Abstract

Background:Chordomas are primary bone tumors derived from the embryonic notochord. They represent 1–4% of all malignant bone tumors. They have a predominantly extra-axial location, arising in the clival region in 35% of reported cases. The prognosis is generally poor, and radical resection remains the first-line treatment. This study aims to describe a case of a clival chordoma that was resected through a combined microscopic transoral and endoscopic endonasal approach, with excellent clinical outcomes.

Case Description:A 24-year-old woman with low cranial nerve symptomatology was admitted for a two-stage surgical approach. An occipital-cervical fixation was performed in the first stage, while a combined endonasaltransoral resection was performed later for tumor resection.

Conclusion:Microscopic transoral and endoscopic endonasal approaches offer advantages for treating clival chordomas, with careful consideration of anatomical constraints and potential for postoperative recurrence being essential in approach selection.

Keywords: Chordoma, Clivus, Endoscopic endonasal approach, Microscopic transoral approach

INTRODUCTION

Chordomas are primary bone tumors derived from the embryonic notochord[ 28 , 31 ] and tend to occur at either end of the spinal axis, with a tendency to occur in the spheno-occipital and sacral regions.[ 1 ] They represent 1–4% of all malignant bone tumors, and incidence has been reported to be 0.5–1/1 million/year, with a prevalence of 1.21/10 million people and only accounting for 0.1–0.2% of all skull base tumors.[ 17 , 21 ] According to the literature, they have a predominantly extra-axial location, arising in the sacrococcygeal (50–55%) or clival region (35%) and vertebrae (10–15%), with rare reports of intra-axial chordomas.[ 8 , 22 , 30 ] Clival chordomas have a 2:1 male/female ratio[ 9 , 11 , 18 ] and usually debut in the fifth decade.[ 16 , 31 ]

Clinically, superior clival lesions may affect the upper cranial nerves, mainly CN VI,[ 19 ] pituitary gland, and hypothalamus, whereas inferior clival tumors may affect the lower cranial nerves initially.[ 27 ]

The prognosis is generally poor, and radical resection remains the first-line treatment. Although the use of adjuvant radiation therapy remains controversial, extensive resection followed by high-dose radiation therapy offers the best chance of achieving long-term tumor control[ 6 , 21 , 24 ] and continues to be the cornerstone of treatment. Five-year survival rates of treated patients range from 50% to 85%.[ 10 ] Local recurrence is the most important prognostic factor for long-term survival after primary resection.[ 5 ]

There are multiple surgical approaches for clival chordoma resection, and classically, the transcranial route has been the preferred method; nevertheless, with the advent of endoscopic techniques, the transoral and transnasal routes are deemed effective for resection, depending on the extension, with the possibility to encompass all the clivus from the posterior clinoids to the craniovertebral junction (CVJ).[ 9 ] Few systematic studies have been conducted to understand the morbidity and efficacy of these approaches for this type of tumor.

Endoscopic endonasal approaches (EEAs) provide better visualization and lower postoperative complication rates than open approaches do, except for cerebrospinal fluid (CSF) leak rates.[ 4 ] Resection rates of up to 100% in middle clivus chordomas and up to 50% in inferior clival lesions have been reported.[ 20 ] However, this approach is limited in lesions around the CVJ due to restriction by the hard palate.[ 14 ]

On the other hand, microscopic transoral approaches (MTAs) are used for midline ventral lesions ranging from the lower third of the clivus to the C2 vertebral body, including a complete view of the vertebrobasilar arterial system and cranial nerves III–XII.[ 13 , 26 , 29 ] Exposure is limited by the degree of mouth opening and size of the patient’s oral cavity.

Both techniques provide direct access to the CVJ. The EEA has the advantage of being a shorter route, while the MTA provides a wider surgical field.[ 23 , 25 ] However, for large tumors extending from the superior clivus to C2, it is important to consider a combined approach to minimize surgical trauma, improve visualization, and achieve gross total resection (GTR).

This study aims to describe the case of a clival chordoma, a brief review of the literature, and a technical note on surgical resection using a combined MTA and EEA.

