Cutis; 2006 Nov;78(5):341-5
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  • [Title] Adult-onset tufted angioma: a case report and review of the literature.
  • Tufted angiomas (TAs) are benign vascular tumors, primarily occurring on the trunk and extremities of children younger than 5 years.
  • [MeSH-major] Hemangioma / pathology. Lip Neoplasms / pathology. Skin Neoplasms / pathology


51. Schaffer JV, Fangman W, Bossenbroek NM, Meehan SA, Kamino H: Tufted angioma. Dermatol Online J; 2008;14(10):20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tufted angioma.
  • Histopathologic evaluation showed multiple, discrete lobules of tightly packed capillaries in a 'cannonball' pattern within the dermis, which confirmed the diagnosis of tufted angioma.
  • The clinical and histopathologic features, natural history, and treatment options for tufted angiomas are reviewed; their relationship to kaposiform hemangioendotheliomas is discussed.
  • [MeSH-major] Hemangioma / diagnosis. Skin Neoplasms / diagnosis


52. Al Dhaybi R, Powell J, McCuaig C, Kokta V: Differentiation of vascular tumors from vascular malformations by expression of Wilms tumor 1 gene: evaluation of 126 cases. J Am Acad Dermatol; 2010 Dec;63(6):1052-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHODS: Based on the International Society for the Study of Vascular Anomalies classification of vascular anomalies, we studied the expression of WT1 in vascular tumors composed of infantile hemangioma, congenital hemangiomas (non-involuting, rapidly involuting, and not otherwise specified), pyogenic granuloma, tufted angioma, cherry angioma, Kaposi sarcoma, and angiosarcoma.
  • We also studied WT1 expression in vascular malformations composed of angiokeratoma/verrucous hemangioma, combined vascular malformations, venous malformations, glomuvenous malformations, lymphatic malformations/lymphangioma, telangiectasia, and targetoid hemosiderotic hemangioma.
  • [MeSH-major] Arteriovenous Malformations / pathology. Biomarkers, Tumor / metabolism. Hemangioma / pathology. Nuclear Proteins / metabolism. Vascular Neoplasms / pathology

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  • [Copyright] Copyright © 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.
  • (PMID = 21093662.001).
  • [ISSN] 1097-6787
  • [Journal-full-title] Journal of the American Academy of Dermatology
  • [ISO-abbreviation] J. Am. Acad. Dermatol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Nuclear Proteins; 0 / WTAP protein, human
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53. Sid-Ahmed S, Seizeur R, Forlodou P, Dam-Hieu P, Quintin-Roue I, Person H, Besson G: [Dumbell hemangioblastoma of the eighth cervical nerve root]. Neurochirurgie; 2006 Dec;52(6):533-6
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  • [Title] [Dumbell hemangioblastoma of the eighth cervical nerve root].
  • [Transliterated title] Hémangioblastome "en sablier" de la huitième racine cervicale.
  • Hemangioblastomas involving cervical nerve roots are extremely rare.
  • We report the case of a 33-year-old man presenting with a 6-month history of upper limb pain.
  • MRI and cervical angiography demonstrated the presence of a dumbell (intra and extradural) and highly vascularized tumor of the right C7-T1 foramina.
  • Histological examination eventually confirmed the diagnosis of hemangioblastoma.
  • [MeSH-major] Cranial Nerve Neoplasms / surgery. Hemangioblastoma / surgery. Vestibulocochlear Nerve / pathology

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  • (PMID = 17203902.001).
  • [ISSN] 0028-3770
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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54. Crisi G, Giombelli E, Ventura E: Non-von Hippel-Lindau Hemangioblastoma in the Hippocampus: Characterization with Time-Resolved MRA Using TRICKS Sequence at 3T. A Case Report. Neuroradiol J; 2010 Sep;23(4):416-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Non-von Hippel-Lindau Hemangioblastoma in the Hippocampus: Characterization with Time-Resolved MRA Using TRICKS Sequence at 3T. A Case Report.
  • Supratentorial hemangioblastomas (HBs) are exceedingly rare tumors accounting for 5%-10% of all HBs and usually present in the setting of von Hippel-Lindau (VHL) disease.
  • Isolated HBs of the central nervous system also occur as a result of spontaneous mutation in the absence of any clinical manifestations of VHL disease.
  • We describe the TRICKS findings in a case of isolated hemangioblastoma in the hippocampus without VHL disease.
  • To our knowledge, this is the second case report of a hippocampal non-VHL hemangioblastoma.

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  • (PMID = 24148630.001).
  • [ISSN] 1971-4009
  • [Journal-full-title] The neuroradiology journal
  • [ISO-abbreviation] Neuroradiol J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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55. Agrawal A, Kakani A, Vagh SJ, Hiwale KM, Kolte G: Cystic hemangioblastoma of the brainstem. J Neurosci Rural Pract; 2010 Jan;1(1):20-2

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  • [Title] Cystic hemangioblastoma of the brainstem.
  • Hemangioblastomas are very highly vascular neoplasm with benign characteristics and; in comparison to cerebellar hemangioblastoma; cases of cystic hemangioblastoma of the brain stem are rare with only a few case reports available in the literature.
  • We report the case of a 43-year-old-female with cystic hemagioblastoma of the brainstem managed successfully and review the relevant literature.

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  • [ISSN] 0976-3155
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  • [Keywords] NOTNLM ; Cystic hemangioblastoma / fourth ventricle / hemangioblastoma / medulla oblongata
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56. Do-Dai DD, Brooks MK, Goldkamp A, Erbay S, Bhadelia RA: Magnetic resonance imaging of intramedullary spinal cord lesions: a pictorial review. Curr Probl Diagn Radiol; 2010 Jul-Aug;39(4):160-85
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  • Rare intramedullary neoplasms including dermoid tumor, astrocytoma, ependymoma, hemangioblastoma, lymphoma, ganglioneuroblastoma, and metastases are also illustrated.
  • The potential pitfalls in the differentiation of tumors from nonneoplastic disease of the spinal cord are also elucidated.

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  • (PMID = 20510754.001).
  • [ISSN] 1535-6302
  • [Journal-full-title] Current problems in diagnostic radiology
  • [ISO-abbreviation] Curr Probl Diagn Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 48
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57. Arkadopoulos N, Karapanos K, Stafyla V, Yiallourou A, Koureas A, Kondi-Pafiti A, Smyrniotis V: Combination of right nephrectomy and total pancreaticoduodenectomy for Von Hippel-Lindau disease. JOP; 2010;11(3):270-2
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  • [Title] Combination of right nephrectomy and total pancreaticoduodenectomy for Von Hippel-Lindau disease.
  • CONTEXT: Von Hippel-Lindau disease is an inherited syndrome of multiorgan neoplasia caused by a germline mutation in the von Hippel-Lindau gene and can include central nervous system tumors, renal cell carcinomas and benign pancreatic cystic tumors.
  • CASE REPORT: We report the case of a 56-year-old patient who had a past history of cerebellar hemangioblastoma and presented with abdominal pain.
  • CONCLUSION: In past cases of von Hippel-Lindau disease, pancreatic adenomas with malignant transformation have not been reported.
  • [MeSH-major] Carcinoma, Renal Cell / surgery. Neoplasms, Multiple Primary / surgery. Nephrectomy / methods. Pancreatic Neoplasms / surgery. Pancreaticoduodenectomy / methods. von Hippel-Lindau Disease / surgery

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  • (PMID = 20442526.001).
  • [ISSN] 1590-8577
  • [Journal-full-title] JOP : Journal of the pancreas
  • [ISO-abbreviation] JOP
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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58. Hes FJ, Höppener JW, Luijt RB, Lips CJ: Von hippel-lindau disease. Hered Cancer Clin Pract; 2005;3(4):171-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Von hippel-lindau disease.
  • A germline mutation in the Von-Hippel Lindau (VHL) gene predisposes carriers to development of abundantly vascularised tumours in the retina, cerebellum, spine, kidney, adrenal gland and pancreas.
  • Most VHL patients die from the consequences of cerebellar haemangioblastoma or renal cell carcinoma.

