Staged Surgical Management of a Chordoma Involving the Sphenoid Sinus, Sella Turcica, and Clivus: A Case Report
By Reza Jarrahy, M.D., Ruchi Mathur, M.D., Shlomo Melmed, M.D., Hrayr K. Shahinian, M.D., FACS
Chordomas of the clivus are aggressive lesions that pose unique diagnostic and management challenges. True diagnosis of chordoma can only be made pathologically. Clinical diagnosis is made by interpretation of patients' presenting symptoms and objective findings on physical examination. Radiographic studies supplement this information. Because symptoms are often vague and chronic and because radiographic presentation is not uniform among different imaging modalities, accurate diagnosis of clival chordoma is often delayed. Primary therapy is surgical and is geared toward as complete a removal of gross tumor mass as technically possible. Many single-stage operative techniques have been described in the surgical management of chordomas; choice among these depends on the location of the tumor and the individual practice of the surgeon. We present the case of a 78-year-old gentleman with a clival chordoma whose surgical management represents a two-stage resection not previously described. Employing transsphenoidal and transoral techniques in two separate procedures, we were able to achieve total resection of the lesion. There was minimal morbidity associated with either surgery and the patient was referred for adjuvant radiotherapy. There was no evidence of residual or recurrent tumor on radiographic imaging studies performed one year postoperatively.
Clival chordoma; surgical therapy; staged approach
Chordomas are tumors of notochordal origin that may affect the axial skeleton anywhere from the coccyx to the base of the skull, in either a midline or paramedian position. The cranial and caudal extremes of the spine are most often affected.1-5 Chordomas of the skull base are particularly debilitating due to aggressive involvement of local structures.6-11 Symptomatology caused by these masses is generally nonspecific. They are therefore usually discovered only after having undergone long periods of undisturbed growth.5,8,11,12
The impetus behind ordering diagnostic tests is usually the development of localizing symptoms.8 Imaging studies contribute to making the diagnosis of clival chordoma in a large number of cases, but not all.3,5,8,10 Several authors have reported the benefits of certain imaging modalities over others, but neither computed tomography nor magnetic resonance imaging has emerged as a definitive diagnostic test for the tumor.3,10,13
Likewise, no single surgical method has emerged as a standard of therapy for resection of these tumors, although numerous surgical approaches to the clivus are described.1,6,8,11-18 These are single-stage procedures that differ in the extent of dissection and the area of optimum exposure. Surgical goals involve as complete a removal of grossly identified tumor as possible.1,2,6,8,11-13 The role of adjuvant radiotherapy thereafter is still subject to debate.2,5,6,8,11,13,14 With the continued development of advanced microsurgical techniques in skull base surgery, more extensive dissections as well as combined approaches to the skull base have been advocated.1,6,11-13 However, more involved craniofacial manipulation may be associated with higher rates of complication and greater patient morbidity without greater disease-free survival.
We present the case of a clival chordoma initially presenting as an intrasphenoidal, intrasellar mass. Differential diagnosis included primary lesions of the pituitary and sphenoid sinus in addition to chordoma of the clivus. Surgical therapy was unique in that a two-stage procedure for removal of the mass was planned and implemented. Initial surgery was performed via a transseptal transsphenoidal approach to the sella. Follow-up surgery was via a midline transpalatal transoral approach to the middle and lower portions of the clivus. This combination of simple techniques allowed us to expose and remove all grossly apparent tumor and the entire body of the clivus.
Presentation and preoperative assessment
This is the case of a 78-year-old gentleman who was referred to his internist with a chief complaint of sudden onset diplopia not associated with dizziness, nausea, headache, tunnel vision, or ptosis. Other than a history of hypertension controlled with a diuretic and a beta-blocker, the patient had been in a good state of health until his presentation. Physical exam findings were significant for diplopia that was elicited on downward gaze and decreased acuity of the lateral visual fields bilaterally. The cranial nerves were otherwise intact.
