Radiosurgery for chordomas and chondrosarcomas of the skull base

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Object

Chordomas and chondrosarcomas of the skull base are aggressive and locally destructive tumors with a high tendency for local progression despite treatment. The authors evaluated the effect of stereotactic radiosurgery (SRS) on local tumor control and survival.

Methods

Twenty-eight patients with histologically confirmed chordomas (18) or chondrosarcomas (10) underwent Gamma Knife SRS either as primary or adjuvant treatment. Their ages ranged from 17 to 72 years (median 44 years). The most common presenting symptom was diplopia (26 patients, 93%). In two patients, SRS was the sole treatment. Twenty-six patients underwent between one and five additional surgical procedures. Two underwent an initial trans-sphenoidal biopsy. The average tumor volume was 9.8 cm3. The median dose to the tumor margin was 16 Gy.

Results

No patient was lost to follow-up. Transient symptomatic adverse radiation effects developed in only one patient. The actuarial local tumor control for chondrosarcomas at 5 years was 80 ± 10.1%. For chordomas both the actuarial tumor control and survival was 62.9 ± 10.4%.

Conclusions

Stereotactic radiosurgery is an important option for skull base chordomas and chondrosarcomas either as primary or adjunctive treatment. Multimodal management appears crucial to improve tumor control in most patients.

Abbreviations used in this paper:CGE = cobalt gray equivalent; CT = computed tomography; long-TR = long relaxation repetition time; MR = magnetic resonance; PBRT = proton-beam radiation therapy; SRS = stereotactic radiosurgery.

Although generally slow growing, skull base chordomas are locally aggressive and destructive tumors with high recurrence rates. These uncommon tumors are thought to arise from remnants of the notochord. Such remnants may persist anywhere along the neuraxis; however, the sacrum and clivus are the most frequent sites.12,28 Chondrosarcomas originate from primitive mesenchymal cells within the cartilaginous matrix of the skull base.12 The imaging features and clinical presentations of patients harboring either chordomas or chondrosarcomas are similar. An abducens palsy causing diplopia is the most frequent presenting sign.23,37 Whereas chordomas have a tendency to cause brainstem compression because they arise from the clivus, chondrosarcomas tend to affect the lower cranial nerves as they frequently originate from the occipitotemporal bone synchondrosis.35

Microsurgical removal is the primary management option for large lesions causing brainstem compression.4,31–33 Complete resection is rarely feasible because these tumors tend to encase critical vessels and cranial nerves or adhere to the brainstem. Surgical and postoperative morbidity and mortality rates remain high even with current single or staged microsurgical procedures.1,10,21,32,34 Recently, endoscopic skull base approaches have shown promising preliminary results.15,17,18 Nevertheless, the recurrent and invasive nature of these lesions warrants multimodal management to improve long-term control rates and outcomes.2,5,6

Various modalities of fractionated radiation therapy have been used to increase local control or halt disease progression.8,13,25,29 Stereotactic radiosurgery delivers more conformal radiation with greater radiobiological effect than conventional radiation therapy. It also reduces the risk of toxicity to surrounding critical structures and is associated with fewer complications.9,14,19,20,22 In the present analysis we seek to define the outcomes of patients harboring a chordoma or chondrosarcoma who underwent SRS as a primary or adjuvant treatment.

Clinical Material and Methods

Patient Population

Between August 1987 and December 2004, 33 patients underwent SRS for initial or recurrent chordomas or chondrosarcomas of the skull base at the Center for Image Guided Neurosurgery at the University of Pittsburgh. This review focuses on 28 patients (11 female and 17 male patients) whose chordoma or chondrosarcoma was confirmed after resection or biopsy. Patient ages ranged from 17 to 72 years (median 44 years). The most common presenting symptom was diplopia (26 patients, 93%). Diplopia was related to abducens nerve palsy alone in 13 patients and to multiple cranial nerve palsies in the remaining patients. Additional cranial nerve deficits prior to SRS are listed in Table 1.

TABLE 1

Presenting cranial neuropathies in 28 patients at the time of presentation*

CNs w/ DeficitsNo. of Patients
I0
II4
III11
IV11
V10
VI26
VII6
VIII4
IX10
X10
XI6
XII5

* CN = cranial nerve.