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  • (PMID = 20223044.001).
  • [ISSN] 1897-4287
  • [Journal-full-title] Hereditary cancer in clinical practice
  • [ISO-abbreviation] Hered Cancer Clin Pract
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Poland
  • [Other-IDs] NLM/ PMC2837060
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59. Sacko O, Bouillot-Eimer S, Sesay M, Uro-Coste E, Roux FE, Loiseau H: Hemangioblastoma of the corpus callosum: A case report and review of the literature on its origin. Neurochirurgie; 2010 Oct;56(5):382-5
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  • [Title] Hemangioblastoma of the corpus callosum: A case report and review of the literature on its origin.
  • A third case of corpus callosum hemangioblastoma (HB) is presented.
  • [MeSH-major] Brain Neoplasms. Corpus Callosum. Hemangioblastoma

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  • [Copyright] Copyright © 2009 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20045160.001).
  • [ISSN] 1773-0619
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] France
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60. Mandigo CE, Ogden AT, Angevine PD, McCormick PC: Operative management of spinal hemangioblastoma. Neurosurgery; 2009 Dec;65(6):1166-77
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  • [Title] Operative management of spinal hemangioblastoma.
  • Hemangioblastomas occur in 2% to 15% of reported series of intramedullary spinal cord tumors.
  • We describe our experience with 16 intramedullary and 2 lumbosacral nerve root hemangioblastomas and review the relevant published literature.
  • [MeSH-major] Hemangioblastoma / surgery. Microsurgery / methods. Neurosurgical Procedures / methods. Spinal Neoplasms / surgery

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  • (PMID = 19934977.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 43
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61. McNeill A, Rattenberry E, Barber R, Killick P, MacDonald F, Maher ER: Genotype-phenotype correlations in VHL exon deletions. Am J Med Genet A; 2009 Oct;149A(10):2147-51
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  • Von Hippel-Lindau (VHL) syndrome is a dominantly inherited familial cancer syndrome caused by mutations in the VHL gene.
  • Type 2 subtypes of VHL syndrome are characterized by the presence of pheochromocytoma and the three Type 2 subtypes are associated with differing risks of hemangioblastoma and renal cell carcinoma (RCC).
  • Type 1 VHL syndrome is most commonly caused by germline exon deletions and truncating mutations and is characterized by susceptibility to hemangioblastomas and RCC but not pheochromocytoma.
  • The risks of hemangioblastomas were similar in both groups.
  • These results add to the growing body of evidence suggesting that patients with VHL syndrome caused by large VHL deletions that include C3orf10 may be designated as having a specific subtype (Type 1B) of the disorder.
  • [MeSH-major] Gene Deletion. Phenotype. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / classification
  • [MeSH-minor] Adrenal Gland Neoplasms / complications. Adrenal Gland Neoplasms / genetics. Carcinoma, Renal Cell / complications. Carcinoma, Renal Cell / genetics. Cerebellar Neoplasms / complications. Cerebellar Neoplasms / genetics. Cytoskeletal Proteins / genetics. DNA Mutational Analysis. Exons / genetics. Fanconi Anemia Complementation Group D2 Protein / genetics. Genotype. Germ-Line Mutation. Hemangioblastoma / complications. Hemangioblastoma / genetics. Humans. Kidney Neoplasms / complications. Kidney Neoplasms / genetics. Pheochromocytoma / complications. Pheochromocytoma / genetics. Retinal Neoplasms / complications. Retinal Neoplasms / genetics

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  • (PMID = 19764026.001).
  • [ISSN] 1552-4833
  • [Journal-full-title] American journal of medical genetics. Part A
  • [ISO-abbreviation] Am. J. Med. Genet. A
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / BRK1 protein, human; 0 / Cytoskeletal Proteins; 0 / FANCD2 protein, human; 0 / Fanconi Anemia Complementation Group D2 Protein; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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62. Sharma GK, Kucia EJ, Spetzler RF: Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report. Neurosurgery; 2009 Sep;65(3):E627-8; discussion E628
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  • [Title] Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report.
  • OBJECTIVE: Spontaneous hemorrhage of a spinal hemangioblastoma is a rare event.
  • METHODS: A 39-year-old man presented with paraplegia and bilateral upper-extremity weakness related to an acute intramedullary hemorrhage from a thoracic spinal hemangioblastoma.
  • Pathological analysis of the tumor biopsy confirmed the diagnosis of hemangioblastoma.
  • CONCLUSION: The risk of spontaneous hemorrhage from a spinal hemangioblastoma is low.
  • Spinal hemangioblastomas presenting with intramedullary hemorrhage tend to cause severe neurological deficits and have a poorer long-term prognosis compared with subarachnoid hemorrhage and nonhemorrhagic lesions.
  • [MeSH-major] Hemangioblastoma / complications. Hemorrhage / etiology. Spinal Neoplasms / complications

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  • (PMID = 19687673.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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63. de San Pedro JR, Rodríguez FA, Níguez BF, Sánchez JF, López-Guerrero AL, Murcia MF, Vilar AM: Massive hemorrhage in hemangioblastomas Literature review. Neurosurg Rev; 2010 Jan;33(1):11-26

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  • [Title] Massive hemorrhage in hemangioblastomas Literature review.
  • Massive hemorrhage is a very uncommon event among hemangioblastomas.
  • Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances.
  • Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment.
  • To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma.
  • When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor.
  • Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.
  • [MeSH-major] Cerebellar Neoplasms / complications. Cerebral Hemorrhage / etiology. Hemangioblastoma / complications
  • [MeSH-minor] Adult. Age Factors. Age of Onset. Aged. Cerebral Angiography. Cerebral Ventricles / pathology. Disease Progression. Embolization, Therapeutic / adverse effects. Female. Humans. Intracranial Aneurysm / complications. Intracranial Aneurysm / pathology. Magnetic Resonance Imaging. Male. Middle Aged. Sex Factors. Spinal Cord Neoplasms / complications. Spinal Cord Neoplasms / epidemiology. Subarachnoid Hemorrhage / epidemiology. Subarachnoid Hemorrhage / etiology. Tomography, X-Ray Computed

