- Email: [email protected]
Hemangioblastoma of Cerebral Aqueduct Removed via Sitting, Supracerebellar Intracollicular Approach
Case Report
Hemangioblastoma of Cerebral Aqueduct Removed via Sitting, Supracerebellar Intracollicular Approach Robert G. Briggs1, Ryan G. Jones1, Andrew K. Conner1, Parker G. Allan1, Hannah B. Homburg1, B. David Maxwell1, Kar-Ming Fung2, Michael E. Sughrue3
Key words Cerebral aqueduct - Colliculus - Hemangioblastoma -
Abbreviations and Acronyms CSF: Cerebrospinal fluid VHL: Von Hippel-Lindau disease From the Departments of 1Neurosurgery and 2Pathology, University of Oklahoma Health Science Center, Oklahoma City, Oklahoma, USA; and 3Center for Minimally Invasive Neurosurgery, Prince of Wales Private Hospital, Sydney, Australia To whom correspondence should be addressed: Michael E. Sughrue, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2019) 127:155-159. https://doi.org/10.1016/j.wneu.2019.03.206 Journal homepage: www.journals.elsevier.com/worldneurosurgery
- BACKGROUND:
Tumors protruding into the cerebral aqueduct are rare, and tumors arising from within the cerebral aqueduct are rarer still.
- CASE
DESCRIPTION: In this report, we discuss the presentation and clinical outcome of a 65-year-old man who presented to us with symptoms of hydrocephalus. Prior imaging had revealed a small enhancing nodule within the cerebral aqueduct. In the 6 months between initial imaging and our seeing the patient, the tumor demonstrated substantial interval growth, so the patient was offered resection. The tumor was accessed using a sitting, supracerebellar, intracollicular approach, which allowed for gross total resection of the mass without complication. Histopathology later revealed the lesion to be a hemangioblastoma. Two years after surgery, the patient was doing well with no neurologic deficits.
- CONCLUSIONS:
We report the first case of an aqueductal hemangioblastoma and describe our use of a sitting, supracerebellar, intracollicular approach to access tumors occupying this cerebrospinal fluid space.
Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
INTRODUCTION Hemangioblastomas are benign, highly vascularized tumors that typically grow in the posterior fossa, especially the cerebellum.1 However, about 2%15% of hemangioblastomas grow in the brainstem.2-4 They can occur sporadically or concurrently with von Hippel-Lindau disease (VHL). Sporadic hemangioblastomas tend to be solitary and occur in patients 40 years and older, while VHLrelated hemangioblastomas are often smaller, tend to appear in multiples, and occur in patients 10 to 15 years younger.3,5-8 Both variants of hemangioblastoma are associated with mutations in the VHL gene.9,10 Hemangioblastomas are not well associated with a particular set of symptoms, though headache and dizziness are common.1 After diagnosis, these tumors may be closely monitored or removed,2 though some recommend removal of all symptomatic hemangioblastomas.3,4 Removal en bloc is strongly recommended.2,11
It is rare that any tumors protrude into the cerebral aqueduct, and it is extremely rare that they arise from within it.12 Occlusion of the cerebral aqueduct caused by the tumor can lead to aqueductal stenosis and noncommunicating hydrocephalus.12-14 This low incidence is fortunate as aqueductal tumors can pose substantial challenges. First, they are surrounded entirely by the midbrain, so the risk of damage to a vital neurologic structure is high.15 In addition, although the aqueduct is a cerebrospinal fluid (CSF) space, the long axis of this space requires an approach through either the lateral and third ventricles from above or the fourth ventricle from below. Larger tumors that have displaced normal brain parenchyma provide some access to this deep space, but smaller tumors pose a challenge to neurosurgeons. The surgeon can attempt a complex and difficult resection at presentation16 or wait for the tumor to grow, thereby displacing the brain, opening the aqueduct and possibly simplifying the procedure. In this study, we report the first case of a hemangioblastoma that arose
WORLD NEUROSURGERY 127: 155-159, JULY 2019
entirely within the cerebral aqueduct. We used a sitting, supracerebellar, intracollicular approach to gain access to a growing lesion in the cerebral aqueduct later identified as a hemangioblastoma. Here, we also review the literature related to lesions originating in the aqueduct. CASE REPORT The patient was a 65-year-old man who initially presented with hydrocephalus that was treated with an endoscopic third ventriculostomy at an outside hospital. Imaging at that time demonstrated a small enhancing nodule within the cerebral aqueduct, which was simply observed. The lesion demonstrated interval growth over a 6-month period before the patient was referred to us (Figure 1). After detailed review of the imaging, we determined that the best approach to access this tumor was via a trajectory between the colliculi. The patient was taken to the operating room, where a suboccipital craniotomy was performed in the sitting position (Figure 2).
