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VIDEO SECTION.OPERATIVE NUANCES: STEP BY STEP
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1200-1203

En-bloc Resection of A Giant Solid Hemangioblastoma of The Vermis


1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India

Date of Submission18-Oct-2021
Date of Acceptance18-Oct-2021
Date of Web Publication30-Oct-2021

Correspondence Address:
Poodipedi Sarat Chandra
Neurosurgery, PI and Team leader COE and MEG Centre, Core Faculty Epilepsy and Functional Neurosurgery Division, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.329537

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 » Abstract 


Introduction: Hemangioblastomas (HMB) are extensively vascularized and benign neoplasms that are diagnosed predominantly in adults. The solid type of HMB is technically challenging to operate. The piecemeal resection of the tumor has been shown to have devastating intraoperative complications. Therefore, en-bloc tumor resection was shown to be the safest procedure to reduce the risk of intraoperative bleeding and facilitate the removal of large solid tumors. Unfortunately, most of these tumours are also not amenable for embolization, as they are fed by multiple pial vessels. However, the large arterial feeders may be embolized.
Objective: This video abstract presents a case of en-bloc resection of a giant solid type of Vermian HMB.
Surgical Technique: A 38-year-old male patient presented with headache and vomiting for 1 year, imbalance during walking for 6 months, and hoarseness of voice for 2 months. He underwent a CSF diversion procedure in another hospital and was referred to our center for definitive surgery. Contrast-enhanced MRI showed a large heterogeneously enhancing solid lesion of size 46 × 33 × 40 mm3 with central necrotic area in the posterior fossa with perilesional edema and several large flow voids on T2-MR sequence. Preoperative embolization was performed; however, there was no significant reduction in the vascularity of the lesion. The patient underwent a wide midline suboccipital craniotomy with C1 laminectomy and gross total en-bloc resection of HMB.
Results: The patient had an uneventful recovery in the postoperative period.
Conclusions: The safest approach in the resection of giant solid HMB involves proper preoperative planning and understanding the vascular pattern of the lesion, wide exposure, circumferential dissection, and the en-bloc delivery of the tumor.


Keywords: En-bloc excision, giant hemangioblastoma, solid hemangioblastoma, suboccipital craniotomy
Key Message: Surgical approach of a giant solid variant of posterior fossa HMB requires meticulous preoperative planning and understanding the vascular pattern of the tumor. En-bloc resection of the giant solid HMB can be achieved following appropriate microneurosurgical principles.


How to cite this article:
Goda R, Dey S, Doddamani RS, Gaikwad S, Chandra PS. En-bloc Resection of A Giant Solid Hemangioblastoma of The Vermis. Neurol India 2021;69:1200-3

How to cite this URL:
Goda R, Dey S, Doddamani RS, Gaikwad S, Chandra PS. En-bloc Resection of A Giant Solid Hemangioblastoma of The Vermis. Neurol India [serial online] 2021 [cited 2021 Nov 30];69:1200-3. Available from: https://www.neurologyindia.com/text.asp?2021/69/5/1200/329537


Surgical management of giant hemangioblastomas (HMB) is technically challenging. Most HMB have cystic or solid-cystic forms (70%–75%), which predominantly occur in the cerebellar hemispheres.[1] In contrast, the solid variants are frequently present in the brainstem, cerebellar vermis, and spinal cord. The characteristics of solid tumors are similar to intracranial arteriovenous malformations.[2],[3] The piecemeal resection and internal decompression of the giant tumors are shown to have devastating intraoperative complications.[4] Thus, the surgical principle of giant solid tumors requires proper planning, preoperative embolization, generous exposure, circumferential dissection, and en-bloc resection following microneurosurgical principles. The following article demonstrates the surgical technique of en-bloc resection of a giant solid variant of HMB from the cerebellar vermis.