ILLUSTRATIVE CASE

A 24-year-old woman with no relevant medical history presented as an outpatient with rhinorrhea, foreign body sensation in the oropharynx, dysphagia, cervicalgia, and tinnitus. Medical records revealed a previous transoral biopsy that had already diagnosed the condition as chordoma, necessitating a neurosurgical consultation. Physical examination revealed malnourishment due to dysphagia, nasal obstruction requiring mouth-breathing, and secondary xerosis. No motor or sensory alterations in cranial nerves were found. Magnetic resonance imaging (MRI) and computed tomography (CT)-angiography were performed [ Figure 1 ]. The patient underwent a two-stage scheduled surgical approach. Before surgery, lower cranial nerve deficits were assessed, and involvement of the ninth and tenth cranial nerves was noted. Preoperative tracheostomy and gastrostomy were performed.

Figure 1:

Preoperative CT scan (a: Axial, b: Sagittal, c: Coronal) and T1- weighted contrast-enhanced MRI (d-f: Sagittal and Parasagittal) showing a heterogenous mass with calcifications located in the paravertebral planum, destroying the clivus, anterior portion of C1, C2, and C3.

Operative technique

First stage: Occipitocervical fixation

With the patient in a prone decubitus position and the head fixed in a Mayfield-Kees head holder, a midline suboccipital incision extending to the spinous process of C6 was performed. Dissection was carried out through soft-tissue planes, performing subperiosteal dissection to expose the CVJ, extending down to C6, exposing its lateral masses. An occipital plate was then positioned beneath the projection of the transverse sinus and the confluence of the sinuses. Transpedicular screws were inserted at C3, C5, and C6 (12 mm, 14 mm, and 14 mm, respectively), followed by fixation with 10 cm titanium rods [ Figure 2 ]. Adequate fixation was confirmed using fluoroscopy, along with hemostasis, before reconstructing the surgical approach, performing closure of soft tissues in a standard fashion [ Figure 3 ].

Figure 2:

Suboccipital and cervical approach. (a) Exposure of occipital scale and cervical posterolateral elements. (b) Occipitocervical fixation from C3, C4, and C5.

Figure 3:

Occipitocervical fixation immediate postoperative computed tomography scan sagittal view showing postsurgical occipitocervical fixation and posterior cervical instrumentation from C3 to C5.

Second stage: Combined microscopic transoral and endoscopic endonasal resection

With the patient positioned supine and the head in a neutral position, 3-point skeletal fixation was applied using a Mayfield-Kees head holder. Oxymetazoline-soaked cottonoids were inserted into both nostrils for 5 min. Subsequently, with a 0° rigid endoscope, the right middle and inferior turbinates were laterally displaced. An incision was made at the mucocutaneous junction of the septum, followed by dissection of the cartilaginous portion in the subperichondrial plane, and a posterior septectomy was performed to access the rostral side of the sphenoid rostrum. Right maxillary uncinectomy was performed, and an ipsilateral pedicled nasoseptal flap was harvested. The sphenoid sinus ostia were identified, and the rostrum was removed with the assistance of a 4 mm diamond burr. Once inside the sphenoid sinus, identification of the sellar region and the clival recess was achieved through drilling and blunt dissection. The tumor was located along the most ventral portion of the clival recess. Resection was initiated using bipolar coagulation, ringed curettes, and a conventional aspirator, as no endonasal attachments for the ultrasonic aspirator were available. The approach was widened by reaming the floor of the sella turcica and the clivus down to its inferior portion, where erosion was observed. Subtotal resection was deemed safe and we decided not to violate the dural plane, as by doing this, a high-flow CSF fistula would certainly arise. This could lead to serious postoperative complications, and as only a very small portion of the lesion was located intradurally (<5% of the total volume), no changes in overall survival would have been achieved. Subsequently, the transoral approach was performed using open microsurgical techniques. A mouth retractor was placed, and the uvula with the soft palate was retracted dorsally using 1-0 silk. A scalpel incision was made in the posterior pharyngeal wall at the level of the anterior arch of C1, followed by deepening over soft tissues with monopolar electrocautery until reaching the tumor. Resection continued with the assistance of an ultrasonic aspirator, with no complications. Tumor consistency was heterogeneous, with soft areas alternating with very hard portions that could be hardly devastated, even with the ultrasonic aspirator. On completion of the resection, reconstruction of the approach was performed. At the transoral level, the pharyngeal mucosa was closed with 2-0 Vicryl sutures. At the endonasal level, hemostasis of the surgical bed was ensured with Surgicel fragments, followed by placement of the pedicled nasoseptal flap. Tissue adherents were applied to the surgical edges, and nasal packing was performed with two Foley tubes, inflating the upper balloon with 7 mL and the lower one with 6 mL. The procedure was then concluded [ Video 1 ].