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HEMANGIOBLASTOMA OF THE AQUEDUCT
injections. The patient ultimately required a ventriculoperitoneal shunt to manage his hydrocephalus. At the 2-year follow-up, the tumor had not recurred, and the patient was fully functional with no new neurologic deficits. DISCUSSION
Figure 1. Preoperative T1-weighted magnetic resonance imaging (A) before and (B) after administration of gadolinium reveals a contrast-enhancing lesion within the cerebral aqueduct (white arrow). Note the dilated temporal horns indicating hydrocephalus. (C) Axial and (D) sagittal postoperative imaging demonstrates complete resection of the lesion (blue arrow). A trajectory through the colliculi was used to remove the lesion en bloc.
A supracerebellar approach was used to reach the tectal plate and split the cerebellomesoencephalic fissure. We made a small incision through the middle of the colliculi using a CO2 laser to reach above and below the tumor’s superior and inferior poles. We identified the tumor, which was clearly vascular in origin, and used a Rhoton #2 dissector to free the mass and remove it en bloc from the aqueduct. Histopathology revealed a tumor composed of polygonal cells associated with a rich vascular network (Figure 3A and B). The tumor cells were
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positive for inhibin but negative for cytokeratin on immunohistochemistry (see Figure 3C). These pathologic features were diagnostic of a hemangioblastoma. Postoperative magnetic resonance imaging demonstrated complete resection of the lesion (see Figure 1). After surgery, the patient exhibited new-onset vertical nystagmus, which resolved by his 2-week follow-up visit in the clinic. His neurologic examination was otherwise intact. His postoperative course was notable for a deep vein thrombosis managed successfully with subcutaneous enoxaparin
In this case, we report the occurrence of a growing hemangioblastoma completely contained within the cerebral aqueduct. The patient underwent successful surgery and demonstrated no new long-term neurologic complications by the time of his 2-year follow-up visit. Tumors contained entirely within this part of the ventricular system are rare. Table 1 summarizes the case information of other occurrences of aqueductal tumors as reported in the literature. To our knowledge, this is the first reported case of an aqueductal hemangioblastoma. Of the 39 previously reported cases of aqueductal tumors, 21/39 (53.8%) were either low-grade or high-grade gliomas, 5/ 39 (12.8%) were angiomas or cavernomas, 3/39 (7.7%) were pilocytic astrocytomas or spongioblastomas, 3/39 (7.7%) were ependymomas, 2/39 (5.1%) were subependymomas, and the remaining tumors were single cases of astroblastoma, subependymal astrocytoma, oligodendroglioma, glioneural tumor, and arteriovenous malformation.13,14,17,18 Notably, this list includes a case series that defied any tumor with an epicenter in the aqueduct as aqueductal in origin.14 A majority of these patients (22/39, 56.4%) were male, and the remainder were female (17/39, 43.6%). The age range of patients presenting with aqueductal tumors spans 5.5 months to 68 years. These data suggest that aqueductal tumors are rare, heterogeneous entities that present with similar frequency in men and women and occur in pediatric, adult, and geriatric patients. The aqueduct itself is the very definition of a dark and dangerous hole, as there are few well-characterized methods for accessing a tumor located within this space. In addition, the surrounding walls of the aqueduct are unforgiving.15,19 Although dividing the tectum is not a benign point of intervention,20 we felt it would be less dangerous than placing the reticular activating system at risk via a tegmental
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.03.206
CASE REPORT ROBERT G. BRIGGS ET AL.