[Figure 1], [Figure 2], [Figure 3]
Figure 1: (a) Axial Non-contrast CT head showing hyperdense lesion with central hypodense area in the posterior fossa without calcification, (b) T2-weighted MR image showing the hypo- to isointense lesion with large flow voids in the lesion, and (c) the lesion is avidly enhancing on contrast. (d) ASL sequence showing the increased perfusion in the lesion

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Figure 2: Diagnostic DSA showing tumoral blush in the posterior fossa with multiple feeders from bilateral SCA, Left AICA, Left PICA and meningeal branches of PICA

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Figure 3: (a) The patient is positioned prone on Mayfield pin holder and Incision marked from inion to C3 level. (b) After craniotomy, the dura was opened in Y-shaped manner and (c and d) A large dilated arterialized draining vein was identified on the tumor surface on the right lateral aspect which was coagulated and divided

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 » Objective Top


The video in this article demonstrates the en-bloc excision of a solid variant of giant HMB by using a midline suboccipital craniotomy along with C1 laminectomy.

Case study

A 38-year-old male patient with no comorbidities presented with complaints of headache and vomiting for one year, imbalance during walking for 6 months, and dysphagia and hoarseness of voice for 2 months. He underwent a CSF diversion procedure in another hospital in view of obstructive hydrocephalus and was referred to our center for definitive surgery. Examination revealed left-sided cerebellar dysfunction, impaired cough and gag on both sides, tongue atrophy on the left side, without sensory-motor deficits. He was evaluated with a contrast-enhanced MRI, which showed a large heterogeneously enhancing solid lesion of size 46 × 33 × 40 mm3 with a central necrotic area in the posterior fossa with perilesional edema and multiple flow voids on T2-MR sequence. The patient also underwent contrast-enhanced computed tomography of chest and abdomen to rule out the presence of intraabdominal malignancy.

In view of the solid variant of giant HMB with extensive vascularity, preoperative embolization was performed; however, there was no significant reduction in the vascularity of the lesion. The patient underwent en-bloc resection within 24 h of embolization. The histopathology confirmed the radiological diagnosis. Postoperative MRI performed at three weeks after surgery showed no residual lesion with a small resolving hematoma.

Surgical plan

With a radiological diagnosis of solid HMB probably arising from the vermis, the patient was counseled and planned for midline suboccipital craniotomy and excision of the tumor.

Procedure

The patient was under general anesthesia, and appropriate neuroprotective measures were taken. A central venous access, an arterial line, and two large-bore peripheral venous lines were secured. Intraoperative neuromonitoring was set up to use motor evoked potentials, somatosensory evoked potentials, and lower cranial nerve monitoring. A cell saver kit was also set up during surgery for autologous transfusion.

The patient was positioned prone, with the neck slightly flexed and head fixed with a Mayfield clamp. A broad, generous midline suboccipital craniotomy was performed, extending superiorly to the transverse sinus and inferiorly to the foramen magnum. The posterior arch of C1 was also removed to tackle the inferior meningeal feeders arising from the vertebral artery. On craniotomy, dura was full and tense. Dura was opened in a Y-shaped manner; the inferior limb extended inferiorly till the lower end of the C1 arch. The two oblique limbs were extended superiorly till the inferior end of the transverse sinus. There were numerous dilated draining venous channels during the dural opening, and the bleeding from these veins was controlled using Liga clips. After durotomy, the brain was full and pulsatile. There was evidence of a large solid mass in the midline splaying the two cerebellar hemispheres with numerous dilated veins over the tumor. The arachnoid membrane over the tumor was dissected and the circumferential dissection around the tumor was done in all directions. There were multiple feeders from bilateral PICA, AICA, and SCA, which were identified and coagulated, and large dilated arterialized veins were coagulated and divided at the end of dissection. After dissection, the tumor was removed en-bloc and hemostasis was achieved. Lax duroplasty was performed with pericranium.

Video link: https://youtu.be/xTt9u17PcR8

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Video timeline with audio transcript

0.06–1.26 min: A 38-year-old male patient presented with headache and vomiting for one year, imbalance during walking for six months, and hoarseness of voice for two months. He underwent a left-sided VPS in view of obstructive HCP at another hospital and was referred to our center for definitive surgery. Contrast-enhanced MRI showed a large heterogeneously enhancing solid lesion of size 4.6cms with central necrotic area in the vermis with perilesional edema and multiple large flow voids on T2-WI sequence. Arterial spin labelling (ASL) revealed a large area of hyper-perfusion suggestive of a highly vascular lesion. Angiogram revealed a highly vascular tumor with arterial feeders from left vertebral, anterior inferior cerebellar, right vertebral. Preoperative embolization performed could only partially reduce the vascularity.