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  • (PMID = 19672640.001).
  • [ISSN] 1437-2320
  • [Journal-full-title] Neurosurgical review
  • [ISO-abbreviation] Neurosurg Rev
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 78
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64. Bishop FS, Liu JK, Chin SS, Fults DW: Recurrent cerebellar hemangioblastoma with enhancing tumor in the cyst wall: case report. Neurosurgery; 2008 Jun;62(6):E1378-9; discussion E1379
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  • [Title] Recurrent cerebellar hemangioblastoma with enhancing tumor in the cyst wall: case report.
  • OBJECTIVE: Hemangioblastomas are the most common primary intra-axial tumors of the adult posterior fossa and the tumors most often associated with von Hippel-Lindau disease.
  • Resection of cerebellar hemangioblastomas involves tumor excision and drainage of associated cysts.
  • We present an unusual variant of a hemangioblastoma that showed pathological evidence of a tumor within the cyst wall that correlated with radiographic cyst wall enhancement in a patient with a recurrent hemangioblastoma.
  • CLINICAL PRESENTATION: 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.
  • Histopathological examination showed a capillary hemangioblastoma with tumor tissue inside the cyst wall.
  • CONCLUSION: This unusual case of a hemangioblastoma with cyst 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 of recurrence.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Hemangioblastoma / pathology. Neoplasm Recurrence, Local / pathology. von Hippel-Lindau Disease / pathology

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  • (PMID = 18824960.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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65. Baggenstos M, Chew E, Butman JA, Oldfield EH, Lonser RR: Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss. J Neurosurg; 2008 Aug;109(2):313-7
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  • [Title] Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss.
  • Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultrafiltrate through permeable neoplastic vessels.
  • The authors report the clinical and imaging findings in a 62-year-old man with von Hippel-Lindau disease who presented with rapid (within 24 hours) loss of color vision and nearcomplete loss of left eye vision (acuity too poor to test).
  • Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations.
  • Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma.
  • Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss.
  • [MeSH-major] Brain Edema / pathology. Hemangioblastoma / pathology. Optic Nerve / pathology. Vision, Low / pathology. von Hippel-Lindau Disease / pathology

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  • (PMID = 18671645.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 NS003053-01
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS152869; NLM/ PMC2770701
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66. De Salles AA, Gorgulho AA, Selch M, De Marco J, Agazaryan N: Radiosurgery from the brain to the spine: 20 years experience. Acta Neurochir Suppl; 2008;101:163-8
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  • RESULTS: Over 4,000 patients with trigeminal neuralgia/intractable pain, arteriovenous malformations/angiomas, metastases, ependymomas, gliomas, meningiomas hemangiopericytomas, schwannomas, adenomas, hemangioblastomas, and chordoma were treated.

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  • (PMID = 18642653.001).
  • [ISSN] 0065-1419
  • [Journal-full-title] Acta neurochirurgica. Supplement
  • [ISO-abbreviation] Acta Neurochir. Suppl.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Austria
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67. Dwarakanath S, Sharma BS, Mahapatra AK: Intraspinal hemangioblastoma: analysis of 22 cases. J Clin Neurosci; 2008 Dec;15(12):1366-9
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  • [Title] Intraspinal hemangioblastoma: analysis of 22 cases.
  • Hemangioblastomas of the spinal cord are relatively uncommon benign tumors.
  • The purpose of this study was to evaluate the clinical features, management strategies and outcomes in patients with spinal hemangioblastomas.
  • We retrospectively analyzed 22 patients who were operated on for spinal hemangioblastoma at the All India Institute of Medical Sciences, New Delhi, India.
  • In our center, 113 patients with hemangioblastomas had been managed over a 14-year period (1992-2006).
  • Ninety-one of these hemangioblastomas were intracranial and 22 were intraspinal.
  • The most common presenting feature was progressive compressive myelopathy (22 patients).
  • This is one of the larger series in the literature on spinal hemangioblastoma.
  • [MeSH-major] Hemangioblastoma. Spinal Cord Neoplasms

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  • (PMID = 18501608.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] Scotland
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68. Michels S, Messmer E, Sutter F, Kurz-Levin MM: [Intravitreal anti-VEGF therapy for capillary hemangioblastomas in von Hippel-Lindau disease]. Klin Monbl Augenheilkd; 2008 Apr;225(4):292-4
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  • [Title] [Intravitreal anti-VEGF therapy for capillary hemangioblastomas in von Hippel-Lindau disease].
  • [Transliterated title] Intravitreale anti-VEGF-Therapie von retinalen kapillären Hämangioblastomen bei von-Hippel-Lindau-Erkrankung.
  • BACKGROUND: Up-regulated expression of the vascular endothelial growth factor (VEGF) in von Hippel-Lindau (VHL) disease has been postulated to induce retinal hemangioblastoma.
  • METHODS: In a single case decision a patient with active retinal hemangioblastomas due to VHL disease received repeated intravitreal injections of 0.5 mg ranibizumab.
  • RESULTS: Subsequent to intravitreal anti-VEGF therapy, the signs of activity of the retinal hemangioblastomas slowly regressed.
  • CONCLUSIONS: Intravitreal anti-VEGF therapy might, as monotherapy or as combination therapy, offer a new treatment option for retinal hemangioblastoma.
  • [MeSH-major] Antibodies, Monoclonal / administration & dosage. Hemangioblastoma / drug therapy. Retinal Neoplasms / drug therapy. Vascular Endothelial Growth Factor A / antagonists & inhibitors. von Hippel-Lindau Disease / drug therapy

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  • (PMID = 18401796.001).
  • [ISSN] 0023-2165
  • [Journal-full-title] Klinische Monatsblätter für Augenheilkunde
  • [ISO-abbreviation] Klin Monbl Augenheilkd
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Vascular Endothelial Growth Factor A; ZL1R02VT79 / Ranibizumab
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69. Park SH, Min HS, Kim B, Myung J, Paek SH: Galectin-3: a useful biomarker for differential diagnosis of brain tumors. Neuropathology; 2008 Oct;28(5):497-506
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  • It showed consistent and diffuse positivity in 100% of the pilocytic astrocytomas, pleomorphic xanthoastrocytomas (PXA), Schwannomas, meningiomas, capillary hemangioblastomas, as well as in ependymomas, but it was completely negative in the diffuse astrocytomas, anaplastic astrocytomas, both low- and high-grades of the oligodendrogliomas, central neurocytomas, and medulloblastomas.

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  • (PMID = 18384511.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Galectin 3
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70. Karabagli H, Karabagli P, Alpman A, Durmaz B: Congenital supratentorial cystic hemangioblastoma. Case report and review of the literature. J Neurosurg; 2007 Dec;107(6 Suppl):515-8
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  • [Title] Congenital supratentorial cystic hemangioblastoma. Case report and review of the literature.
  • Supratentorial hemangioblastomas are rarely encountered tumors even in the pediatric population; an extensive review of the literature has revealed approximately 118 cases.
  • The histopathological diagnosis was a reticular variant of hemangioblastoma.
  • Given that von Hippel-Lindau (VHL) gene mutations may be associated with hemangioblastomas, sequencing analysis of the VHL gene was performed; sequencing of the three exons of the VHL gene showed no exonic mutations.
  • The authors reviewed the literature concerning congenital supratentorial hemangioblastomas, and they discuss the clinical and histopathological characteristics and differential diagnosis associated with such lesions.
  • [MeSH-major] Hemangioblastoma / congenital. Supratentorial Neoplasms / congenital
  • [MeSH-minor] DNA / genetics. Humans. Infant. Magnetic Resonance Imaging. Male. Neurosurgical Procedures. von Hippel-Lindau Disease / genetics