HEMANGIOBLASTOMA OF THE AQUEDUCT
Figure 2. (A and B) Demonstration of the sitting position used during surgery. The Mayfield is pinned above the superior temporal line bilaterally with the neck in a slightly flexed position.
approach.21 The intercollicular approach also allowed access to the CSF space above and below the tumor, which having confirmed the diagnosis as a hemangioblastoma, was best removed en bloc. In retrospect, the thought of attempting to achieve adequate hemostasis at the apex of a difficult, long and narrow
(B) A marking pen has been used to plan the initial incision for a suboccipital craniotomy, shown in (C).
pathway, such as through the fourth ventricle, is one we are glad we avoided. Although the sitting approach was initially popularized as a route to the pineal region,22,23 growing literature describes its use in accessing other regions of the quadrigeminal space.24-26 One particularly helpful aspect of the sitting,
Figure 3. Following en bloc removal of the lesion, histopathology revealed a tumor composed of polygonal cells associated with a rich vascular network (A and B). The tumor cells were positive
WORLD NEUROSURGERY 127: 155-159, JULY 2019
supracerebellar approach is its ability to allow the cerebellar culmen to descend, thereby allowing access to the tectal plate and cerebellomesoencephalic fissure. In our case, this approach allowed us to reach below the tumor and resect it in 1 piece. This raises the important point that, although it is always better to avoid
for inhibin but negative for cytokeratin on immunohistochemistry (C), features consistent with a diagnosis of hemangioblastoma.
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HEMANGIOBLASTOMA OF THE AQUEDUCT
Table 1. Summary of the Literature Describing Cases of Aqueductal Tumors Year Reported
Reference
Age (years)
Gender
Type of Lesion
Survival (months)
1917
18
5 1/2 months
M
Glioma
3
1923
18
9
M
Glioma
1
1930
18
10
F
Astroblastoma
36
1930
18
8
M
Spongioblastoma
17
1931
18
9
M
Fibrillary astrocytoma
3
1933
18
12
M
Astrocytoma
10
1934
18
16
F
Fibrillary astrocytoma
24
1936
18
14
M
Spongioblastoma (pilocytic astrocytoma)
9
Venous angioma
12
1937
18
58
F
1945
18
18
F
Hemangioma
24
1945
18
51
M
Hemangioma
0*
1945
18
29
M
Subependymal astrocytoma
0.7
1952
18
27
F
Ependymoma
48
1954
18
10
M
Ependymoma
24
1958
18
7 months
M
Vascular malformation
6
1961
18
53
F
Cavernous angioma
48
1975
13
17
M
Low-grade astrocytoma
0*
1977
13
17
M
Low-grade astrocytoma
0*
1978
18
56
M
Subependymoma
—
1979
18
29
F
Oligodendroglioma
72
1982
18
16
M
Pilocytic astrocytoma
24
1982
18
68
M
Subependymoma
60
1999e2015
14y
11
9 F, 7 M
1 Ependymoma
>28
33
1 Glioneuronal tumor
>28
31
1 Glioblastoma multiforme
>42
5 1/2 months to 57 years
13 Low-grade glioma
>17.5e140
2017
17
62
F
Cavernous angioma
>12
2017
Present case
65
M
Hemangioblastoma
>24
M, male; F, female. *Diagnosis postmortem. yThis report defined all tumors with epicenters in the aqueduct to be aqueductal.