1.26–1.50 min: The patient was positioned prone with the head fixed on a Mayfield head holder with neck flexion. A wide midline suboccipital craniotomy and C1 laminectomy was fashioned.

1.50–2.21 min: Dura was opened in a Y-shaped manner, with the vertical limb inferiorly extending till the superior border of the C2 vertebra and both the oblique limbs extending superiorly and laterally till the inferior border of transverse sinus. During the durotomy, there were multiple dilated draining venous channels, and the bleeding from these veins was controlled using Liga clips. After durotomy, tonsillar herniation was noted inferiorly below the C1 arch.

2.22–2.38 min: There was a large yellowish solid vascular mass present in the midline splaying both the cerebellar hemispheres. The arachnoid dissection was commenced on the superior aspect of the tumor and a small arterial feeder was identified, which was coagulated and cut.

2.38–3.00 min: The circumferential tumor dissection was performed beginning on the left side of the tumor. The tumor surface was coagulated to shrink and facilitate circumferential dissection around the tumor. The large veins were preserved during the initial stage of dissection.

3.03-3.14 min: An arterial feeder from the left PICA was identified, coagulated, and divided on the tumor surface.

3.15-3.35 min: The dissection was further performed around the tumor continuing superiorly on the left side. As seen, the tumor has lost its turgidity and has become supple and pale as a result of circumferential dissection and coagulation of the arterial feeders.

3.35–3.50 min: An arterial feeder from left SCA was identified and coagulated on the tumor surface, and the dissection was continued further toward the superior aspect.

3.50–4.20 min: Similarly, the tumor dissection was performed along the inferior part of the tumor.

4.21–4.32 min: This was followed by dissection along the inferolateral aspect on the right side.

4.32–4.58 min: A large dilated arterialized draining vein was identified on the tumor surface on the right lateral aspect, which was coagulated and divided.

4.58–5.09 min: After performing the circumferential dissection, ventral dissection from the vermis was performed and the arterial feeders on the tumor capsule were coagulated and cut.

5.09–5.41 min: The tumor was delivered en-bloc following complete dissection. In this step, it is not uncommon to encounter bleeding from the remaining tumor remanent, which can be controlled with coagulation and tumor removal.

5.41–6.14 min: The small residual component of the tumor in the inferior aspect of the tumor was removed.

6.14–6.21 min: Following complete resection of the tumor, meticulous hemostasis was achieved with coagulation and layering of FloSeal (a combination of gelatin and human thrombin). Watertight dural closure was achieved using a pericranial patch.

6.21–6.27 min: Bone flap was replaced and fixed using miniplate screws.

6.27–6.33 min: Postoperative CT showed a good operative cavity with minor bleed in the cavity.

6.33–6.45 min: The patient was electively ventilated for 48 h after surgery and was tracheostomized in view of impaired gag and cough reflex in the preoperative period. The patient was decannulated and discharged after 3 weeks.

Outcome

Following surgery, the patient was tracheostomized in view of impaired gag due to lower cranial nerve paresis and hence was started on Ryles tube feeding. Postoperative CT showed a good cavity with small tumor bed hematoma, which did not require any intervention. The patient was decannulated after three weeks once the cough and gag reflex improved gradually. Contrast-enhanced MRI performed after one month of surgery showed no residual tumor with resolving hematoma.

Pearls and pitfalls

  • Preoperative embolization is beneficial as it reduces intraoperative bleeding.
  • A wide craniotomy is a prerequisite to facilitate dissection with minimal retraction.
  • The most critical principle during surgery is to perform circumferential dissection and not violating the tumor capsule.
  • These lesions should be removed in a similar fashion as an arteriovenous malformation; therefore, en-bloc removal should be the goal and piecemeal removal should never be attempted.
  • The division of the main draining veins should be performed during the last step of the surgery because an early division of veins can induce tumor swelling, leading to massive bleeding with disastrous outcome.
  • It is imperative to distinguish the feeding arteries from the draining veins as most of these veins become arterialized. The feeding arteries should be tackled during the early stages of surgery while performing the circumferential dissection around the tumor.
  • It is preferable to operate under high magnification, which allows careful dissection of the tumor vessels and surrounding en-passage vessels.
  • The use of intraoperative microvascular Doppler ultrasonography may be a useful adjunct and has been described by some authors.



