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A Robust Allegation of Von Hippel-Lindau (VHL) Associated Hemangioblastoma in the Central Nervous System (CNS): A Case Report

Received: 22 October 2013     Published: 10 January 2014
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Abstract

Haemangioblastoma, a benign vascular tumor derived from the capillary of endothelial cells, is an inherited disorder due to an autosomal dominant trait. The prevalence rates accounted for 1% to 2.5% of all intracranial tumors and 2% to 3% of all intramedullary spinal cord tumors. We report the infrequent case of a robust allegation of von Hippel-Lindau (VHL) associated haemangioblastoma in the central nervous system of a man. A 20 years old male presented to the hospital with the chief complaint of progressive chronic cephalgia three months prior to hospitalization. He complained also of blurred vision on neurologic examination; there is papilla edema with suspicion of retinal edema. Radiological examination result suggested a cerebellar haemangioblastoma dextra with noncommunicating hydrocephalus. After the first operation (installation of VP shunting) and subsequent operation (excision of the tumor), the pathological anatomy results revealed a cavernous angioma.

Published in International Journal of Medical Imaging (Volume 2, Issue 1)
DOI 10.11648/j.ijmi.20140201.11
Page(s) 1-4
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2014. Published by Science Publishing Group

Keywords

Haemangioblastoma, CNS, CT-Scan, Von Hippel-Lindau Diseases

References
[1] James TJ, Yung WKA. Hemangioblastoma. In : Goetz CG, ed. Text book of clinical neurology. 2nd ed. Philadelphia : Saunders, 2003; 1037.
[2] Dahret W. Hemangioblastoma of CNS. In : Grayson TH, ed. Radiology review manual. 2nd ed. Baltimore : Williams & Wilkins, 1993: 180-1.
[3] Gilroy J. Hemangioblastoma. In : Basic neurology. 3rd ed. New York : McGraw-Hill, 2000: 416-7.
[4] Lindau A. Studien über Kleinhirnzysten. Bau, Pathogenese und Beziehungen zur Angiomatosis retinae. Acta Pathol Microbiol Scand 1926; S1: 1–128.
[5] von Hippel E. Ueber eine sehr seltene Erkrankung der Netzhaut. Klinische Beobachtungen. A von Graefe’s Arch Ophthalmol 1904; 59: 83–106.
[6] Vates GE, Auguste KI, Berger MS. Hemangioblastomas. In : Berger MS, Prades MD, eds. Textbook of neuro-oncology. 1st ed. Philadelphia : Elsevier Saunders, 2005: 294-300.
[7] Solomon RA, Spellman JP, Mohr JP. Hemangioblastoma. In : Rowland LP, ed. Merritt’s neurology. 10th ed. Philadelphia : Lippincott Williams & Wilkins, 2000; 374-5
[8] Neumann HPH, Eggert HR, Weigel K, et al. Hemangioblastomas of the central nervous system. A 10 year study with special reference to von Hippel–Lindau syndrome. J Neurosurg 1989; 70:24–30.
[9] Bohling T, Plate KH, Haltia M, et al: von Hippel-Lindau disease and capillary haemangioblastoma. In Cavanee WK (ed): World Health Organization Classification of Tumours: Pathology and Genetics of Tumors of the Nervous System. Lyon, France, IARC Press, 2000.
[10] Frantzen C, Links TP, Giles RH. Von Hippel-Lindau Disease. 2000 May 17 [Updated 2012 Jun 21]. In: Pagon RA, Bird TD, Dolan CR, et al., editors. GeneReviews™ [Internet]. Seattle (WA): University of Washington, Seattle;1993-.Available from: http://www.ncbi.nlm.nih.gov/books/NBK1463/
[11] Resche F, Moisan JP, Mantoura J, de Kersaint-Gilly A, Andre MJ, Perrin-Resche I et al. Haemangioblastoma, haemangioblastomatosis, and von Hippel-Lindau disease. Adv Tech Stand Neurosurg 1993; 20:197- 304: 197-304.
[12] Friedrich CA. Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer 1999; 86(11 Suppl):2478-2482.
[13] Richard S, Campello C, Taillandier L, Parker F, Resche F. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. French VHL Study Group. J Intern Med 1998; 243(6):547-553.
[14] Solomon RA, Spellman JP, Mohr JP. Hemangioblastoma. In : Rowland LP, ed. Merritt’s neurology. 10th ed. Philadelphia : Lippincott Williams & Wilkins, 2000; 374-5.
[15] Victor M, Ropper AH. Hemangioblastoma of the cerebellum. In : Adams and Victor’s principles of neurology. 7th ed. New York : McGraw-Hill, 2001; 705-6.
[16] Vates GE, Auguste KI, Berger MS. Hemangioblastomas. In : Berger MS, Prades MD, eds. Textbook of neuro-oncology. 1st ed. Philadelphia: Elsevier Saunders, 2005: 294-300.
[17] Haughton VM, Daniels DL. Hemangioblastoma. In: William AL, Haughton VM, eds. Cranial computed tomography. ST Louis: The CV Mosby Company, 1985: 382-6.
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  • APA Style