cutting into the brainstem, in some cases, it may be worth the risk to achieve safe resection of a tumor in a narrow space. It is well-known that radiosurgery for hemangioblastoma can be effective.27 However, surgical intervention in this case was chosen in large part because we did not know the diagnosis, and we were
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concerned that radiosurgery to the brainstem would be harmful, especially given the location of the lesion within the midbrain. In our view, there are 3 principle indications to perform brain tumor surgery when the lesion is otherwise difficult to access: 1) cases that may benefit from tumor cytoreduction
based on histopathology, 2) cases where the diagnosis is not known but the differential includes diseases that have different management strategies, and 3) cases where the diagnosis is known but the tumor is known to respond poorly to radiosurgery or radiotherapy. Of course, surgery should only be considered by experienced operators when a safe path into the tumor can be identified to prevent damaging intact neurologic structures. CONCLUSIONS Hemangioblastomas within the brainstem and tumors within the cerebral aqueduct are both uncommon. Here, we report the first case of a hemangioblastoma originating within this CSF space. Using a sitting, supracerebellar, intracollicular approach proved effective for en bloc resection of the tumor. REFERENCES 1. Zhou LF, Du G, Mao Y, Zhang R. Diagnosis and surgical treatment of brainstem hemangioblastomas. Surg Neurol. 2005;63:307-315. 2. Ma D, Wang Y, Du G, Zhou L. Neurosurgical management of brainstem hemangioblastomas: a single-institution experience with 116 patients. World Neurosurg. 2015;84:1030-1038. 3. Yin L, Zhang L, Hao S, Zhang J, Wu Z. Medullary hemangioblastoma: 34 patients at a single institution. J Clin Neurosci. 2014;21:250-255. 4. Weil RJ, Lonser RR, DeVroom HL, Wanebo JE, Oldfield EH. Surgical management of brainstem hemangioblastomas in patients with von HippelLindau disease. J Neurosurg. 2003;98:95-105. 5. Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfield EH. Surgical management of cerebellar hemangioblastomas in patients with von HippelLindau disease. J Neurosurg. 2008;108:210-222. 6. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg. 1989;70:24-30. 7. 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. 2010; 152:287-292. 8. Wind JJ, Bakhtian KD, Sweet JA, et al. Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2011;114:1312-1318. 9. Kanno H, Kondo K, Ito S, et al. Somatic mutations of the von Hippel-Lindau tumor suppressor
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CASE REPORT ROBERT G. BRIGGS ET AL.
HEMANGIOBLASTOMA OF THE AQUEDUCT
gene in sporadic central nervous system hemangioblastomas. Cancer Res. 1994;54:4845-4847.
18. Ho KL. Tumors of the cerebral aqueduct. Cancer. 1982;49:154-162.
10. Shankar GM, Taylor-Weiner A, Lelic N, et al. Sporadic hemangioblastomas are characterized by cryptic VHL inactivation. Acta Neuropathol Communic. 2014;2:167.
19. Mai JC, Ramanathan D, Kim LJ, Sekhar LN. Surgical resection of cavernous malformations of the brainstem: evolution of a minimally invasive technique. World Neurosurg. 2013;79:691-703.
11. Chen LF, Yang Y, Yu XG, Bu B, Xu BN, Zhou DB. Operative management of brainstem hemangioblastomas. J Clin Neurosci. 2013;20:1727-1733.
20. Kaku Y, Yonekawa Y, Taub E. Transcollicular approach to intrinsic tectal lesions. Neurosurgery. 1999;44:338-343 [discussion: 343-344].
12. Tamura R, Miwa T, Ohira T, Yoshida K. Diagnosis and treatment for pure aqueductal tumor. J Clin Neurosci. 2017;44:260-263.
21. Jang SH, Yeo SS. Injury of the lower ascending reticular activating system in patients with pontine hemorrhage: diffusion tensor imaging study. Medicine. 2016;95:e5527.
13. Sanford RA, Bebin J, Smith RW. Pencil gliomas of the aqueduct of Sylvius. Report of two cases. J Neurosurg. 1982;57:690-696. 14. Roth J, Chaichana KL, Jallo G, Mirone G, Cinalli G, Constantini S. True aqueductal tumors: a unique entity. Acta Neurochir (Wien). 2015;157: 169-177. 15. Cavalheiro S, Yagmurlu K, da Costa MD, et al. Surgical approaches for brainstem tumors in pediatric patients. Childs Nerv Syst. 2015;31:1815-1840. 16. Schroeder HW. Intraventricular tumors. World Neurosurg. 2013;79(suppl 2). S17.e15-S17.e19. 17. Feletti A, Dimitriadis S, Pavesi G. Cavernous angioma of the cerebral aqueduct. World Neurosurg. 2017;98, 876.e815-876.e822.