 » Discussion Top


HMBs are benign neoplasms that are predominantly noted in adults, and the complete surgical removal is the goal of surgery. Approximately two-thirds of these tumors are cystic with hypervascular mural nodules. A solid variant of HMB is rare and is associated with an increased risk of massive intraoperative bleeding and postoperative complications.[5] Owing to their AVM-like characteristics, giant solid subtypes are more technically challenging.

In our case, selective preoperative embolization of the feeding arteries was done to reduce vascularity, but there was no significant reduction of vascularity of the lesion. The role of embolization remains controversial owing to its associated risks, including bleeding, ischemia, and increased intracranial pressure.[6],[7] The indications of embolization should be decided on an individual basis and should be performed within one day or immediately before surgery by neuro-endovascular experts.[8]

Owing to the improvements in the microsurgical techniques and a proper understanding of the tumor vascular pattern, en-bloc tumor resection can be safely achieved with minimal intraoperative bleeding and postoperative complications.[3],[9] The tumor-feeding arteries are often situated deep anteriorly on both sides of the tumor, whereas the draining veins are observed on the posterior surface of the tumor.[7]


 » Conclusions Top


Solid giant-sized HMBs require preoperative embolization whenever feasible. However, when embolization is least effective, as in the case described here, en-bloc excision using standard microsurgical technique (without violating the tumor capsule) aids in safe removal of these difficult lesions.

Declaration of patient consent

Full and detailed consent from the patient/guardian has been taken. The patient's identity has been adequately anonymized. If anything related to the patient's identity is shown, adequate consent has been taken from the patient/relative/guardian. The journal will not be responsible for any medico-legal issues arising out of issues related to the patient's identity or any other matters arising from the public display of the video.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Singounas EG. Haemangioblastomas of the central nervous system. Acta Neurochir (Wien) 1978;44:107–13.  Back to cited text no. 1
    
2.
Kamitani H, Hirano N, Takigawa H, Yokota M, Miyata H, Ohama E, et al. Attenuation of vascularity by preoperative radiosurgery facilitates total removal of a hypervascular hemangioblastoma at the cerebello-pontine angle: Case report. Surg Neurol 2004;62:238–43; discussion 243-4.  Back to cited text no. 2
    
3.
Cui H, Zou J, Bao Y-H, Wang M-S, Wang Y. Surgical treatment of solid hemangioblastomas of the posterior fossa: A report of 28 cases. Oncol Lett 2017;13:1125–30.  Back to cited text no. 3
    
4.
Roberti F, Jones RV, Wright DC. Cranial nerve hemangioblastomas. Report of a rare case and review of literature. Surg Neurol 2007;67:640–6; discussion 646.  Back to cited text no. 4
    
5.
Nair BR, Joseph V, Chacko G, Keshava SN. Giant solid hemangioblastoma of the cerebellopontine angle: A technically challenging case. Neurol India 2014;62:228–9.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Cornelius JF, Saint-Maurice JP, Bresson D, George B, Houdart E. Hemorrhage after particle embolization of hemangioblastomas: Comparison of outcomes in spinal and cerebellar lesions. J Neurosurg 2007;106:994–8.  Back to cited text no. 6
    
7.
Montano N, Doglietto F, Pedicelli A, Albanese A, Lauretti L, Pallini R, et al. Embolization of hemangioblastomas. J Neurosurg 2008;108:1063–4; author reply 1064-5.  Back to cited text no. 7
    
8.
Biondi A, Ricciardi GK, Faillot T, Capelle L, Van Effenterre R, Chiras J. Hemangioblastomas of the lower spinal region: Report of four cases with preoperative embolization and review of the literature. AJNR Am J Neuroradiol 2005;26:936–45.  Back to cited text no. 8
    
9.
Matsushima T, Kawashima M, Masuoka J, Mineta T, Inoue T. Transcondylar fossa (supracondylar transjugular tubercle) approach: Anatomic basis for the approach, surgical procedures, and surgical experience. Skull Base 2010;20:83–91.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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