Recurrent Skull Base Chordomas: Role of Surgery




Abstract


Skull base chordomas have a notorious tendency to recur even after wide radical removal of the soft tissue and osseous tumor and extensive drilling removal of surrounding bone. Their high recurrence rates warrant adjuvant high-dose radiation therapy. This combination of radical removal and high-dose radiotherapy delays the time of recurrence, but in most cases, the tumor eventually recurs locally. These recurrent cases present quite a challenge, particularly in the reconstruction intended to prevent CSF leak. Scar and radiation-induced angiopathy challenge tumor dissection and increase the risk of vascular injury, and tissue devitalized by prior surgery and radiation is less useful for closure and prevention of CSF fistulas. These considerations favor an approach for reoperation that differs from the original. Multiple and staged reoperations require thorough planning that considers potential extent of resection, possible complications, particularly poor wound healing and CSF leak, reconstruction, including donor sites of autologous tissue, and the patient’s health and likely recovery time. As a rule, the surgical treatment of recurrent chordomas should aim to achieve complete tumor removal. This requires the mastery and use of multiple skull base approaches and facility with all tools of the skull base surgical armamentarium, including intraoperative imaging and/or navigation.




Keywords

Chordoma, Clivus, Recurrent skull base tumors, Surgical approaches, Tumor excision

 






  • Outline



  • Introduction 393



  • Diagnosis of Recurrent Tumor 393



  • Decision-Making 395



  • Morbidity and Mortality of Reoperation 397



  • Conclusions 398



  • References 398


© 2018 Elsevier Inc. All rights reserved. Please note that the copyright for the original figures submitted by the contributors is owned by Contributors.




Introduction


Chordomas are unusual malignant dysontogenic neoplasms with a low incidence (less than 0.03 per 100,0000 person) and unpredictable growth rate. Chordomas are invasive and locally destructive. Their pathological diagnosis can only be confirmed by immunohistochemical stains. Midline origin from rather thin bone and bilateral extension through the base of the skull usually precludes en bloc oncological removal of clival chordomas. Even so, the role of radical resection is well established and is often undertaken. Even after wide radical removal of the soft tissue and osseous tumor and extensive drilling removal of surrounding bone skull base, chordomas have a notorious tendency to recur. Their high recurrence rates after surgery alone warrant adjuvant high-dose radiation therapy. This combination of radical removal and high-dose radiotherapy delays the time of recurrence, but in most cases, the tumor eventually recurs locally. These recurrent cases present quite a challenge, particularly in the reconstruction intended to prevent CSF leak. Scar and radiation-induced angiopathy challenge tumor dissection and increase the risk of vascular injury, and tissue devitalized by prior surgery and radiation is less useful for closure and prevention of CSF fistulas. These considerations favor an approach for reoperation that differs from the original. In this chapter we address the role of reoperation in the management of recurrent skull base chordomas ( Figs. 37.1–37.4 ).




Figure 37.1


A 40-year-old woman presented with diplopia (left VI nerve paresis). The preoperative magnetic resonance imaging (MRI) showed a tumor of the midclivus (A, preop sagittal; B, preop axial). The patient underwent an extended transsphenoidal approach. Postoperative MRI (C, postop sagittal; D, postop axial) confirmed radical removal of the mass.

Used with permission from Prof. L.A.B. Borba academic collection.



Figure 37.2


The patient received radiation therapy, but 2 years later, a new MRI depicted a recurrent tumor in the left petroclival junction, petrous apex, and cavernous sinus (E, preop axial; F, preop sagittal).

Used with permission from Prof. L.A.B. Borba academic collection.



Figure 37.3


She underwent a left middle fossa approach with radical resection of the mass, as depicted by the postoperative MRI (G, postop axial).

Used with permission from Prof. L.A.B. Borba academic collection.



Figure 37.4


Fifteen months later, follow-up MRI depicted a recurrent tumor in the right petroclival junction, petrous apex, and cavernous sinus (H, preop axial; I, preop sagittal; J, preop coronal).

Used with permission from Prof. L.A.B. Borba academic collection.




Diagnosis of Recurrent Tumor


Chordoma recurrence presents clinically and radiographically. Postoperative follow-up must be regular and unlimited in duration. The extent of resection should be evaluated by CT and MR imaging within 48 h of the initial operation and then 3 and 6 months later. Immediate postoperative scanning is mandatory to assess the extent of surgical removal, to guide postoperative radiotherapy, and to provide a baseline for comparison with subsequent studies. Tumor recurrence, radiation tissue injury, infection, and craniospinal instability can cause new signs and symptoms. The surgical route should be carefully scrutinized for seeding of tumor. New symptoms without radiologic evidence of tumor presence are warning signs that should be closely followed. Despite the high resolution of MRI, recurrent tumor can be misinterpreted as surgical scar or radiation effects. CT is often valuable in revealing new invasion and erosion of the surrounding bone ( Figs. 37.5–37.7 ).




Figure 37.5


The patient underwent a right cranioorbitozygomatic approach, with radical removal of the mass, without postoperative neurological complications, as depicted by the MRI (K, postop sagittal; L, postop coronal).

Used with permission from Prof. L.A.B. Borba academic collection.

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Feb 21, 2019 | Posted by in NEUROSURGERY | Comments Off on Recurrent Skull Base Chordomas: Role of Surgery
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