Twenty-six patients (93%) had undergone one or more resections (mean 2.3) before SRS. Two had a transsphenoidal biopsy for tissue confirmation without tumor removal. Among the 26 patients with previous resections, near-total tumor resection (≥ 80% of the tumor volume) was achieved in 16 patients (62%) and partial resection in 10. Nineteen (73%) of these 26 patients developed new post-surgical neurological deficits or permanent cranial neuropathies (Table 2). Fifteen patients had fractionated radiation therapy (mean dose 65 Gy) and seven patients had PBRT (mean dose 75 CGE) before radiosurgery, 20 within 3 months of their initial resection. One patient underwent radiation therapy 16 months after a second resection because of tumor progression. Another patient had radiation therapy 25 months after a two-stage resection. Table 3 summarizes patient demographics and radiosurgery indications.

TABLE 2

New neurological deficits after initial resection in 23 patients

Neurological DeficitNo. of Patients
no. of CN deficits
 15
 23
 ≥39
hemiparesis2
hydrocephalus4
TABLE 3

Patient demographics and indications for SRS*

Case No.Age (yrs), SexTumor TypeNo. of Prior ResectionsResidual Tumor at SRSRecurrence Postop & FRT at Time of SRSPrimary SRS
143, FCS2noyesno
241, MCS3noyesno
338, MCS3noyesno
441, FCS2yesnono
553, MCS1noyesno
617, MCS2noyesno
771, FCS1noyesno
830, FCS4noyesno
962, FCS3noyesno
1042, FCS2noyesno
1167, MCD3noyesno
1272, MCD1yesnono
1334, FCD1yesnono
1437, MCD3noyesno
1552, FCD2noyesno
1624, FCD2noyesno
1726, FCD3noyesno
1841, MCD2noyesno
1958, MCD2noyesno
2045, MCD4noyesno
2166, MCD2noyesno
2227, MCD2yesnono
2344, MCD0nonoyes
2455, MCD3noyesno
2556, MCD3noyesno
2623, MCD4noyesno
2747, MCD0nonoyes
2828, FCD5noyesno
total4222

* CD = chordoma; CS = chondrosarcoma; FRT = fractionated radiation therapy.

Gamma Knife SRS was used as primary treatment in two patients after biopsy alone. After resection four patients had SRS without fractionated radiation therapy.

Radiosurgery Technique

The technique of Gamma Knife SRS has been described in detail in our previous publications.22 Briefly, the Leksell Model G stereotactic frame (Elekta AB) was applied to the head after administration of mild sedation and local anesthesia. Axial stereotactic images were obtained using CT scanning before 1991 and MR imaging since then. Stereotactic volumetric contrast-enhanced spoiled gradient recalled acquisitions were obtained. Axial 1- to 1.5-mm-thick slices covered the entire tumor and the skull base. These images were supplemented with long-TR images. For long-TR images we performed a variable echo multiplanar sequence with both T2-weighted and proton density images. The dose planning was performed using either the KULA software (in eight patients) or the Leksell GammaPlan Software (in 20 patients). The current 4C version of the GammaPlan software allows us to coregister follow-up images with studies performed at radiosurgery for volumetric calculations to evaluate tumor control.

The mean tumor volume at radiosurgery was 9.8 cm3 (range 0.078–22 cm3). Radiation doses to the tumor margin varied between 10.5 and 25 Gy, with a mean of 16.5 Gy and a median of 16 Gy. Maximum tumor doses ranged from 21 to 50 Gy with an average of 33.0 Gy. The 50% isodose line was used in all but one patient whose tumor volume corresponded to the 70% isodose. The number of iso-centers used to achieve conformal plans varied from one to 15 (median nine). The maximum tumor diameter was 35 mm or smaller in most cases. For larger lesions SRS was performed only if additional microsurgical or endoscopic resection was rejected by the patient. Progression was defined as an increase in tumor volume of 25% or greater on follow-up images.

Data were entered into an SPSS database (SPSS, Inc.) and analyzed using univariate and multivariate models for significant factors. Kaplan–Meier survival plots were obtained for tumor control.

Results

No patient was lost to follow-up. The mean follow-up period was 7.7 years (range 2–17 years). For the purpose of this study we considered patients with chordomas and chondrosarcomas as two separate groups with different expectations and prognosis. In the multivariate analysis none of the factors (age, sex, marginal dose, tumor volume, and number of prior resections) reached statistical significance for tumor control or survival.

Tumor Groups

Chondrosarcomas

Stereotactic radiosurgery was used in 10 patients with chondrosarcomas; eight were still alive at the time of this analysis and all had stable lesions. The mean follow-up interval was 86 months, with a median of 88 months. None of these patients required additional surgical intervention or radiation therapy after radiosurgery. One patient underwent radiosurgery twice. After treatment of a residual tumor in the cavernous sinus region, a recurrence in the contralateral cavernous sinus was found 150 months later. Another patient, whose tumor had a high proliferation index and nuclear pleomorphism, died 4 months after radiosurgery of tumor progression and multiple distant metastases to the lung, liver, kidney, and bone. This tumor was categorized as a malignant chondrosarcoid tumor. One patient died of unrelated disease without evidence of tumor progression. The MR images in this patient showed stable tumor 78 months after radiosurgery (Fig. 1).

