55 Management of an Extensive Recurrent Clivus Chordoma

55

Management of an Extensive Recurrent Clivus Chordoma

LALIGAM N. SEKHAR AND RAMIN RAK

Diagnosis Extensive recurrent clivus chordoma

Problems and Tactics The patient presented here is a young woman with a history of an extensive and recurrent skull base chordoma, for which she had undergone two partial resections with proton beam radiation between the surgeries in another institution, both via sublabial transmaxillary approach. Postoperative magnetic resonance imaging after these two procedures showed regrowth of the residual tumor with worsening of her symptoms. She was then referred to us for the management of this extensive clivus chordoma.

Keywords Extensive recurrent clivus chordoma, three-stage microsurgical resection

Clinical Presentation

The patient is a 34-year-old woman with a history of an extensive skull base chordoma. Her initial symptoms started ~6 months prior to the first medical consultation. These included blurry vision, neck pain with spasm, and dizziness. With these symptoms she was initially diagnosed to be stressed from work. Her symptoms worsened over time and she also developed a right-sided hearing difficulty. The patient then started having double vision, and magnetic resonance imaging (MRI) of the head was ordered by an ophthalmologist. An extensive clivus lesion consistent with chordoma was discovered with this MRI and she was referred to another neurosurgeon for surgical management of this lesion. First, the patient underwent a partial resection of this giant chordoma through a sublabial transmaxillary approach. Two months postoperatively she underwent proton beam radiation therapy in 40 sessions. However, the tumor continued to grow; and her symptoms progressed with severe headaches and left-sided tongue weakness. She then underwent a second partial resection of the tumor through the same sublabial transmaxillary approach. This was followed by 30 sessions of hyper-baric O₂ treatments. The tumor continued to grow, with the patient now developing right vocal cord palsy and difficulty swallowing and speaking. At this point she was referred to us for continuation of care. At our examination she had a partial right cranial nerve (CN) III palsy, a complete right CN VI palsy, decreased right hearing, right vocal cord palsy, and a left CN XII palsy. The patient had full strength in all extremities. She had difficulty with walking and balance and was losing weight.

Surgical Technique

This was a very difficult and extensive skull base tumor (Fig. 55–1), and a three-stage resection via three different approaches was initially planned.

FIGURE 55–1 (A–C) Magnetic resonance imaging at presentation (after the first two previous resections through transmaxillary approaches in an outside institution) showing the extent of the tumor recurrence.

FIGURE 55–2 Drawing of the subtemporal–infratemporal approach used for the first-stage resection of the tumor.

Stage One: Preauricular Subtemporal–Infratemporal Approach

The first procedure was a partial resection of the tumor via a right subtemporal–infratemporal approach (Fig. 55–2). A right-sided orbitozygomatic, frontotemporal craniotomy (Fig. 55–3) was performed for this approach.¹ After head fixation in a position slightly rotated (70 degrees) to the right and extension, the neuronavigation system was set up and the patient registered into the system with acceptable accuracy. A bicoronal incision, including exposure of the zygoma on the right side, was performed. A right-sided orbitozygomatic osteotomy and frontotemporal craniotomy were performed.