Hemangioblstoma: Presentation, Diagnosis & Treatment

1. HEMANGIOBLASTOMA
DR. FARRUKH JAVEED
NEUROSURGERY JPMC

2. HAMANGIOBLASTOMA
 These are slow growing benign vascular neoplasm.
 They arises from the hemangioblast cells.
 Classified as WHO grade I tumors.
 There are 2 Subtypes:
 Reticular; more common
 Cellular

3. Location
 Hemangioblastomas are found nearly exclusively(over 95%) in the
 brainstem,
 spinal cord(2 to 10%)
 cerebellum
 They account for 7% to 12% of posterior fossa tumors.
 About 1-5% of hemangioblastomas are found in the supratentorial compartment,
most frequently (30%) originate in the region of the pituitary stalk and tuber
cinereum.
 10% of patients with VHL disease have supratentorial hemangioblastomas.
 Other locations that can be involved are:
 Optic Nerves
 Peripheral Nerves
 Soft Tissues

4. Epidemiology
 M:F is 2:1
 Age: 30 - 50
 Uncommon (1-3% of all intracranial neoplasms)
 Hemangioblastoma-related symptoms typically occur earlier in patients with
VHL disease (30 to 40 years) than in those with sporadic tumors (40 to 50
years).
 Sometimes referred to as Lindau Tumors

5. ETIOLOGY/ASSOCIATION
 Sporadic (75%)
 Von Hippel Lindau Disease (25%)

6. VON HIPPEL-LINDAU DISEASE
 a familial neoplasia syndrome with autosomal dominant transmission
 caused by a VHL gene germline mutation.
 Visceral manifestations include renal cysts, renal carcinomas, pancreatic
cysts, pancreatic neuroendocrine tumors, pheochromocytomas, and
adnexal organ cystadenomas (broad ligament and epididymis).
 CNS manifestations include hemangioblastomas (the most common
manifestation of VHL disease) and endolymphatic sac tumors.
 the primary cause of VHL disease–related death is renal cell carcinoma or
CNS hemangioblastoma.

7. VON HIPPEL-LINDAU DISEASE 7

8. PATHOLOGIC FINDINGS
 Hemangioblastomas are bright red or red-orange and are invariably
associated with intense vascularity.
 Typically, large abnormal veins are found at resection to be draining these
tumors.
 Hemangioblastomas are well-circumscribed, encapsulated tumors that
occasionally have intratumoral cysts but more frequently are associated
with peritumoral cysts whose walls are composed of compressed brain
tissue and reactive gliosis.
 Histologically, the tumors are characterized by proliferation of stromal cells
and endothelial cells.

9. PATHOLOGIC FINDINGS
 These tumors often appear histologically similar to renal cell carcinoma
metastases.
 Further, because patients with VHL disease often have contemporaneous
renal cell carcinomas that can metastasize to the CNS or metastasize to
hemangioblastomas, immunohistochemical differentiation between renal
cell carcinoma and hemangioblastoma may be necessary.
 Jarrell and colleagues reported that 8% of a series of CNS
hemangioblastomas removed from consecutive patients with VHL disease
had either a renal cell carcinoma or pancreatic neuroendocrine tumor that
had metastasized to a coexisting hemangioblastoma.

10. PERITUMORAL CYST
 About 80% of CNS hemangioblastomas are associated with a peritumoral
cyst.
 About 70% of symptomatic cerebellar and brainstem hemangioblastomas
are associated with peritumoral cyst, and more than 90% of symptomatic
spinal cord hemangioblastomas are associated with peritumoral cyst
(syringomyelia).
 Alternatively, asymptomatic cerebellar, brainstem, or spinal cord
hemangioblastomas are uncommonly associated with peritumoral cysts
(only 5% to 10% of tumors with cysts).

11. PERITUMORAL CYST FORMATION
 Increased vascular permeability of the hemangioblastoma.
 Extravasation of a plasma ultrafiltrate into the tumor interstitial spaces.
 High interstitial tumor pressure then drives this fluid into the surrounding CNS
parenchyma.
 Peritumoral edema forms when the resorptive capacity of the peritumoral
tissue is exceeded.
 As the mismatch between fluid production and tissue resorption increases, the
surrounding tissues swell owing to solid stresses (stretching) that favor cyst
formation.
 The formation of a cyst changes the interstitial flow patterns, and most of the
excess interstitial fluid flows along the path of least resistance into the
peritumoral cyst, expanding it.

12.  Because the hemangioblastoma is the
source of extravasated plasma ultrafiltrate,
peritumoral edema and cysts resolve after
tumor removal, and treatment does not
require cyst wall resection or fenestration.