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  • (PMID = 18154025.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 9007-49-2 / DNA
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71. Schreinemakers JM, Zonnenberg BA, Höppener JW, Hes FJ, Rinkes IH, Lips CJ: A patient with bilateral pheochromocytoma as part of a Von Hippel-Lindau (VHL) syndrome type 2C. World J Surg Oncol; 2007;5:112
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A patient with bilateral pheochromocytoma as part of a Von Hippel-Lindau (VHL) syndrome type 2C.
  • BACKGROUND: Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited disease.
  • As an illustration, an interesting case is presented of a pregnant woman with refractory hypertension.
  • The family history may indicate the diagnosis, but only identification of a germ line mutation in the DNA of a patient will confirm carriership.
  • VHL disease can be classified into several subtypes.
  • VHL type 2C patients present with pheochromocytomas without evidence of haemangioblastomas in the central nervous system and/or retina and a low risk of renal cell carcinoma.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / genetics. Pheochromocytoma / diagnosis. Pheochromocytoma / genetics. Pregnancy Complications, Neoplastic / diagnosis. Pregnancy Outcome. von Hippel-Lindau Disease / genetics


72. Bonneville F, Savatovsky J, Chiras J: Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions. Eur Radiol; 2007 Nov;17(11):2908-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Finally, brain stem or ventricular tumours can present with a significant exophytic component in the CPA that may be difficult to differentiate from an extra-axial lesion (lymphoma, hemangioblastoma, choroid plexus papilloma, ependymoma, glioma, medulloblastoma, dysembryoplastic neuroepithelial tumour).

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  • (PMID = 17569053.001).
  • [ISSN] 0938-7994
  • [Journal-full-title] European radiology
  • [ISO-abbreviation] Eur Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] AU0V1LM3JT / Gadolinium
  • [Number-of-references] 75
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73. Bisceglia M, Spagnolo D, Galliani C, Fisher C, Suster S, Kazakov DV, Cooper K, Michal M: Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix. Pathologica; 2006 Aug;98(4):239-98

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  • In an eleven part series published in Pathologica, we have presented various tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue (ST), which emerged as new entities or as variants of established entities during the last quarter of a century.
  • Detailed clinicomorphological and differential diagnostic features of approximately sixty entities were chosen on the basis of their clinical significance and morphologic distinctiveness.
  • The series included fibrous and myofibroblastic tumors (e.g. solitary fibrous tumor, high grade classic and pigmented dermatofibrosarcoma protuberans, inflammatory myofibroblastic tumor and myofibrosarcomas), fibromyxoid and fibrohistiocytic neoplasms (e.g., Evans' tumor, phosphaturic mesenchymal tumor, inflammatory myxohyaline tumor), special adipocytic/vascular/and smooth muscle lesions (e.g., chondroid lipoma, Dabska's tumor, ST hemangioblastoma, lipoleiomyosarcoma), epithelioid mesenchymal malignancies of diverse lineages (e.g., epithelioid liposarcoma, proximal-type epithelioid sarcoma, neuroendocrine extraskeletal chondromyxoid sarcoma), ST Ewing's tumor and peripheral nerve sheath tumors (perineuriomas and pigmented and rosetting tumors of the schwannoma/neurofibroma group), extranodal dendritic or histiocytic proliferative processes (follicular dendritic cell sarcoma, Rosai-Dorfman disease, Castleman's disease, and plexiform xanthomatous tumor), and tumors with myoepithelial differentiation.

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  • (PMID = 17175794.001).
  • [ISSN] 0031-2983
  • [Journal-full-title] Pathologica
  • [ISO-abbreviation] Pathologica
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 272
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74. Vortmeyer AO, Tran MG, Zeng W, Gläsker S, Riley C, Tsokos M, Ikejiri B, Merrill MJ, Raffeld M, Zhuang Z, Lonser RR, Maxwell PH, Oldfield EH: Evolution of VHL tumourigenesis in nerve root tissue. J Pathol; 2006 Nov;210(3):374-82
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  • Haemangioblastomas are the key central nervous system manifestation of von Hippel-Lindau (VHL) disease, which is caused by germline mutation of the VHL gene.
  • We have recently shown that 'tumour-free' spinal cord from patients with VHL disease contains microscopic, poorly differentiated cellular aggregates in nerve root tissue, which we descriptively designated 'mesenchymal tumourlets'.
  • We show that a small subset of mesenchymal tumourlets extends beyond the nerve root to form proliferative VHL-deficient mesenchyme and frank haemangioblastoma.
  • We thus demonstrate that tumourlets present potential, but true precursor material for haemangioblastoma.
  • The evolution of frank haemangioblastoma seems to involve multiple steps from a large pool of precursor lesions.
  • [MeSH-major] Spinal Cord Neoplasms / pathology. Spinal Nerve Roots / pathology. von Hippel-Lindau Disease / pathology
  • [MeSH-minor] Antigens, CD31 / immunology. Basic Helix-Loop-Helix Transcription Factors / metabolism. Cerebellar Neoplasms / metabolism. Cerebellar Neoplasms / pathology. Hemangioblastoma / metabolism. Hemangioblastoma / pathology. Humans. Hypoxia-Inducible Factor 1, alpha Subunit / metabolism. Immunohistochemistry / methods. In Situ Hybridization / methods. Microscopy, Electron / methods. Neoplasm Proteins / metabolism. Neovascularization, Pathologic / pathology. Stem Cells / pathology. Up-Regulation / physiology. Vascular Endothelial Growth Factor A / metabolism. Von Hippel-Lindau Tumor Suppressor Protein / metabolism

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  • [Copyright] Copyright 2006 Pathological Society of Great Britain and Ireland.
  • (PMID = 16981244.001).
  • [ISSN] 0022-3417
  • [Journal-full-title] The Journal of pathology
  • [ISO-abbreviation] J. Pathol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / Hypoxia-Inducible Factor 1, alpha Subunit; 0 / Neoplasm Proteins; 0 / Vascular Endothelial Growth Factor A; 0 / endothelial PAS domain-containing protein 1; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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75. Rickert CH, Hasselblatt M, Jeibmann A, Paulus W: Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles. Hum Pathol; 2006 Nov;37(11):1452-7
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  • [Title] Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles.
  • Capillary hemangioblastomas of the central nervous system are benign tumors and occur either sporadically or as a manifestation of von Hippel-Lindau disease.
  • To investigate whether these subtypes differ in their cytogenetic profile, a comparative genomic hybridization analysis of 10 cellular and 10 reticular hemangioblastomas was undertaken.
  • Comparative genomic hybridization revealed DNA copy number changes in 14 of 20 cases (8 of 10 cellular and 6 of 10 reticular hemangioblastomas).
  • In conclusion, our data may point toward different genetic pathways in the pathogenesis of the 2 histologic subtypes of capillary hemangioblastoma.
  • [MeSH-major] Hemangioblastoma / chemistry. Hemangioblastoma / pathology

