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  1. 1. Medhat Mustafa, MD, Department of Neurosurgery Suez Canal University, Ismailia, Egypt
  2. 2. Hemangioblastomas
  4. 4. The tumor usually grows inside the parenchyma of the cerebellum, brain stem, or spinal cord; it is attached to the pia mater and gets its rich vascular supply from the pial vessels. However, extramedullary and extradural hemangioblastomas have also been described.
  5. 5. Upon gross examination, hemangioblastomas are usually cherry red in color. They may include a cyst that contains a clear fluid, but solid tumors are as common as cystic ones.
  6. 6. Epidemology Hemangioblastomas are relatively uncommon, accounting for 2% of all brain tumors and 2% to 10% of spinal cord tumors. Recurrent hemangioblastomas may signal that a patient has von Hippel-Lindau disease (VHL). VHL is a hereditary gene mutation that causes hemangioblastomas, cysts, and other tumors to grow
  7. 7. Most hemangioblastomas are located in the posterior cranial fossa; in that region, hemangioblastomas comprise 8-12% of neoplasms. Hemangioblastoma is the most common primary adult intraaxial posterior fossa tumor
  8. 8. The second most common location of hemangioblastomas is the spinal cord, where the frequency ranges from 2-3% of primary spinal cord neoplasms to 7-11% of spinal cord tumors. This tumor's occurrence in other locations, such as the supratentorial compartment, the optic nerve, the peripheral nerves, or the soft tissues of extremities is extremely rare.
  9. 9. Sex and age distribution Hemangioblastomas are more common in men than in women. In most clinical series, the male- to-female ratio is approximately 2:1. Although hemangioblastomas may develop at any age, they rarely affect children; the usual age at diagnosis is between the third and fifth decades.
  10. 10. The peak age of incidence has been noted to be between 20 and 50 years. Hemangioblastomas are uncommon but not rare in patients older than 65 years.
  11. 11. Von Hippel-Lindau disease Most hemangioblastomas arise sporadically. However, in approximately one quarter of all cases, they are associated with von Hippel- Lindau (VHL) disease, an autosomal dominant hereditary syndrome that includes retinal angiomatosis, central nervous system hemangioblastomas, and various visceral tumors most commonly involving the kidneys and adrenal glands.
  12. 12. This syndrome is classified as a phakomatosis, although it does not include any cutaneous manifestations. The syndrome has variable penetrance, but its dominant mode of transmission compels performing at least a screening of family members of patients diagnosed with VHL disease. In some patients with VHL disease, hemangioblastomas may produce erythropoietin- like substances, resulting in polycythemia at the time of diagnosis.
  13. 13. Types of Hemangioblastomas Hemangioblastomas are classified based on the type of fluid they contain and the size of the vascular channels inside the tumor. They are typically defined as cystic or solid, and are further broken down into four types:
  14. 14. Type 1: A simple cyst without a nodule (the rarest, at 5% of tumors) Type 2: A cyst with a mural nodule (60% of tumors) Type 3: A solid tumor (26% of tumors) Type 4: A solid tumor containing small internal cysts (9% of tumors)
  15. 15. Etiology Etiology of the hemangioblastoma is obscure, but its presence in various clinical syndromes may suggest an underlying genetic abnormality.
  16. 16. However, in 25% of cases, an inherited disorder called von Hippel-Lindau disease (VHL) causes the growth of hemangioblastomas and other types of tumors and cysts in the body. VHL disease is hereditary and occurs in 1 out of 36,000 people
  17. 17. Hemangioblastoma Symptoms The clinical presentation of hemangioblastomas usually depends on the anatomic location and growth patterns. Cerebellar lesions may present with signs of cerebellar dysfunction, such as ataxia and discoordination, or with symptoms of increased intracranial pressure due to associated hydrocephalus.
  18. 18. In general, intracranial hemangioblastomas present with a long history of minor neurologic symptoms that, in most cases, are followed by a sudden exacerbation, which may necessitate immediate neurosurgical intervention.