22. Choudhri O, Chang SD. Supracerebellar infratentorial sitting craniotomy for a pinealoblastoma. Neurosurg Focus. 2016;40(video suppl 1), 2016.2011.FocusVid.15417. 23. Nazzaro JM, Shults WT, Neuwelt EA. Neuroophthalmological function of patients with pineal region tumors approached transtentorially in the semisitting position. J Neurosurg. 1992;76:746-751. 24. Howng SL, Chang JW. Cerebellar lipoma: report of a case. Kaohsiung J Med Sci. 1991;7:609-613. 25. Laborde G, Gilsbach JM, Harders A, Seeger W. Experience with the infratentorial supracerebellar approach in lesions of the quadrigeminal region, posterior third ventricle, culmen cerebelli, and
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cerebellar peduncle. Acta Neurochir (Wien). 1992; 114:135-138.
26. Sanli AM, Turkoglu E, Kertmen H, Gurer B. Hydatid cyst of the ambient cistern radiologically mimicking an arachnoid cyst. J Neurosurg Pediatr. 2012;10:186-188.
27. Liebenow B, Tatter A, Dezarn WA, Isom S, Chan MD, Tatter SB. Gamma Knife stereotactic radiosurgery favorably changes the clinical course of hemangioblastoma growth in von HippelLindau and sporadic patients [e-pub ahead of print]. J Neuro-oncol https://doi.org/10.1007/s11060019-03118-x. Accessed February 7, 2019.
Conflict of interest statement: We confirm that there are no known conflicts of interest associated with this publication and there has been no significant financial support for this work that could have influenced its outcome. Received 20 February 2019; accepted 20 March 2019 Citation: World Neurosurg. (2019) 127:155-159. https://doi.org/10.1016/j.wneu.2019.03.206 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
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Hemangioblastoma of Cerebral Aqueduct Removed via Sitting, Supracerebellar Intracollicular Approach Robert G. Briggs1, Ryan G. Jones1, Andrew K. Conner1, Parker G. Allan1, Hannah B. Homburg1, B. David Maxwell1, Kar-Ming Fung2, Michael E. Sughrue3
Key words Cerebral aqueduct - Colliculus - Hemangioblastoma -
Abbreviations and Acronyms CSF: Cerebrospinal fluid VHL: Von Hippel-Lindau disease From the Departments of 1Neurosurgery and 2Pathology, University of Oklahoma Health Science Center, Oklahoma City, Oklahoma, USA; and 3Center for Minimally Invasive Neurosurgery, Prince of Wales Private Hospital, Sydney, Australia To whom correspondence should be addressed: Michael E. Sughrue, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2019) 127:155-159. https://doi.org/10.1016/j.wneu.2019.03.206 Journal homepage: www.journals.elsevier.com/worldneurosurgery
- BACKGROUND:
Tumors protruding into the cerebral aqueduct are rare, and tumors arising from within the cerebral aqueduct are rarer still.
- CASE
DESCRIPTION: In this report, we discuss the presentation and clinical outcome of a 65-year-old man who presented to us with symptoms of hydrocephalus. Prior imaging had revealed a small enhancing nodule within the cerebral aqueduct. In the 6 months between initial imaging and our seeing the patient, the tumor demonstrated substantial interval growth, so the patient was offered resection. The tumor was accessed using a sitting, supracerebellar, intracollicular approach, which allowed for gross total resection of the mass without complication. Histopathology later revealed the lesion to be a hemangioblastoma. Two years after surgery, the patient was doing well with no neurologic deficits.
- CONCLUSIONS:
We report the first case of an aqueductal hemangioblastoma and describe our use of a sitting, supracerebellar, intracollicular approach to access tumors occupying this cerebrospinal fluid space.
Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
INTRODUCTION Hemangioblastomas are benign, highly vascularized tumors that typically grow in the posterior fossa, especially the cerebellum.1 However, about 2%15% of hemangioblastomas grow in the brainstem.2-4 They can occur sporadically or concurrently with von Hippel-Lindau disease (VHL). Sporadic hemangioblastomas tend to be solitary and occur in patients 40 years and older, while VHLrelated hemangioblastomas are often smaller, tend to appear in multiples, and occur in patients 10 to 15 years younger.3,5-8 Both variants of hemangioblastoma are associated with mutations in the VHL gene.9,10 Hemangioblastomas are not well associated with a particular set of symptoms, though headache and dizziness are common.1 After diagnosis, these tumors may be closely monitored or removed,2 though some recommend removal of all symptomatic hemangioblastomas.3,4 Removal en bloc is strongly recommended.2,11
It is rare that any tumors protrude into the cerebral aqueduct, and it is extremely rare that they arise from within it.12 Occlusion of the cerebral aqueduct caused by the tumor can lead to aqueductal stenosis and noncommunicating hydrocephalus.12-14 This low incidence is fortunate as aqueductal tumors can pose substantial challenges. First, they are surrounded entirely by the midbrain, so the risk of damage to a vital neurologic structure is high.15 In addition, although the aqueduct is a cerebrospinal fluid (CSF) space, the long axis of this space requires an approach through either the lateral and third ventricles from above or the fourth ventricle from below. Larger tumors that have displaced normal brain parenchyma provide some access to this deep space, but smaller tumors pose a challenge to neurosurgeons. The surgeon can attempt a complex and difficult resection at presentation16 or wait for the tumor to grow, thereby displacing the brain, opening the aqueduct and possibly simplifying the procedure. In this study, we report the first case of a hemangioblastoma that arose
WORLD NEUROSURGERY 127: 155-159, JULY 2019
entirely within the cerebral aqueduct. We used a sitting, supracerebellar, intracollicular approach to gain access to a growing lesion in the cerebral aqueduct later identified as a hemangioblastoma. Here, we also review the literature related to lesions originating in the aqueduct. CASE REPORT The patient was a 65-year-old man who initially presented with hydrocephalus that was treated with an endoscopic third ventriculostomy at an outside hospital. Imaging at that time demonstrated a small enhancing nodule within the cerebral aqueduct, which was simply observed. The lesion demonstrated interval growth over a 6-month period before the patient was referred to us (Figure 1). After detailed review of the imaging, we determined that the best approach to access this tumor was via a trajectory between the colliculi. The patient was taken to the operating room, where a suboccipital craniotomy was performed in the sitting position (Figure 2).
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CASE REPORT ROBERT G. BRIGGS ET AL.
HEMANGIOBLASTOMA OF THE AQUEDUCT
injections. The patient ultimately required a ventriculoperitoneal shunt to manage his hydrocephalus. At the 2-year follow-up, the tumor had not recurred, and the patient was fully functional with no new neurologic deficits. DISCUSSION
Figure 1. Preoperative T1-weighted magnetic resonance imaging (A) before and (B) after administration of gadolinium reveals a contrast-enhancing lesion within the cerebral aqueduct (white arrow). Note the dilated temporal horns indicating hydrocephalus. (C) Axial and (D) sagittal postoperative imaging demonstrates complete resection of the lesion (blue arrow). A trajectory through the colliculi was used to remove the lesion en bloc.