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  • (PMID = 16949923.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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76. Kaelin WG: The von Hippel-Lindau tumor suppressor protein: roles in cancer and oxygen sensing. Cold Spring Harb Symp Quant Biol; 2005;70:159-66
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  • [Title] The von Hippel-Lindau tumor suppressor protein: roles in cancer and oxygen sensing.
  • Biallelic inactivation of the von Hippel-Lindau (VHL) tumor suppressor gene is a common event in hereditary (von Hippel- Lindau disease) and sporadic hemangioblastomas and clear-cell renal carcinomas.
  • Down-regulation of HIF appears to be both necessary and sufficient for renal tumor suppression by pVHL, and HIF is strongly suspected of contributing to hemangioblastoma development as well.
  • [MeSH-major] Neoplasms / etiology. Oxygen / metabolism. Von Hippel-Lindau Tumor Suppressor Protein / genetics. Von Hippel-Lindau Tumor Suppressor Protein / physiology
  • [MeSH-minor] Adrenal Gland Neoplasms / etiology. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / physiopathology. Animals. Disease Models, Animal. Genes, Tumor Suppressor. Hemangioblastoma / etiology. Hemangioblastoma / genetics. Hemangioblastoma / physiopathology. Humans. Kidney Neoplasms / etiology. Kidney Neoplasms / genetics. Kidney Neoplasms / physiopathology. Models, Biological. Mutation. Pheochromocytoma / etiology. Pheochromocytoma / genetics. Pheochromocytoma / physiopathology. von Hippel-Lindau Disease / etiology. von Hippel-Lindau Disease / genetics. von Hippel-Lindau Disease / physiopathology

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  • (PMID = 16869749.001).
  • [ISSN] 0091-7451
  • [Journal-full-title] Cold Spring Harbor symposia on quantitative biology
  • [ISO-abbreviation] Cold Spring Harb. Symp. Quant. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein; S88TT14065 / Oxygen
  • [Number-of-references] 116
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77. Kanno H, Ozawa Y, Sakata K, Sato H, Tanabe Y, Shimizu N, Yamamoto I: Intraoperative power Doppler ultrasonography with a contrast-enhancing agent for intracranial tumors. J Neurosurg; 2005 Feb;102(2):295-301
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  • The tumors included 37 intracranial neoplasms (14 gliomas, six meningiomas, three hemangioblastomas, two malignant lymphomas, three other primary neoplasms, nine metastatic tumors, and three nonneoplastic lesions).
  • Among the tumors, hemangioblastomas displayed particularly strong contrast enhancement.
  • CONCLUSIONS: Intraoperative power Doppler ultrasonography performed using a contrast-enhancing agent can facilitate intraoperative real-time navigation and assessment of the intratumoral vasculature and peritumoral vessels, particularly for tumors having abundant vessels such as hemangioblastomas.

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  • (PMID = 15739558.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media; 0 / Polysaccharides; 127279-08-7 / SHU 508
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78. Shimada S, Ishizawa K, Hirose T: Expression of E-cadherin and catenins in meningioma: ubiquitous expression and its irrelevance to malignancy. Pathol Int; 2005 Jan;55(1):1-7
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  • In addition, hemangioblastoma (15 cases) and hemangiopericytoma (four cases) were also evaluated.
  • Hemangiopericytoma was positive for alpha- and beta-catenins, and hemangioblastomas were positive for beta-catenin alone, which was distinct from the expression pattern in meningiomas.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Diagnosis, Differential. Female. Hemangioblastoma / metabolism. Hemangioblastoma / pathology. Hemangiopericytoma / metabolism. Hemangiopericytoma / pathology. Humans. Immunohistochemistry. Male. Middle Aged

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  • (PMID = 15660696.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cadherins; 0 / Cytoskeletal Proteins
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79. Matsunaga S, Shuto T, Inomori S, Fujino H, Yamamoto I: Gamma knife radiosurgery for intracranial haemangioblastomas. Acta Neurochir (Wien); 2007 Oct;149(10):1007-13; discussion 1013
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  • [Title] Gamma knife radiosurgery for intracranial haemangioblastomas.
  • BACKGROUND: The results of gamma knife radiosurgery for haemangioblastomas were retrospectively studied to assess the efficacy for tumour growth control and clarify the clinical indications for gamma knife radiosurgery in these tumours.
  • METHODS: The medical records of 22 patients with 67 tumours, 12 men and 10 women aged 20-73 years (mean 51.9 years), who underwent gamma knife radiosurgery for haemangioblastomas between January 1993 and January 2006, were retrospectively reviewed.
  • Ten patients with 54 lesions had von Hippel-Lindau disease.
  • The only factor affecting tumour growth control was the presence of a cystic component at the time of gamma knife radiosurgery in both univariate and multivariate analysis.
  • CONCLUSION: The presence of cystic components at the time of gamma knife radiosurgery was the only factor significantly correlated with unfavourable tumour growth control by gamma knife radiosurgery for haemangioblastomas.
  • [MeSH-major] Brain Neoplasms / surgery. Hemangioblastoma / surgery. Radiosurgery. von Hippel-Lindau Disease / surgery

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  • (PMID = 17712513.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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80. Wu YM, Wong HF, Ng SH: Direct Intratumoral Pre-surgical Embolization of Orbital Hemangioblastoma. A Case Report. Neuroradiol J; 2008 Dec 17;21(5):693-7

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  • [Title] Direct Intratumoral Pre-surgical Embolization of Orbital Hemangioblastoma. A Case Report.
  • Orbital hemangioblastomas of extraretinal origin are extremely rare and few cases have been reported.
  • We describe a 48-year-old woman with progressive right visual loss and proptosis.
  • The histopathologic characteristics of this tumor were identical to those seen in central nervous system hemangioblastomas.

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  • (PMID = 24257013.001).
  • [ISSN] 1971-4009
  • [Journal-full-title] The neuroradiology journal
  • [ISO-abbreviation] Neuroradiol J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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81. Deb P, Sahni H, Bhatoe HS: Cystic angiomatous meningioma in the cerebellopontine angle mimicking hemangioblastoma. J Cancer Res Ther; 2010 Oct-Dec;6(4):560-3
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  • [Title] Cystic angiomatous meningioma in the cerebellopontine angle mimicking hemangioblastoma.
  • Immunopositivity for epithelial membrane antigen (EMA), vimentin and S-100 protein proved vital in excluding a hemangioblastoma.
  • [MeSH-major] Cerebellopontine Angle / pathology. Hemangioblastoma / pathology. Meningioma / pathology

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  • (PMID = 21358102.001).
  • [ISSN] 1998-4138
  • [Journal-full-title] Journal of cancer research and therapeutics
  • [ISO-abbreviation] J Cancer Res Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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82. Kong HS, Lee S, Beebe K, Scroggins B, Gupta G, Lee MJ, Jung YJ, Trepel J, Neckers L: Emetine promotes von Hippel-Lindau-independent degradation of hypoxia-inducible factor-2α in clear cell renal carcinoma. Mol Pharmacol; 2010 Dec;78(6):1072-8
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  • [Title] Emetine promotes von Hippel-Lindau-independent degradation of hypoxia-inducible factor-2α in clear cell renal carcinoma.
  • Inactivating mutations of the von Hippel-Lindau (VHL) tumor suppressor gene are associated with inherited VHL syndrome, which is characterized by susceptibility to a variety of neoplasms, including central nervous system hemangioblastoma and clear cell renal cell carcinoma (CCRCC).
  • [MeSH-major] Basic Helix-Loop-Helix Transcription Factors / metabolism. Carcinoma, Renal Cell / metabolism. Emetine / pharmacology. Kidney Neoplasms / metabolism. Von Hippel-Lindau Tumor Suppressor Protein / physiology