    Muhammad Yunus Amran, Hasmawaty Basir, Andi Kurnia Bintang, Muhammad Akbar. (2014). A Robust Allegation of Von Hippel-Lindau (VHL) Associated Hemangioblastoma in the Central Nervous System (CNS): A Case Report. International Journal of Medical Imaging, 2(1), 1-4. https://doi.org/10.11648/j.ijmi.20140201.11

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    ACS Style

    Muhammad Yunus Amran; Hasmawaty Basir; Andi Kurnia Bintang; Muhammad Akbar. A Robust Allegation of Von Hippel-Lindau (VHL) Associated Hemangioblastoma in the Central Nervous System (CNS): A Case Report. Int. J. Med. Imaging 2014, 2(1), 1-4. doi: 10.11648/j.ijmi.20140201.11

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    AMA Style

    Muhammad Yunus Amran, Hasmawaty Basir, Andi Kurnia Bintang, Muhammad Akbar. A Robust Allegation of Von Hippel-Lindau (VHL) Associated Hemangioblastoma in the Central Nervous System (CNS): A Case Report. Int J Med Imaging. 2014;2(1):1-4. doi: 10.11648/j.ijmi.20140201.11

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  • @article{10.11648/j.ijmi.20140201.11,
      author = {Muhammad Yunus Amran and Hasmawaty Basir and Andi Kurnia Bintang and Muhammad Akbar},
      title = {A Robust Allegation of Von Hippel-Lindau (VHL) Associated Hemangioblastoma in the Central Nervous System (CNS): A Case Report},
      journal = {International Journal of Medical Imaging},
      volume = {2},
      number = {1},
      pages = {1-4},
      doi = {10.11648/j.ijmi.20140201.11},
      url = {https://doi.org/10.11648/j.ijmi.20140201.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijmi.20140201.11},
      abstract = {Haemangioblastoma, a benign vascular tumor derived from the capillary of endothelial cells, is an inherited disorder due to an autosomal dominant trait. The prevalence rates accounted for 1% to 2.5% of all intracranial tumors and 2% to 3% of all intramedullary spinal cord tumors. We report the infrequent case of a robust allegation of von Hippel-Lindau (VHL) associated haemangioblastoma in the central nervous system of a man. A 20 years old male presented to the hospital with the chief complaint of progressive chronic cephalgia three months prior to hospitalization. He complained also of blurred vision on neurologic examination; there is papilla edema with suspicion of retinal edema. Radiological examination result suggested a cerebellar haemangioblastoma dextra with noncommunicating hydrocephalus. After the first operation (installation of VP shunting) and subsequent operation (excision of the tumor), the pathological anatomy results revealed a cavernous angioma.},
     year = {2014}
    }
    

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    AU  - Muhammad Yunus Amran
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    AB  - Haemangioblastoma, a benign vascular tumor derived from the capillary of endothelial cells, is an inherited disorder due to an autosomal dominant trait. The prevalence rates accounted for 1% to 2.5% of all intracranial tumors and 2% to 3% of all intramedullary spinal cord tumors. We report the infrequent case of a robust allegation of von Hippel-Lindau (VHL) associated haemangioblastoma in the central nervous system of a man. A 20 years old male presented to the hospital with the chief complaint of progressive chronic cephalgia three months prior to hospitalization. He complained also of blurred vision on neurologic examination; there is papilla edema with suspicion of retinal edema. Radiological examination result suggested a cerebellar haemangioblastoma dextra with noncommunicating hydrocephalus. After the first operation (installation of VP shunting) and subsequent operation (excision of the tumor), the pathological anatomy results revealed a cavernous angioma.
    VL  - 2
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Author Information
  • Hasanuddin University Teaching Hospital, Department of Neurology, Faculty of Medicine, Hasanuddin University, Makassar, South Sulawesi, Indonesia

  • Hasanuddin University Teaching Hospital, Department of Neurology, Faculty of Medicine, Hasanuddin University, Makassar, South Sulawesi, Indonesia

  • Hasanuddin University Teaching Hospital, Department of Neurology, Faculty of Medicine, Hasanuddin University, Makassar, South Sulawesi, Indonesia

  • Hasanuddin University Teaching Hospital, Department of Neurology, Faculty of Medicine, Hasanuddin University, Makassar, South Sulawesi, Indonesia

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