Sagittal T1 weighted axial proton density and post-contrast T2 weighted M.R.I. scans of the head were obtained, revealing a large mass within the sphenoid sinus extending into the sella turcica and suprasellar spaces (Fig. 1). The mass measured 4 cm. in greatest diameter and was heterogeneous in nature, yet sharply defined. It extended to the clivus posteriorly. Impingement upon the optic chiasm was not definitively appreciated in coronal views. The right cavernous sinus was compressed but not invaded; similarly, the right carotid artery was displaced by the mass. These images also showed invasion of the clivus by the mass. Differential diagnosis included primary lesions of the pituitary or sphenoid (adenoma, inflammatory or fungal mass, lymphoma, carcinoma, melanoma) and chordoma of the clivus.
The patient was scheduled for surgical removal of the lesion via a translabial transseptal approach to the sphenoid sinus. He was informed that if the mass turned out to be a pituitary adenoma or sphenoid lesion, the transsphenoidal technique would most likely allow for a total resection. He was also made aware of our intention to perform an additional procedure in the event that pathological analysis revealed a chordoma: we would perform the second-stage operation via a transpalatal transoral approach to resect the lower portions of the clivus not adequately exposed transsphenoidally.
Dissection was initiated with a sublabial incision and continued transseptally until the vomer was identified and removed. Upon entering the sphenoid sinus, tumor was immediately visualized: it was fleshy, multi-lobulated, and tan in appearance, grossly suggestive of a chordoma. Frozen section analysis of the specimen by the neuropathologists confirmed this suspicion. Tumor was removed from the sphenoid sinus, the sella turcica, the environs of the cavernous sinuses bilaterally, and from the anterior clivus. The pituitary gland was normal in appearance. The clivus had a diseased and macerated appearance; it was resected as thoroughly as possible. Following the resection, 0°, 30°, and 70° 4.0-mm. endoscopes (K. Storz Endoscopy™) were placed into the surgical field to confirm gross removal of all tumor remnants. A small Teflon-coated pledget was placed in the region of the clivus; it would mark the extent of this dissection during the second-stage procedure.
Final pathological analysis on the specimens collected revealed epithelioid cells with vacuolated cytoplasm arranged in aggregates and chords separated by mucinous material. The cells showed positive reaction to S-100, keratin, EMA, and Vimentin immunostains.
The patient tolerated the procedure well and was discharged from the hospital three days after the surgery. His diplopia resolved and he continued to remain asymptomatic in the postoperative period. Follow-up M.R.I. performed three weeks later showed evidence of residual tumor involving the lower clivus. These findings were not evident on the preoperative radiographic studies. The bulk of the tumor documented on the original scans was no longer present and the pituitary gland, optic chiasm, cavernous sinuses, and carotid arteries were radiographically normal. Resection of the remainder of the clivus and residual chordoma was scheduled.
With the oral cavity held open by a Cockard transoral retractor, and with use of the C-arm for intraoperative fluoroscopic visualization of the clivus, the soft palate was dissected and retracted and the mucosa of the posterior oropharynx was incised in the midline. Bilateral mucosal flaps were elevated, exposing the underlying bone. Grossly, the clivus had a moth-eaten appearance and a soft texture consistent with invasion by a neoplastic process. The Teflon marker placed at the initial surgery was identified. The entire bony matter of the clivus was drilled and removed. The dura was identified and judged to be grossly free of disease. The mucosal flaps were reapproximated and the palate was repaired.
The patient's postoperative course was again unremarkable. He was discharged home without any complications with referral for adjuvant radiotherapy. A follow-up M.R.I. study done one year following the initial surgical procedure demonstrated normal sellar and parasellar architecture with regional changes consistent with postsurgical effects.