13. CLINICAL FEATURES
 Depend on location
 Cerebellar Hemangioblastomas
 headache (70% to 80% of patients)
 gait ataxia (55% to 65%)
 dysmetria (30% to 65%)
 hydrocephalus (20% to 30%)
 nausea and vomiting (5% to 30%)
 Brainstem hemangioblastomas
 hypoesthesia (40% to 55%)
 gait ataxia (20% to 30%)
 dysphagia (20% to 30%)
 hyperreflexia (20% to 25%)
 headache (10% to 20%)
 disorders of appetite and feeding (2% to 5%)

14.  Spinal cord hemangioblastomas
 hypoesthesia (80% to 90%)
 weakness (60% to 70%)
 gait ataxia (50% to 65%)
 hyperreflexia (40% to 60%)
 pain (10% to 30%).
 Eye
 Visual Changes like diplopia
 Elevated Erythropoietin
 Secondary polycythemia

15. RADIOLOGY
MRI of the brain and spine
Angiography
CT Abdomen - evaluate the kidneys,
pancreas, adrenals
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16. MRI with Contrast
 The most sensitive and accurate imaging modality.
 Discretely enhance on T1-weighted MRI sequences after infusion of a
gadolinium-based contrast agent.
 Even small tumors (2 to 3 mm in diameter) can be detected.
 Peritumoral edema and cysts are best detected and monitored with fluid-
attenuated inversion recovery (FLAIR) or T2-weighted MRI sequences.
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17.  T1-weighted, contrast-enhanced axial
image of a cerebellar hemangioblastomas
(solidly enhancing area) with an associated
cyst (homogenous associated dark region)
in a 40-year-old woman with VHL disease.

18.  Midsagittal T1-weighted postcontrast
image of a medullary hemangioblastoma
(solidly enhancing region) with associated
brainstem edema (surrounding darker
region) in a 12-year-old girl with VHL
disease.

19.  Midsagittal postcontrast T1-weighted image of
the cervical region of the spinal cord of a 50-
year-old man with VHL disease.

20.  Postcontrast sagittal (A) and axial (B) images
reveal an intensely enhancing
hemangioblastoma with an associated syrinx.
T2-weighted sagittal (C) and axial (D) images
precisely define the extent of the syrinx and
the edema associated with this tumor.
A
B
C
D

21. Angiography
 Angiography of vessels supplying and draining a hemangioblastoma
reveals a highly vascular tumor nodule with prolonged contrast staining
that can be associated with an avascular peritumoral cyst.
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22. MANAGEMENT
 Screening for VHL gene using molecular genetic testing.
 Endovascular embolization
 Open Surgical resection
 Radiosurgery
 Antiangiogenic treatment

23. Von Hippel-Lindau Disease–Related versus
Sporadic Hemangioblastomas
 The management strategy for hemangioblastomas of these 2 categories are different.
 Because most patients with VHL disease have multiple CNS hemangioblastomas and because
the growth rate of these tumors is unpredictable, treatment of individual tumors is generally
postponed until they become symptomatic. They maintain excellent neurological function
over long-term evaluation, and unnecessary surgical resection can be avoided.
 Alternatively, patients with sporadic hemangioblastomas are frequently investigated because
they have symptoms, and the diagnosis of hemangioblastoma cannot be confirmed until
resection. Thus, to relieve symptoms and to provide a diagnosis in sporadic cases, surgery
may be required.

24. Surgical Resection
 The treatment of choice for CNS hemangioblastomas is complete
resection.
 Microsurgical resection is curative, and most craniospinal
hemangioblastomas can be completely and safely resected.
 When the tumor is associated with a cyst (regardless of anatomic location),
the gliotic cyst walls are left undisturbed, because they are not neoplastic
and simply removing the source of the cyst (the tumor) inactivates the cyst,
which and will collapse. This is the case regardless of the location of the
tumor along the craniospinal axis.
 When complete resection of the tumor is accomplished, recurrence is rare,
and stabilization or improvement of symptoms occurs in more than 90% of
patients.

25. Cerebellar Hemangioblastomas
 Cerebellar hemangioblastomas are commonly (74%) located in the posterior
and/or medial cerebellar regions. A midline suboccipital approach can most
often be used to resect such tumors.
 A midline incision through the skin and dermis from the inion to the second
cervical spinous process is made.
 The nuchal musculature is opened in the midline and stripped laterally in the
subperiosteal plane over the suboccipital region and first and second cervical
laminae.
 A suboccipital craniectomy is created with a high-speed drill and rongeurs
from 1 cm above the upper edge of the tumor or the inferior edge of the
transverse sinus (whichever is lower) to the foramen magnum.
 A laminectomy of the first cervical lamina can be used for additional caudal
exposure.
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26.  A Y-shaped dural incision is made.
 The dural edges are secured laterally and superiorly.
 With a microscissors, the arachnoid is sharply opened to expose the underlying
cerebellum and tumor.
 If necessary, increased posterior fossa pressure can be reduced by removal of
cerebrospinal fluid from the cisterna magna or needle decompression of
peritumoral or intratumoral cysts.
 The pia at the tumor-pia junction is circumferentially incised, providing clear
exposure of the tumor capsule–cerebellum interface.
 Deeper circumferential dissection precisely at the tumor capsule–cerebellum
interface is performed to remove the tumor from the surrounding tissue.
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27.  Once the capsule is clearly identified, the hemangioblastoma capsule–
cerebellum interface is precisely defined, and the tumor is removed by
means of deeper circumferential dissection at the interface. Tumor-
associated vessels (those entering and leaving the tumor) are cauterized
with bipolar cautery and sharply divided.
 After hemangioblastoma removal, the dura, nuchal musculature, fascia,
and subcutaneous/cutaneous layers are closed in standard fashion. A
sterile dressing is applied to the wound
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28. Brainstem Hemangioblastomas
 Because most brainstem hemangioblastomas (60%) are located in the
region of the medullary obex, a midline suboccipital-cervical approach is
used to gain access to such tumors.
 The dura and arachnoid are opened as described for cerebellar
hemangioblastoma resection.
 For midline hemangioblastomas that do not extend to the posterior
surface of the brainstem, a midline pial incision is made, and the posterior
median raphe is separated to access the deeper tumors.

