eMedicine Specialties > Neurosurgery > Neoplasm

Hemangioblastoma: Workup

Author: Konstantin V Slavin, MD, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago; Head, Section of Stereotactic and Functional Neurosurgery, University of Illinois at Chicago
Contributor Information and Disclosures

Updated: Mar 24, 2009

Workup

Laboratory Studies

  • Perform blood tests to help reveal associated lesions that may be a part of the VHL disease complex. Unfortunately, finding polycythemia does not help in diagnosing the tumor.

Imaging Studies

  • The diagnostic workup of suspected hemangioblastomas must include, in addition to history, physical, and thorough neurological examination, complete neural axis imaging and abdominal CT scan or ultrasound. The goal of these additional tests is to reveal associated lesions that may be a part of VHL disease complex.
  • Radiographically, hemangioblastomas are best diagnosed with MRI.22 MRI of hemangioblastomas usually shows an enhancing mass clearly delineated from the surrounding brain or spinal cord tissue. The tumor tissue may be hypointense or isointense on precontrast T1-weighted images and hyperintense on T2-weighted images.
  • Plain radiographs usually do not aid in diagnosis. Myelography and cisternography, which were considered the tests of choice in the past, now are almost never used in the diagnostic workup of hemangioblastomas.
  • Plain computed tomography (CT) scan may reveal hypodensity of the tumoral cyst and associated hydrocephalus. CT scans with intravenous contrast show uniform enhancement of the tumor nodule that, in association with the adjacent cyst, may be extremely characteristic of posterior fossa hemangioblastomas.
  • Cerebral and spinal angiography reveals a highly vascular tumor blush, and this diagnostic modality may be extremely useful for assessing the vascular supply to the tumor. This information may help the surgeon during tumor resection.
  • In patients with hemangioblastomas, complete neural axis imaging usually is recommended in order to rule out multiple lesions, especially in those cases in which VHL syndrome is either diagnosed or clinically suspected.

Other Tests

  • Perform a detailed ophthalmologic evaluation to help reveal associated lesions that may be a part of the VHL disease complex.

Histologic Findings

Histologically, hemangioblastomas are vascular neoplasms. In addition to relatively normal-appearing endothelial cells that line capillary spaces, hemangioblastomas have 2 distinct cellular components that may occur in the same tumor in different proportions. The first type is small, perivascular, endothelial cells that have dark compact nuclei and sparse cytoplasm. Cells of the second type contain multiple vacuoles and granular eosinophilic cytoplasm rich in lipids. These stromal cells may show some nuclear pleomorphism, but mitotic figures rarely are seen. The exact histogenetic origin of stromal cells is unknown, but the latest studies indicate that they may represent a heterogeneous population of abnormally differentiating mesenchymal cells of angiogenic lineage, with some morphological features of endothelium, pericytes, and smooth-muscle cells.23

Two histological subtypes (cellular and reticular) have been described in primary hemangioblastomas of the central nervous system and have been found to correlate with the probability of tumor recurrence.24 The reticular subtype is more commonly encountered; the cellular subtype is associated with higher probability of recurrence.

No histologic grading system exists for hemangioblastomas.

Staging

No established histologic grading system exists for hemangioblastomas.

More on Hemangioblastoma

Overview: Hemangioblastoma
Workup: Hemangioblastoma
Treatment: Hemangioblastoma
Follow-up: Hemangioblastoma
Multimedia: Hemangioblastoma
References
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References

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Further Reading

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Keywords

hemangioblastoma, Lindau tumor, Lindau's tumor, capillary hemangioblastoma, vascular neoplasm, tumor, tumors, brain tumors, brain tumor, head tumors, neoplasm, meningeal tumor, intracranial neoplasm, intracranial tumor, vascular tumor, von Hippel-Lindau disease, VHL disease, Gamma Knife 

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Contributor Information and Disclosures

Author

Konstantin V Slavin, MD, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago; Head, Section of Stereotactic and Functional Neurosurgery, University of Illinois at Chicago
Konstantin V Slavin, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Pain Society, American Society of Stereotactical and Functional Neurosurgery, and Congress of Neurological Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Duc Hoang Duong, MD, Professor, Chief Physician, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles R Drew University
Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association, Congress of Neurological Surgeons, and North American Skull Base Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ryszard M Pluta, MD, PhD, Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences at Warsaw, Poland; Senior Researcher, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH
Ryszard M Pluta, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons and Polish Society of Neurosurgeons
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD, Former Medical Director, Northstar Neuroscience, Inc
Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons
Disclosure: Nothing to disclose.

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