A supracerebellar approach was used to reach the tectal plate and split the cerebellomesoencephalic fissure. We made a small incision through the middle of the colliculi using a CO2 laser to reach above and below the tumor’s superior and inferior poles. We identified the tumor, which was clearly vascular in origin, and used a Rhoton #2 dissector to free the mass and remove it en bloc from the aqueduct. Histopathology revealed a tumor composed of polygonal cells associated with a rich vascular network (Figure 3A and B). The tumor cells were
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positive for inhibin but negative for cytokeratin on immunohistochemistry (see Figure 3C). These pathologic features were diagnostic of a hemangioblastoma. Postoperative magnetic resonance imaging demonstrated complete resection of the lesion (see Figure 1). After surgery, the patient exhibited new-onset vertical nystagmus, which resolved by his 2-week follow-up visit in the clinic. His neurologic examination was otherwise intact. His postoperative course was notable for a deep vein thrombosis managed successfully with subcutaneous enoxaparin
In this case, we report the occurrence of a growing hemangioblastoma completely contained within the cerebral aqueduct. The patient underwent successful surgery and demonstrated no new long-term neurologic complications by the time of his 2-year follow-up visit. Tumors contained entirely within this part of the ventricular system are rare. Table 1 summarizes the case information of other occurrences of aqueductal tumors as reported in the literature. To our knowledge, this is the first reported case of an aqueductal hemangioblastoma. Of the 39 previously reported cases of aqueductal tumors, 21/39 (53.8%) were either low-grade or high-grade gliomas, 5/ 39 (12.8%) were angiomas or cavernomas, 3/39 (7.7%) were pilocytic astrocytomas or spongioblastomas, 3/39 (7.7%) were ependymomas, 2/39 (5.1%) were subependymomas, and the remaining tumors were single cases of astroblastoma, subependymal astrocytoma, oligodendroglioma, glioneural tumor, and arteriovenous malformation.13,14,17,18 Notably, this list includes a case series that defied any tumor with an epicenter in the aqueduct as aqueductal in origin.14 A majority of these patients (22/39, 56.4%) were male, and the remainder were female (17/39, 43.6%). The age range of patients presenting with aqueductal tumors spans 5.5 months to 68 years. These data suggest that aqueductal tumors are rare, heterogeneous entities that present with similar frequency in men and women and occur in pediatric, adult, and geriatric patients. The aqueduct itself is the very definition of a dark and dangerous hole, as there are few well-characterized methods for accessing a tumor located within this space. In addition, the surrounding walls of the aqueduct are unforgiving.15,19 Although dividing the tectum is not a benign point of intervention,20 we felt it would be less dangerous than placing the reticular activating system at risk via a tegmental
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.03.206
CASE REPORT ROBERT G. BRIGGS ET AL.
HEMANGIOBLASTOMA OF THE AQUEDUCT
Figure 2. (A and B) Demonstration of the sitting position used during surgery. The Mayfield is pinned above the superior temporal line bilaterally with the neck in a slightly flexed position.
approach.21 The intercollicular approach also allowed access to the CSF space above and below the tumor, which having confirmed the diagnosis as a hemangioblastoma, was best removed en bloc. In retrospect, the thought of attempting to achieve adequate hemostasis at the apex of a difficult, long and narrow
(B) A marking pen has been used to plan the initial incision for a suboccipital craniotomy, shown in (C).
pathway, such as through the fourth ventricle, is one we are glad we avoided. Although the sitting approach was initially popularized as a route to the pineal region,22,23 growing literature describes its use in accessing other regions of the quadrigeminal space.24-26 One particularly helpful aspect of the sitting,
Figure 3. Following en bloc removal of the lesion, histopathology revealed a tumor composed of polygonal cells associated with a rich vascular network (A and B). The tumor cells were positive
WORLD NEUROSURGERY 127: 155-159, JULY 2019
supracerebellar approach is its ability to allow the cerebellar culmen to descend, thereby allowing access to the tectal plate and cerebellomesoencephalic fissure. In our case, this approach allowed us to reach below the tumor and resect it in 1 piece. This raises the important point that, although it is always better to avoid
for inhibin but negative for cytokeratin on immunohistochemistry (C), features consistent with a diagnosis of hemangioblastoma.