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  • (PMID = 20813864.001).
  • [ISSN] 1521-0111
  • [Journal-full-title] Molecular pharmacology
  • [ISO-abbreviation] Mol. Pharmacol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / endothelial PAS domain-containing protein 1; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein; X8D5EPO80M / Emetine
  • [Other-IDs] NLM/ PMC3202510
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83. Hwang SW, Malek AM, Schapiro R, Wu JK: Intraoperative use of indocyanine green fluorescence videography for resection of a spinal cord hemangioblastoma. Neurosurgery; 2010 Sep;67(3 Suppl Operative):ons300-3; discussion ons303
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  • [Title] Intraoperative use of indocyanine green fluorescence videography for resection of a spinal cord hemangioblastoma.
  • We describe a novel application of this previously defined surgical tool to assist in the resection of a residual spinal cord hemangioblastoma.
  • CLINICAL PRESENTATION: Our patient is a 49-year-old woman with a residual symptomatic cervical hemangioblastoma that was previously embolized and resected at another institution.
  • After initial symptomatic improvement, she returned with progressive symptoms, increasing radiographic spinal cord edema, and a residual lesion at the level of C1.
  • [MeSH-major] Hemangioblastoma / surgery. Indocyanine Green. Monitoring, Intraoperative / methods. Neurosurgical Procedures / methods. Spinal Cord Neoplasms / surgery. Video Recording / methods

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  • (PMID = 20679920.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] IX6J1063HV / Indocyanine Green
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84. Karabagli H, Genc A, Karabagli P, Abacioglu U, Seker A, Kilic T: Outcomes of gamma knife treatment for solid intracranial hemangioblastomas. J Clin Neurosci; 2010 Jun;17(6):706-10
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  • [Title] Outcomes of gamma knife treatment for solid intracranial hemangioblastomas.
  • The aim of this study was to examine the results of gamma knife radiosurgery for 13 patients with residual/recurrent or newly diagnosed solid hemangioblastomas.
  • The 13 patients had 34 solid hemangioblastomas, and all patients underwent gamma knife radiosurgery.
  • Seven patients had von Hippel-Lindau disease and six had sporadic disease.
  • Our findings reinforce the view that gamma knife radiosurgery is effective and safe for the management of solid hemangioblastomas with a diameter less than 3 cm, whether they are sporadic or associated with von Hippel-Lindau disease.
  • [MeSH-major] Brain Neoplasms / surgery. Hemangioblastoma / surgery. Radiosurgery / methods

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  • [Copyright] Copyright 2009 Elsevier Ltd. All rights reserved.
  • (PMID = 20303274.001).
  • [ISSN] 1532-2653
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Scotland
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85. Adam Y, Benezech J, Blanquet A, Fuentes JM, Bousigue JY, Debono B, Duplessis E, Espagno C, Plas JY, Lescure JP, Destandau J, Hladky JP, Grunewald P, Mahla K, Remond J, Louis E: [Intramedullary tumors. Results of a national investigation in private neurosurgery]. Neurochirurgie; 2010 Aug;56(4):344-9

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  • [Title] [Intramedullary tumors. Results of a national investigation in private neurosurgery].
  • [Transliterated title] Les tumeurs intramédullaires. Résultat d'une enquête nationale en neurochirurgie libérale.
  • MATERIAL: Seventy-nine cases were distributed in the following manner: ependymomas, 38; astrocytomas, 22; oligodendrogliomas, four; gangliogliomas, two; hemangioblastomas, 10 (nine sporadic cases and one case of Von Hippel-Lindau disease); primitive melanoma, one; and intramedullary neurinomas, two.
  • Tumor removal was complete in the cases of ependymoma and hemangioblastoma and subtotal in the cases of astrocytoma.
  • Hemangioblastoma: 10 cases, nine sporadic and one case of Von Hippel-Lindau disease.

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  • [Copyright] Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20097390.001).
  • [ISSN] 1773-0619
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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86. Till V, Koprivsek K, Stojanovic S, Avramov P, Vulekovic P: Transient cortical blindness following vertebral angiography in a young adult with cerebellar haemangioblastoma. Pediatr Radiol; 2009 Nov;39(11):1223-6
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  • [Title] Transient cortical blindness following vertebral angiography in a young adult with cerebellar haemangioblastoma.
  • We report a long episode of transient cortical blindness in a 17-year-old boy with cerebellar haemangioblastoma, which started during the preoperative vertebral angiography and lasted for 5 days.
  • [MeSH-major] Blindness, Cortical / diagnosis. Blindness, Cortical / etiology. Brain Neoplasms / diagnosis. Cerebral Angiography / adverse effects. Hemangioblastoma / diagnosis. Magnetic Resonance Imaging

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  • (PMID = 19779710.001).
  • [ISSN] 1432-1998
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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87. Prabhu K, Daniel RT, Chacko G, Chacko AG: Optic nerve haemangioblastoma mimicking a planum sphenoidale meningioma. Br J Neurosurg; 2009;23(5):561-3
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  • [Title] Optic nerve haemangioblastoma mimicking a planum sphenoidale meningioma.
  • Haemangioblastomas are rarely seen in the suprasellar region, arising from the optic apparatus or pituitary stalk, mimicking meningiomas on the preoperative MRI scan.
  • They may be suspected in the presence of large flow voids and the absence of a dural tail.
  • [MeSH-major] Hemangioblastoma / diagnosis. Meningioma / diagnosis. Optic Nerve Neoplasms / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. von Hippel-Lindau Disease / diagnosis

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  • (PMID = 19718547.001).
  • [ISSN] 1360-046X
  • [Journal-full-title] British journal of neurosurgery
  • [ISO-abbreviation] Br J Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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88. Parker F, Aghakhani N, Ducati LG, Yacubian-Fernandes A, Silva MV, David P, Richard S, Tadie M: Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups. J Neurooncol; 2009 May;93(1):133-7
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  • [Title] Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups.
  • PURPOSE: To analyze the surgical outcome of a consecutive and single center series of medulla oblongata (MO) and spinal cord hemangioblastomas (HB).
  • Twenty-five (73%) patients had Von Hippel Lindau (VHL) disease confirmed by genetic screening, and nine patients had sporadic disease.
  • CONCLUSION: Our results confirmed that surgery remains a safe and effective treatment for medulla oblongata and spinal hemangioblastoma.
  • [MeSH-major] Brain Neoplasms / surgery. Hemangioblastoma / surgery. Medulla Oblongata / surgery. Neurosurgical Procedures. Spinal Cord Neoplasms / surgery
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Angiography. Magnetic Resonance Imaging. Male. Microsurgery. Recovery of Function. Retrospective Studies. Treatment Outcome. von Hippel-Lindau Disease / complications. von Hippel-Lindau Disease / pathology. von Hippel-Lindau Disease / surgery

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  • (PMID = 19430890.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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89. Jost G, Zimmerer S, Frank S, Cordier D, Merlo A: Intradural spinal metastasis of renal cell cancer. Report of a case and review of 26 published cases. Acta Neurochir (Wien); 2009 Jul;151(7):815-21; discussion 821
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intradural spinal metastasis of renal cell cancer. Report of a case and review of 26 published cases.
  • Metastatic disease in the intradural compartment of the spine is a rare manifestation of cancer.
  • 18 patients had sporadic renal cell carcinoma, and 9 patients had von Hippel-Lindau disease (VHL) in which the metastases of the renal cell carcinoma were embedded within spinal haemangioblastomas.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Back Pain / etiology. Female. Hemangioblastoma / secondary. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Neurosurgical Procedures / methods. Paraparesis / etiology. Secondary Prevention. Subdural Space / pathology. Subdural Space / physiopathology. Subdural Space / surgery. Survival Rate. Urinary Bladder, Neurogenic / etiology. von Hippel-Lindau Disease / complications

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  • (PMID = 19415167.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Austria
  • [Number-of-references] 46
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90. Hue YH, Chun HJ, Yi HJ, Oh SH, Oh SJ, Ko Y: Unilateral posterior atlantoaxial transarticular screw fixation in patients with atlantoaxial instability : comparison with bilateral method. J Korean Neurosurg Soc; 2009 Mar;45(3):164-8

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  • The causes of unilateral TAF were anomalous course of vertebral artery in 20 patients (74%), severe degenerative arthritis in 3 (11%), fracture of C1 in 2, hemangioblastoma in one, and screw malposition in one.