Chordomas are rare tumors of notochordal origin that present primarily in males6,8,9 in the third and fourth decades of life.6,8 They may appear in the axial skeleton anywhere from the sacrum to the base of the skull. Of the potential areas of cranial involvement, the sphenoocciput is the most likely area of manifestation.1,3-5,8,14 Chordomas of the clivus account for 1% of all intracranial tumors8,14 and may present in the midline or in paramedian position. Most are histologically benign without evidence of nuclear mitotic figures, although they do possess metastatic potential.8-10,19 They are most devastating, however, due to the degree of local invasiveness they demonstrate in such a critical region: morbidity at mortality from these tumors is attributable to their involvement of surrounding structures.
Symptoms associated with clival chordomas are typically non-specific and may persist chronically before acute or focal neurological findings develop.7,8,14 Among the most common complaints at initial presentation are diplopia and headache.4-7,14
Because the signs and symptoms caused by clival chordoma are common to a range of diseases, radiographic studies become an important source of information in identifying the nature and precise location of the disease process.3,5,8,10 CT and MRI are both used to image the bony and soft tissue anatomical relationship of the critical structures in the region of the skull base. However, neither is completely efficacious at differentiating clival chordoma from other lesions that may exist in the region.
Our patient presented with a mass involving the sphenoid sinus, sella turcica, and upper clivus. We decided that a transsphenoidal approach would be the best initial surgical procedure for attempted resection. We uniformly perform transseptal transsphenoidal hypophysectomy; excellent results with tumor removal via this route are well documented.20-23 If our patient's lesion proved to be a primary sphenoidal lesion or a pituitary adenoma with supra- and parasellar extension, a solitary surgical procedure would likely have been sufficient for complete resection. In the event that frozen section analysis confirmed the diagnosis of chordoma, the procedure would be the first of a two-stage operation geared toward complete removal of the clivus. A second procedure using a transoral technique would allow access to the middle and lower regions of the clivus.
The transseptal transsphenoidal approach to the clivus has been described but has been accorded limited usefulness in the surgical therapy of clival chordomas.1,4,12,13,24 Some authors state it is only indicated in small lesions confined to the midline of the upper clivus.1,4,8,11,13,14 Larger lesions, lesions involving the middle and lower portions of the clivus, and lesions extending into paramedian areas are not adequately exposed by this dissection.1,11,12,14 Similarly, the transoral approach does not allow adequate access to the upper clivus and parasellar areas for resection of tumor in these regions.8,11,12,14 The use of extensive craniofacial dissections to maximize exposure has been advocated in the setting of large and complex clival lesions. Transbasal, cranioorbitozygomatic, subtemporal, transcondylar, transmaxillary, and extended transsphenoidal techniques have been proposed. Variable complication rates have been reported.5,6,13 These procedures are all geared toward as complete a resection as possible in one procedure. To date we have only identified three references to the role of staged procedures in the resection of these tumors.6,11,12 However, no particular surgical protocols are described.
In order to minimize patient morbidity and the potential complications associated with the more involved manipulations of the craniofacial skeleton and skull base, we used a two-stage procedure to remove a clival chordoma involving the sphenoid sinus, sella turcica, parasellar spaces, and upper and middle clivus. The transsphenoidal and transoral techniques were implemented due to the location of the tumor: the exposure achieved in the initial surgery allowed removal of the bulk of the tumor and examination of neighboring structures in the parasellar areas. The follow-up procedure enabled us to remove the remainder of the clivus and to thoroughly examine the dura for tumor remnants. Individually, neither technique could provide an exposure adequate enough for thorough resection of extensive lesions. In tandem, however, we were able to approach and resect the entirety of the clivus as well as fully appreciate the dura and all involved structures neighboring the sella turcica and sphenoid sinus. Postoperative complications were minimal due to the simple methods of dissection.
Although we are unable to draw definitive conclusions regarding patient morbidity and the incidence of complications or recurrence from a singular case report and without long term follow-up, it is worthwhile to continue to consider staged procedures--each involving less extensive dissection--in the surgical management of clival chordomas.
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