  19. 19. Patients with spinal cord lesions most frequently present with pain, followed by signs of segmental and long tract dysfunction due to progressive compression of the spinal cord. Patients with VHL disease may present with ocular or systemic symptoms due to involvement of other organs and systems.
  20. 20. Spontaneous hemorrhage is possible in both intraspinal and intracranial hemangioblastomas,but this risk is low, and tumors smaller than 1.5 cm carry virtually no risk of spontaneous hemorrhage.
  21. 21. Diagnosis 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.
  22. 22. 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.
  23. 23. In patients with hemangioblastomas, complete neural axis imaging usually is recommended to rule out multiple lesions, especially in those cases in which VHL syndrome is either diagnosed or clinically suspected.
  24. 24. 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.
  25. 25. 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 are rarely seen..
  26. 26. The exact histogenetic origin of stromal cells is unknown, but studies indicate that they may represent a heterogeneous population of abnormally differentiating mesenchymal cells of angiogeni lineage, with some morphologic features of endothelium, pericytes, and smooth- muscle cells
  27. 27. Two histologic 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. The reticular subtype is more commonly encountered; the cellular subtype is associated with higher probability of recurrence.
  28. 28. Treatment Because hemangioblastomas are benign tumors and generally are not invasive in nature they may be cured by surgical excision. Therefore, surgical resection is considered a standard of treatment and should be offered to the patient unless the risk of operation outweighs its potential benefits.
  29. 29. The tumors usually are well demarcated from the surrounding brain or spinal cord, but this border of separation does not contain any particular membrane or capsule.
  30. 30. Thorough evaluation of preoperative imaging studies is the key to the safest possible exposure of the tumor. In addition to MRI and CT scans, review the angiography findings to identify the principal blood supply to the tumor mass.
  31. 31. Other therapeutic modalities include endovascular embolization of the solid component of the tumor, which may decrease the vascularity of the tumor and lower blood loss during its resection, and stereotactic radiosurgery of the tumor using either a linear accelerator or a gamma knife.
  32. 32. Regarding general surgical management, having blood products available for transfusion is very important because the vascular character of hemangioblastomas may result in serious intraoperative blood loss. Additionally, anesthesia for patients with VHL disease may be quite challenging because of associated renal and endocrine dysfunction.
  33. 33. The surface of the tumor may be coagulated with wide bipolar forceps; however, avoid penetration of the tumor itself because of its extreme vascularity and difficulties with hemostasis. Try to dissect the tumor circumferentially by careful coagulation and cutting the small feeding vessels and adhesions between the tumor and the surrounding brain or spinal cord and by putting cottonoid strips into the developing plane to avoid direct pressure on the brain or spinal cord tissue.
  34. 34. If an associated hydrocephalus exists, it must be addressed separately, usually by means of external ventricular drainage (EVD) prior to tumor resection. After the tumor is removed, the need for permanent shunt placement may be determined by the patient's response to EVD clamping. In most cases, an intramedullary syrinx does not require a separate drainage procedure because it usually resolves after tumor removal.
  35. 35. Outcome and Prognosis Long-term results of hemangioblastoma management are generally favorable. Advancement of neuroimaging methods, improvements in microsurgical technique, and the addition of preoperative embolization have significantly lowered morbidity and mortality associated with hemangioblastoma surgery.
  36. 36. Subarachnoid dissemination of hemangioblastomas is extremely rare, and local recurrences after complete tumor resection seem to be more frequent in patients with von Hippel-Lindau (VHL) disease, in patients diagnosed at a young age, and in patients with multiple hemangioblastomas.
  37. 37. The recurrence rates vary in different surgical series but generally remain less than 25%. Histologic subtype has been found to correlate with a probability of hemangioblastoma recurrence, with a 25% recurrence rate in cellular subtype and an 8% recurrence rate in reticular subtype.