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  • (PMID = 19352478.001).
  • [ISSN] 2005-3711
  • [Journal-full-title] Journal of Korean Neurosurgical Society
  • [ISO-abbreviation] J Korean Neurosurg Soc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2666118
  • [Keywords] NOTNLM ; Atlantodental interval (ADI) / Atlatoaxial instability (AAI) / Transarticular screw fixation (TAF) / Unilateral / Vertebral artery
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91. Thirabanjasak D, Basturk O, Altinel D, Cheng JD, Adsay NV: Is serous cystadenoma of the pancreas a model of clear-cell-associated angiogenesis and tumorigenesis? Pancreatology; 2009;9(1-2):182-8
MedlinePlus Health Information. consumer health - Pancreatic Cancer.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Similar to the other von Hippel-Lindau (VHL)-related tumors such as renal cell carcinomas and capillary hemangioblastomas, serous cystadenomas (SCAs) of the pancreas are also characterized by clear cells.
  • Over the years, we have also noticed that the tumor epithelium shows a prominent capillary network.
  • RESULTS: There was an extensively rich capillary network that appears almost intraepithelially in all cases of SCA, which was confirmed by CD31 stain that showed, on average, 26 capillaries per every 100 epithelial cells.
  • CONCLUSION: As in other VHL-related clear-cell tumors, there is a prominent capillary network immediately adjacent to the epithelium of SCA, confirming that the clear-cell- angiogenesis association is also valid for this tumor type.

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  • [Copyright] Copyright 2008 S. Karger AG, Basel and IAP.
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  • (PMID = 19077470.001).
  • [ISSN] 1424-3911
  • [Journal-full-title] Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
  • [ISO-abbreviation] Pancreatology
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P20 CA101936; United States / NCI NIH HHS / CA / CA101936
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Glucose Transporter Type 1; 0 / Hypoxia-Inducible Factor 1, alpha Subunit; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A
  • [Other-IDs] NLM/ PMC2835376
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92. Alexiou GA, Tsiouris S, Voulgaris S, Doukas M, Goussia A, Kyritsis AP, Polyzoidis KS, Fotopoulos AD: Brain scintigraphy with 99mTc-tetrofosmin for the differential diagnosis of a posterior fossa tumor. Hell J Nucl Med; 2008 May-Aug;11(2):114-7

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  • [Title] Brain scintigraphy with 99mTc-tetrofosmin for the differential diagnosis of a posterior fossa tumor.
  • The patient underwent surgery and haemangioblastoma was confirmed by histology.
  • [MeSH-major] Cerebellar Neoplasms / radionuclide imaging. Cerebellum / radionuclide imaging. Hemangioblastoma / radionuclide imaging. Organophosphorus Compounds. Organotechnetium Compounds

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  • (PMID = 18815667.001).
  • [ISSN] 1790-5427
  • [Journal-full-title] Hellenic journal of nuclear medicine
  • [ISO-abbreviation] Hell J Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Organophosphorus Compounds; 0 / Organotechnetium Compounds; 0 / Radiopharmaceuticals; 0 / technetium Tc 99m 1,2-bis(bis(2-ethoxyethyl)phosphino)ethane
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93. Wang C: Spinal hemangioblastoma: report on 68 cases. Neurol Res; 2008 Jul;30(6):603-9
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  • [Title] Spinal hemangioblastoma: report on 68 cases.
  • OBJECTIVE: Rare papers focusing on spinal hemangioblastoma, especially with large clinical series, had been published before.
  • In our hospital, there are 68 cases of spinal hemangioblastoma treated surgically.
  • METHODS: The clinical characteristics, the relationship between the tumor and spine, the anatomical segments, and surgical outcome of the 68 spinal hemangioblastoma patients were analysed.
  • CONCLUSION: For single spinal hemangioblastoma, the surgical outcome is quite satisfied.
  • [MeSH-major] Hemangioblastoma / surgery. Neurosurgery / methods. Spinal Neoplasms / surgery

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  • (PMID = 18647501.001).
  • [ISSN] 0161-6412
  • [Journal-full-title] Neurological research
  • [ISO-abbreviation] Neurol. Res.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] England
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94. Pavesi G, Feletti A, Berlucchi S, Opocher G, Martella M, Murgia A, Scienza R: Neurosurgical treatment of von Hippel-Lindau-associated hemangioblastomas: benefits, risks and outcome. J Neurosurg Sci; 2008 Jun;52(2):29-36
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  • [Title] Neurosurgical treatment of von Hippel-Lindau-associated hemangioblastomas: benefits, risks and outcome.
  • AIM: Von Hippel-Lindau (VHL) disease is a genetic syndrome predisposing to central nervous system (CNS) hemangioblastomas and several lesions in many organs.
  • METHODS: The authors evaluated 20 VHL subjects (7 males and 13 females, age at surgery 32+/-10 years) who underwent 28 operations in order to remove 48 CNS hemangioblastomas and 1 endolymphatic sac tumor.
  • Genetic testing revealing the presence of a VHL disease-causing mutation was a prerequisite for inclusion in the study.
  • Symptomatic hemangioblastomas were associated with a cyst or a syrinx in 22/27 circumstances (81%).
  • CONCLUSION: VHL-associated hemangioblastomas generally affect a young adult population and can be successfully removed, either when symptomatic, or when they reach a critical volume.
  • Microsurgery of hemangioblastomas has a favourable impact on survival and quality of life of VHL patients, although it is strongly influenced by preoperative conditions.
  • Transient surgical complications are possible, particularly with brainstem and spinal cord hemangioblastomas.
  • [MeSH-major] Cerebellar Neoplasms / etiology. Cerebellar Neoplasms / surgery. Hemangioblastoma / etiology. Hemangioblastoma / surgery. von Hippel-Lindau Disease / complications

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  • (PMID = 18500215.001).
  • [ISSN] 0390-5616
  • [Journal-full-title] Journal of neurosurgical sciences
  • [ISO-abbreviation] J Neurosurg Sci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
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95. Skalova A, Síma R, Bohus P, Curík R, Lukás J, Michal M: Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): report of two cases with analysis of the VHL gene. Pathol Res Pract; 2008;204(8):599-606
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Endolymphatic sac tumor (Heffner tumor) (ELST) is a very rare nonmetastasizing, locally aggressive low-grade adenocarcinoma of endolymphatic sac origin, which is linked to von-Hippel-Lindau disease (VHLD).
  • VHLD is an autosomal dominant disorder characterized by an inherited genetic abnormality of the VHL gene located on the short arm of chromosome 3 (3p26-p25).
  • The tissues obtained from Heffner tumor and cerebellar hemangioblastoma from the patient with inherited VHLD possess a point mutation in exon 1 of VHL gene.
  • [MeSH-major] Adenocarcinoma / genetics. Ear Neoplasms / genetics. Ear, Middle / pathology. Endolymphatic Sac / pathology. Gene Expression Regulation, Neoplastic. Skull Neoplasms / genetics. Temporal Bone / pathology. Von Hippel-Lindau Tumor Suppressor Protein / genetics. von Hippel-Lindau Disease / genetics
  • [MeSH-minor] Adult. Aged. DNA Mutational Analysis. Exons. Female. Genetic Predisposition to Disease. Humans. Introns. Neoplasm Invasiveness. Pedigree. Point Mutation