Video 1

Follow-up

There were no postoperative complications and the patient was dispatched after 8 days of hospitalization. After 3 months, the patient presented with clinical and radiological signs of hydrocephalus; thus, a ventriculoperitoneal shunt was placed. At follow-up as an outpatient no additional neurological deficits were found, however, tracheostomy and gastrostomy were left in place due to persistence of lower cranial nerve dysfunction. Postoperative CT-scan and MRI [ Figure 4 ] revealed a residual lesion, for which adjuvant radiotherapy was provided in 30 fractions for a total dose of 72 Gy.

Figure 4:

Resection immediate postoperative (a) Computed Tomography Scan and (b) coronal, (c) axial Magnetic Resonance Imaging (MRI) view of T1-weighted contrast-enhanced MRI showing partial resection of the lesion with two Foley catheter insufflation for fistula prevention.

DISCUSSION

Occipitocervical fixation was first performed due to the involvement of the condyles and craniocervical junction (C1–C3), which posed a high risk of instability after resection. This fixation ensured structural stability and prevented complications. Once stabilized, a combined microscopic transoral and endoscopic endonasal approach was used for resection.

When we consider the advantages of these approaches (MTA/EEA), both have the advantage of providing an excellent extradural route without brain retraction.

Exposure from an MTA is limited by the degree of mouth opening and depends on the patient’s tongue size, position of the soft palate, and size of the oral cavity.[ 26 ] It provides midline exposure of the inferior third of the clivus, reaching the body of C2–C3.[ 11 ] On the other hand, the EEA surgical field extends from the frontal sinus to the CVJ.[ 26 ] Superior clivus chordomas GTR rates through an EEA approach 100%; however, the percentage decreases to 50% when the chordoma extends toward the middle third of the clivus.[ 20 ] Considering this, chordomas that involve the lower clivus and the CVJ present a limitation for EEA.

The nasopalatine line described by Kassam predicts the inferior limit of the view in EEA.[ 12 ] This line is formed from a projection that begins at the inferior tip of the nasal bone, joining the posterior limit of the hard palate on the midline and extending to the CVJ. Analyzing this, we can anticipate a remnant tumor on the caudal portion and complement it with an MTA. Furthermore, it is important to define the lateral extension of the chordoma, knowing that the lateral limit is the hypoglossal canal and, in some cases, the Eustachian tube.[ 15 ]

The postoperative outcomes of the EEA and MTA must be analyzed when selecting the procedure. The median length of hospital stay on the EEA in some series has been reported to be around 9 days, with a postoperative Karnofsky Performance Scale (KPSS) of 90% and recovery rates from cranial nerve palsy up to 75%.[ 7 ]

GTR is the desirable result when we operate on any brain tumor, but we need to consider the importance of the surrounding structures of each surgical field. Thus, a marginal resection should be considered as an acceptable result, taking into consideration that 10-year recurrence rates vary from 16% to 45%. Even when the macroscopic result seems to achieve GTR, most chordomas are likely to regrow.[ 3 ] CSF leak is one of the main complications of the procedure, with varying rates from 3% to 30%.[ 7 ] Clival chordomas are attached to the dura, and it is important to detach the tumor from it as far as possible without damaging its integrity, given that it is associated with higher CSF leak rates.[ 2 ] Considering this and the surrounding vital structures of the area, the resection of our case was planned to be, most likely, subtotal.

CONCLUSION

MTA and EEA offer significant advantages for the management of clival chordomas. While the EEA tends to displace fewer structures, the MTA provides adequate exposure to the lower third of the clivus. However, achieving GTR can be challenging due to anatomical limitations, and postoperative recurrence is a risk to consider. Selection of the surgical approach should be based on careful assessment of risks and benefits, as well as the specific anatomy of the patient and the lesion.

Ethical approval

The Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Video available on:

https://doi.org/10.25259/SNI_323_2024

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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