Fig. 1.
Fig. 1.

Axial contrast-enhanced T1-weighted MR images showing a residual chondrosarcoma at the time of radiosurgery (left) and significant regression of the treated lesion at 78 months (right). This patient died of malignant metastatic melanoma.

The local tumor control with a single SRS at 5 years was 80 ± 10.1%. Actuarial tumor control at 6 years was 88 ± 10.5% (Fig. 2).

Fig. 2.
Fig. 2.

Kaplan–Meier plot showing tumor control after radiosurgery for skull base chondrosarcomas.

Chordomas

Eighteen patients with chordomas underwent SRS. The average follow-up interval in this group was 7.6 years (median 88 months); 12 were still alive at the time of analysis. Figure 3 shows an example of tumor regression 10 years after radiosurgery. One patient at 88 months showed tumor progression and underwent two additional resections followed by PBRT. This patient developed radiation necrosis of the pharyngeal mucosa, a fistula, and a subdural retroclival empyema requiring surgical drainage. The patient remained severely disabled. Seven patients died of tumor progression despite additional resections in all seven; two of these patients had also undergone additional fractionated radiation therapy.

Fig. 3.
Fig. 3.

Axial (A and C) and coronal (B and D) T1-weighted contrast-enhanced MR images. A and B: A recurrent chordoma is revealed in a patient who had third, fourth, and sixth cranial nerve palsies and upper-extremity Grade 3 weakness, related to a left cavernous sinus and upper clivus lesion at the time of radiosurgery. C and D: Images obtained at 144-month follow-up showing regression of the tumor. This patient had complete resolution of cranial nerve deficits and improvement in right upper-extremity motor function.

The actuarial tumor control for chordomas was 53.4 ± 9.7% at 5 years after a single SRS session (Fig. 4). With the addition of a second radiosurgical procedure in three patients, overall tumor control and survival at 5 and 10 years was 62.9 ± 10.4% (Fig. 5).

Fig. 4.
Fig. 4.

Kaplan–Meier plot showing tumor control after a single SRS procedure for skull base chordomas.

Fig. 5.
Fig. 5.

Kaplan–Meier plot showing tumor control and survival after single or repeated SRS (three patients) for skull base chordomas.

Adverse Radiation Effects

None of the patients suffered any early morbidity after radiosurgery. Three patients developed adjacent brain T2 signal changes noted on MR imaging at 5, 6, and 12 months. Two patients did not have any associated clinical symptoms and did not require any intervention. These imaging changes resolved after 3 and 4 months. One patient suffered concomitant dizziness and nausea 5 months after radiosurgery. The symptoms resolved completely after a 2-week course of corticosteroids. No new cranial nerve, motor, or sensory deficits were detected during follow-up.

Discussion

Study Overview

The present study indicates both the promise and difficulties of multimodal management of chordomas and chondrosarcomas of the skull base. We found that chondrosarcomas generally respond well to radiosurgery, a modality that can be considered as a primary management for selected cases defined by biopsy or compatible imaging. The local tumor control rate of chondrosarcomas at 5 years was 80 ± 10.1%. In contrast, chordomas, which are frequently aggressive and locally destructive tumors with a high tendency for local progression, have an actuarial tumor control rate of 53% at 5 years after a single procedure. The addition of a second radiosurgical procedure for eligible patients improved the overall tumor control and survival at both 5 and 10 years to 62.9 ± 10.4%. In this study the 18 patients with chordomas underwent multimodality treatment, including resection, repeated resection, radiation therapy (in most patients), and radiosurgery. At present, radiosurgery for chordomas is best considered as an adjuvant management except for very small, biopsy-proven chordomas wherein high-dose, highly conformal, highly selective radiosurgery can be given. The addition of radiosurgery after resection and radiation therapy or PBRT has been shown to increase both local tumor control and survival (Table 4).9,19,20,22,26,35

TABLE 4

Results of Gamma Knife surgery for patients with chordomas and chondrosarcomas in published studies*

Authors & YearTumor TypeTreatmentMean Vol (cm3)Tumor Control (%)FU (mos)
Muthukumar et al., 1998CD, CSresect & FRT4.67340
Pamir et al., 2004CDresect & FRT<307223
Feigl et al., 2005CD, CSresect9.79317
Krishnan et al., 2005CD, CSresect & FRT14.475NR
Wanebo et al., 2006CSresect, FRT, & PBRTNR93NR
present series
 10 patientsCSresect9.88076
 18 patientsCDresect, FRT, & PBRT10.16290

* FU = follow-up; NR = not reported; resect = resection.