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HEMANGIOBLASTOMA OF THE AQUEDUCT
Table 1. Summary of the Literature Describing Cases of Aqueductal Tumors Year Reported
Reference
Age (years)
Gender
Type of Lesion
Survival (months)
1917
18
5 1/2 months
M
Glioma
3
1923
18
9
M
Glioma
1
1930
18
10
F
Astroblastoma
36
1930
18
8
M
Spongioblastoma
17
1931
18
9
M
Fibrillary astrocytoma
3
1933
18
12
M
Astrocytoma
10
1934
18
16
F
Fibrillary astrocytoma
24
1936
18
14
M
Spongioblastoma (pilocytic astrocytoma)
9
Venous angioma
12
1937
18
58
F
1945
18
18
F
Hemangioma
24
1945
18
51
M
Hemangioma
0*
1945
18
29
M
Subependymal astrocytoma
0.7
1952
18
27
F
Ependymoma
48
1954
18
10
M
Ependymoma
24
1958
18
7 months
M
Vascular malformation
6
1961
18
53
F
Cavernous angioma
48
1975
13
17
M
Low-grade astrocytoma
0*
1977
13
17
M
Low-grade astrocytoma
0*
1978
18
56
M
Subependymoma
—
1979
18
29
F
Oligodendroglioma
72
1982
18
16
M
Pilocytic astrocytoma
24
1982
18
68
M
Subependymoma
60
1999e2015
14y
11
9 F, 7 M
1 Ependymoma
>28
33
1 Glioneuronal tumor
>28
31
1 Glioblastoma multiforme
>42
5 1/2 months to 57 years
13 Low-grade glioma
>17.5e140
2017
17
62
F
Cavernous angioma
>12
2017
Present case
65
M
Hemangioblastoma
>24
M, male; F, female. *Diagnosis postmortem. yThis report defined all tumors with epicenters in the aqueduct to be aqueductal.
cutting into the brainstem, in some cases, it may be worth the risk to achieve safe resection of a tumor in a narrow space. It is well-known that radiosurgery for hemangioblastoma can be effective.27 However, surgical intervention in this case was chosen in large part because we did not know the diagnosis, and we were
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concerned that radiosurgery to the brainstem would be harmful, especially given the location of the lesion within the midbrain. In our view, there are 3 principle indications to perform brain tumor surgery when the lesion is otherwise difficult to access: 1) cases that may benefit from tumor cytoreduction
based on histopathology, 2) cases where the diagnosis is not known but the differential includes diseases that have different management strategies, and 3) cases where the diagnosis is known but the tumor is known to respond poorly to radiosurgery or radiotherapy. Of course, surgery should only be considered by experienced operators when a safe path into the tumor can be identified to prevent damaging intact neurologic structures. CONCLUSIONS Hemangioblastomas within the brainstem and tumors within the cerebral aqueduct are both uncommon. Here, we report the first case of a hemangioblastoma originating within this CSF space. Using a sitting, supracerebellar, intracollicular approach proved effective for en bloc resection of the tumor. REFERENCES 1. Zhou LF, Du G, Mao Y, Zhang R. Diagnosis and surgical treatment of brainstem hemangioblastomas. Surg Neurol. 2005;63:307-315. 2. Ma D, Wang Y, Du G, Zhou L. Neurosurgical management of brainstem hemangioblastomas: a single-institution experience with 116 patients. World Neurosurg. 2015;84:1030-1038. 3. Yin L, Zhang L, Hao S, Zhang J, Wu Z. Medullary hemangioblastoma: 34 patients at a single institution. J Clin Neurosci. 2014;21:250-255. 4. Weil RJ, Lonser RR, DeVroom HL, Wanebo JE, Oldfield EH. Surgical management of brainstem hemangioblastomas in patients with von HippelLindau disease. J Neurosurg. 2003;98:95-105. 5. Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfield EH. Surgical management of cerebellar hemangioblastomas in patients with von HippelLindau disease. J Neurosurg. 2008;108:210-222. 6. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg. 1989;70:24-30. 7. 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. 2010; 152:287-292. 8. Wind JJ, Bakhtian KD, Sweet JA, et al. Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2011;114:1312-1318. 9. Kanno H, Kondo K, Ito S, et al. Somatic mutations of the von Hippel-Lindau tumor suppressor
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Conflict of interest statement: We confirm that there are no known conflicts of interest associated with this publication and there has been no significant financial support for this work that could have influenced its outcome. Received 20 February 2019; accepted 20 March 2019 Citation: World Neurosurg. (2019) 127:155-159. https://doi.org/10.1016/j.wneu.2019.03.206 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
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