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  • (PMID = 18423895.001).
  • [ISSN] 0344-0338
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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96. Smoker WR, Gentry LR, Yee NK, Reede DL, Nerad JA: Vascular lesions of the orbit: more than meets the eye. Radiographics; 2008 Jan-Feb;28(1):185-204; quiz 325
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  • Vascular lesions of the orbit may be classified on the basis of their natural history, growth pattern, and histologic composition as capillary hemangiomas, venous vascular malformations, venous lymphatic malformations, arterial and arteriovenous lesions, or neoplasms.
  • Cavernous malformations are septate and well circumscribed, may exhibit progressive enhancement on delayed images, and do not involute.
  • Hemangioblastomas appear as enhancing mural nodules with associated cysts and serpentine flow voids on magnetic resonance (MR) images.

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  • (PMID = 18203938.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 61
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97. Zhou M, Shen D, Head JE, Chew EY, Chévez-Barrios P, Green WR, Chan CC: Ocular clusterin expression in von Hippel-Lindau disease. Mol Vis; 2007;13:2129-36
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ocular clusterin expression in von Hippel-Lindau disease.
  • Its mRNA is ubiquitously expressed, with high levels in von Hippel-Lindau (VHL) target organs such as the brain, liver, kidney, and adrenal medulla.
  • Decreased clusterin secretion has been reported in renal cell carcinoma associated with VHL disease.
  • The purpose of this study was to investigate ocular clusterin expression in VHL disease.
  • METHODS: This retrospective case series included nine eyes with retinal hemangioblastoma/hemangioma associated with VHL disease, one eye from a patient with a history of VHL disease and central nervous system hemangioblastomas but without ocular lesions, one surgically-excised optic nerve with optic nerve hemangioblastoma/hemangioma, and three normal control eyes.
  • Ocular specimens were evaluated by routine histology, immunohistochemistry for clusterin expression, and molecular detection of clusterin transcripts within ocular VHL hemangioblastomas compared with normal tissue from the same eye using microdissection and quantitative real-time PCR.
  • RESULTS: All retinal hemangioblastoma were composed of typical VHL tumor cells admixed with small vascular channels as well as glial cells.
  • Marked decrease of clusterin immunoreactivity was detected in all retinal hemangioblastoma and the optic nerve hemangioblastoma, whereas positive clusterin reactivity of the vascular and glial components was similar to that of normal retina.
  • Quantitative real-time PCR analysis confirmed the decrease of clusterin mRNA in the VHL associated retinal hemangioblastoma and optic nerve hemangioblastoma in five cases.
  • CONCLUSIONS: Clusterin shows possible important functions in tumor suppression by the VHL gene product (pVHL) and the potential to be a novel biomarker in retinal hemangioblastoma associated VHL disease.
  • Further investigation of clusterin may provide better understanding of retinal hemangioblastoma associated with VHL disease.
  • [MeSH-major] Clusterin / metabolism. Eye / metabolism. von Hippel-Lindau Disease / metabolism
  • [MeSH-minor] Adult. Female. Hemangioblastoma / etiology. Hemangioblastoma / metabolism. Hemangioblastoma / pathology. Humans. Immunohistochemistry. Male. Middle Aged. Neuroglia / metabolism. Optic Nerve Neoplasms / etiology. Optic Nerve Neoplasms / metabolism. Optic Nerve Neoplasms / pathology. Polymerase Chain Reaction. RNA, Messenger / metabolism. Retina / metabolism. Retinal Neoplasms / etiology. Retinal Neoplasms / metabolism. Retinal Neoplasms / pathology. Retinal Vessels / metabolism. Retrospective Studies

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  • (PMID = 18079682.001).
  • [ISSN] 1090-0535
  • [Journal-full-title] Molecular vision
  • [ISO-abbreviation] Mol. Vis.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000222-22; United States / Intramural NIH HHS / / Z99 EY999999
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Clusterin; 0 / RNA, Messenger
  • [Other-IDs] NLM/ NIHMS36212; NLM/ PMC2173882
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98. Minagawa T, Nishizawa S, Kamigaito M, Nakayama T, Okaneya T: [Sarcomatoid renal cell carcinoma with von Hippel-Lindau disease: a case report]. Nihon Hinyokika Gakkai Zasshi; 2007 Jul;98(5):723-6
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  • [Title] [Sarcomatoid renal cell carcinoma with von Hippel-Lindau disease: a case report].
  • We report a 68-year-old woman who had bilateral renal cell carcinoma (RCC) associated with von Hippel-Lindau (VHL) disease.
  • Surgical resection of a central nervous system hemangioblastoma had been done previously.
  • The patient eventually died of her disease at 5 years after resection of the right RCC.
  • [MeSH-major] Carcinoma, Renal Cell / complications. Kidney Neoplasms / complications. von Hippel-Lindau Disease / complications

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  • (PMID = 17682453.001).
  • [ISSN] 0021-5287
  • [Journal-full-title] Nihon Hinyōkika Gakkai zasshi. The japanese journal of urology
  • [ISO-abbreviation] Nippon Hinyokika Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 9008-11-1 / Interferons
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99. Taylor KL, Oates RK, Grane R, Leaman DW, Borden EC, Lindner DJ: IFN-alpha1,8 inhibits tumor-induced angiogenesis in murine angiosarcomas. J Interferon Cytokine Res; 2006 May;26(5):353-61
COS Scholar Universe. author profiles.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Interferon-alpha (IFN-alpha) has proved effective in the treatment of hemangiomas, hemangioblastomas, and Kaposi's sarcoma.
  • [MeSH-minor] Animals. Cell Line, Tumor. Cell Proliferation / drug effects. Chemokine CXCL10. Chemokines, CXC / biosynthesis. Chemokines, CXC / blood. Disease Progression. Humans. Mice. Xenograft Model Antitumor Assays

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  • (PMID = 16689662.001).
  • [ISSN] 1079-9907
  • [Journal-full-title] Journal of interferon & cytokine research : the official journal of the International Society for Interferon and Cytokine Research
  • [ISO-abbreviation] J. Interferon Cytokine Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01CA089344-04
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chemokine CXCL10; 0 / Chemokines, CXC; 0 / Interferon-alpha
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100. Merrill MJ, Oldfield EH: A reassessment of vascular endothelial growth factor in central nervous system pathology. J Neurosurg; 2005 Nov;103(5):853-68
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 2) the involvement of VEGF in CNS disorders other than tumors (traumatic and ischemic injuries arteriovenous malformations, inflammation); and 3) the role of VEGF in brain tumor biology (gliomas and the associated vasogenic edema, and hemangioblastomas).

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  • (PMID = 16304990.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Vascular Endothelial Growth Factor A
  • [Number-of-references] 191
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