For tumors without compression of the brainstem or local mass effect, SRS is an alternative to resection. Radio-surgery is used as a primary or adjuvant management for small- to medium-volume tumors. We are able to define the target using high-resolution MR imaging coupled with long-TR as well as contrast-enhanced imaging, and occasionally supplemented by concomitant CT imaging to define bone skull destructive edges. The selection of dose for radiosurgery is dependent on the past administration of conventional fractionated radiation therapy, the tumor volume, and the expected tolerance of surrounding critical neurovascular structures. In our experience, none of our patients suffered permanent radiation effects. The decision to deliver radiosurgery in 22 patients after one or more re-sections and radiation therapy was made at the time of symptomatic progression. In the future, it is possible that adjuvant radiosurgery should be considered early after resection. At that time, the tumor volume can be defined by repeated MR and CT imaging. Only in selected patients can SRS for chordoma be considered the primary management modality after biopsy. There were two such cases in this series, and tumor control was achieved at 57 and 98 months. It is also possible that radiosurgery should be considered in a prospective clinical trial after conventional fractionated radiation therapy and after subtotal removal. Early upfront multimodal management may improve overall tumor response and long-term tumor growth control.

Many clinical series have been published regarding the possible optimal management of chordomas and chondrosarcomas of the skull base. Randomized clinical trials comparing results using these available options have not been conducted, but in the future, data from multiple centers with experience related to this rare tumor may improve our clinical understanding of various management strategies and the timing of their application. Most clinical series (including the present study) often report the results of a combination of two or more therapeutic interventions, usually applied after documented tumor progression. For example, in a series of 26 patients with cranial chordomas diagnosed between 1940 and 1984, Raffel et al.27 reported mortality rates of 43% at 5 years and a median survival of 6.4 years. Twenty-three of 26 patients had radiation therapy after resection.

The Role of Microsurgery

The use of microsurgical techniques combined with newer skull base endoscopic strategies may facilitate better subtotal or even radical total resection of tumor using one or more procedures. Following conventional microsurgical resection in a series of 60 patients, Gay et al.10 reported a 65% survival rate in patients with chordomas and a 90% survival rate in those with chondrosarcomas with a median follow-up of 3.9 years. Although three patients died after surgery, two others sustained strokes, four had postoperative hematomas, eight had cerebrospinal fluid leakage, and six developed meningitis, this aggressive approach may be warranted in view of the long-term overall poor prognosis of patients with chordomas.

Al-Mefty and Borba1 reported on 23 patients with chordomas who underwent one or more surgical procedures. Radical removal was possible in 10 patients (43%) but was subtotal in 11 (48%) and partial in two. Most patients underwent photon or PBRT after surgery. At a median follow-up of 25 months, 71% of the patients were alive. One patient died postoperatively, two patients developed new cranial nerve deficits, and three had radiation-related complications. Sekhar et al.30 reported long-term outcomes in 64 patients who underwent skull base procedures for either chordomas or chondrosarcomas. A total or near-total resection was possible in 36 patients (56%). Cerebrospinal fluid leakage occurred in 13 patients (20%), and five patients developed meningitis. Thirteen patients (20%) developed new abducens nerve palsies, and 10 (16%) developed new trigeminal nerve deficits. Two patients sustained a major stroke, and eight had other vascular injuries. These authors stated that complications were greater in patients who had already undergone initial management elsewhere.

Role of Fractionated Radiation Therapy

To date, the overall results of fractionated radiation therapy for residual or recurrent chordomas and chondrosarcomas of the skull base are disappointing. The poor tumor control rate after radiation therapy has been attributed to the poor sensitivity of these tumors to conventional fractionated radiation therapy. In addition, higher complication rates related to the important neurovascular structures nearby limit doses to the tumor itself. The dose to the visual pathway is the most common limiting factor.3,11 Jiang et al.16 reported the incidence of optic nerve injury and visual pathway injury following fractionated radiation. Of 219 patients who received radiation therapy between 1969 and 1985, 4% developed ipsilateral blindness due to optic neuropathy, and 11 (5%) developed bilateral visual impairment secondary to chiasmal radiation injury. Toxicity and optic neuropathy increased in patients who received more than 50 Gy to the optic apparatus. Brainstem tolerance was assessed by Debus et al.7 in a series of 367 patients who underwent PBRT between 1974 and 1995. Patients received radiation doses ranging from 63 to 79 CGE (based on the putative increased radiobiological responsiveness of protons to photons). Seventeen patients (4.6%) developed brainstem toxicity resulting in the death of three patients. The risk of developing toxicity was higher in diabetic patients and was dependent on the volume of the brainstem receiving greater than 60 CGE. Temporal lobe toxicity, as defined by MR imaging findings in patients who had headache, memory loss, alterations in mental status, coma, and death, is associated with either photon radiotherapy or PBRT necrosis and is not related to tumor progression.