29.  As with spinal cord hemangioblastomas, maintenance of a well-defined
interface of the bright red or red-orange tumor surface with the
surrounding brainstem is critically important because the inability to define
this tissue plane and dissection into the brainstem risk serious neurological
impairment.
 After hemangioblastoma removal, the dura, nuchal musculature, fascia,
and subcutaneous and cutaneous layers are closed in standard fashion. A
sterile dressing is put on the wound.
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30. Spinal Cord Hemangioblastomas
 Because most (96%) spinal cord hemangioblastomas are located posterior
to the dentate ligament, a direct posterior approach is most often used to
remove them.
 Hemangioblastomas located in the anterior (anterior to the dentate
ligament) or anterolateral region of the spinal cord may be accessed with
an anterior or anterolateral approach.
 These direct approaches to anteriorly located tumors allow for minimal
manipulation and rotation of the spinal cord during resection and can be
associated with improved postoperative outcome.
 Occasionally, anteriorly located tumors associated with rotation of the
spinal cord can be best accessed from a posterior approach.
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31. Spinal Cord Hemangioblastomas
 The incision site is prepared and a midline incision over the spinous
processes is made that provides exposure of at least one spinous process
above and one below the rostral and caudal poles of the tumor.
 After skin incision, the paraspinous musculature is opened in the midline
and stripped laterally in the subperiosteal plane over the involved lamina.
 With a high-speed drill and rongeurs, laminectomies are created.
 After bone removal (laminectomies), the dura is incised in the midline
while the underlying arachnoid is kept intact.
 The dura is reflected laterally and temporarily secured with tacking sutures
to the paraspinous musculature.
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32. Spinal Cord Hemangioblastomas
 The operative microscope is used to sharply open the arachnoid with
microscissors.
 Once the arachnoid is opened and the tumor is exposed, vessels entering
the tumor are coagulated at the tumor-pia interface and then sharply
transected starting at the caudal pole of the tumor.
 The hemangioblastoma capsule–spinal cord pia interface is sharply opened
with a diamond knife and microscissors in a circumferential manner.
 Dissection is continued at the rostral and caudal poles of the tumor with
gentle retraction and elevation of the poles of the hemangioblastoma,
which permits the deep anterior margin of the tumor to be visualized and
separated from the spinal cord.
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33. Spinal Cord Hemangioblastomas
 For hemangioblastomas that originate in the dorsal nerve root entry zone,
sensory nerve rootlets embedded in the tumor are cauterized and sharply
interrupted at the margin of the hemangioblastoma.
 For tumor with associated peritumoral syrinx, avoid entering the syrinx if
possible. Opening or drainage of an associated syrinx can result in
magnification of the physiologic pulsations of the spinal cord and tumor. This
accentuation of physiologic tumor movement during resection can make
removal more difficult.
 Removal of the hemangioblastoma inactivates the syrinx, so syrinx drainage
is not necessary.
 After hemangioblastoma removal, the dura, paraspinous musculature, fascia,
and subcutaneous and cutaneous layers are closed in standard fashion. A
sterile dressing is put on the wound.

35.  Intraoperative view of a hemangioblastoma of
the cervical spinal cord. Hemangiomas are
bright red or orange-yellow, owing to the high
lipid content of their stromal cells, and are
invariably associated with large tortuous
arterialized draining veins.

36.  Preoperative (A) and postoperative (B)
sagittal T2-weighted magnetic
resonance images from a patient with
von Hippel-Lindau disease and a
hemangioblastoma in the cervical
segment of the spinal cord and
surrounding syrinx. After selective
resection of the hemangioblastoma,
complete resolution of the associated
syrinx and surrounding edema has
occurred.
A B

37. POST-OP COMPLICATIONS
 Hemorrhage
 Hydrocephalus
 Diplopia
 Hemiparesis

38. Outcome
 Generally curable with surgery
 Local recurrence after surgery is higher with the following:
 VHL Syndrome
 Multiple Hemangioblastomas
 Younger Age
 Cellular Histology
 Cellular type has a 20 - 25% recurrence rate
 Reticular type has a 5 - 10% recurrence rate
 Subarachnoid dissemination is rare.

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