Proton-Beam Radiation Therapy

Proton-beam radiation therapy for chordomas and chondrosarcomas has been based on the theoretically beneficial radiobiological effectiveness of protons in comparison with photon irradiation. In a series of 58 patients treated with PBRT and current 3D planning systems, the control rate (at an average follow-up of 33 months) was 76% for chordoma and 92% for chondrosarcoma. Delayed toxicity was confirmed in four patients (7%) including temporal lobe necrosis in one, seizures in one, hearing loss in two, bilateral visual loss in one, and pituitary insufficiency in four.13 A longer follow-up in this series would be needed to assess control at 5 or 10 years. Noël et al.24,25 reported on 90 patients with chordomas and chondrosarcomas who were treated with a combination of protons (4 days per week) and photons (1 day per week). At a median follow-up of 34 months, local control was achieved in 65 patients (72%). All 90 patients developed immediate adverse radiation effects, usually mild. However, 6% reported late Grade III or IV radiation toxicities including cranial nerve deficits and visual loss.

Conclusions

The ability to achieve tumor growth control of chordomas and chondrosarcomas is likely to be enhanced by earlier recognition and the application of multimodal treatment in appropriate patients. In patients with larger tumors cytoreductive surgery by craniotomy or expanded endonasal approaches may facilitate early application of radiosurgery. Patients with chordomas may benefit from additional conformal fractionated external-beam radiation therapy. In our experience, chondrosarcomas have high tumor control rates and may be suitable for surgery followed by radiosurgery alone. In contrast, chordomas represent more aggressive tumors that, over the course of time, require multimodal management including surgery, repeat surgery, fractionated radiation therapy, and boost radiosurgery.

Disclosure

Drs. L. Dade Lunsford, Douglas Kondziolka, and Ajay Niranjan are consultants for Elekta AB.

References

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    al-Mefty OBorba LA: Skull base chordomas: a management challenge. J Neurosurg 86:1821891997

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    Arnautovic KIAl-Mefty O: Surgical seeding of chordomas. J Neurosurg 95:7988032001

  • 3

    Austin JPUrie MMCardenosa GMunzenrider JE: Probable causes of recurrence in patients with chordoma and chondrosarcoma of the base of skull and cervical spine. Int J Radiat Oncol Biol Phys 25:4394441993

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    Bosma JJKirollos RWBroome JEldrige PR: Primary intradural classic chondrosarcoma: case report and literature review. Neurosurgery 48:4204232001

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    Crockard HACheeseman ASteel TRevesz THolton JPlowman N: A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg 95:1841892001

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    Crockard HASteel TPlowman NSingh ACrossman JRevesz T: A multidisciplinary team approach to skull base chordomas. J Neurosurg 95:1751832001

  • 7

    Debus JHug EBLiebsch NJO'Farrel DFinkelstein DEfird J: Brainstem tolerance to conformal radiotherapy of skull base tumors. Int J Radiat Oncol Biol Phys 39:9679751997

  • 8

    Debus JSchulz-Ertner DSchad LEssig MRhein BThillmann CO: Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:5915962000

  • 9

    Feigl GCBundschuh OGharabaghi ASafavi-Abassi SEl Shawarbi ASamii M: Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas. J Neurosurg 102:Suppl1651702005

  • 10

    Gay ESekhar LNRubinstein EWright DCSen CJanecka IP: Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:8878971995

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    Glosser GMcManus PMunzenrider JAustin-Seymour MFullerton BAdams J: Neuropsychological function in adults after high dose fractionated radiation therapy of skull base tumors. Int J Radiat Oncol Biol Phys 38:2312391997

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    Heffelfinger MJDahlin DCMacCarty CSBeabout JW: Chordomas and cartilaginous tumors at the skull base. Cancer 32:4104201973

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    Hug EBLoredo LNSlater JDDeVries AGrove RISchaefer RA: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:4324391999

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    Hug EBSlater JD: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. Neurosurg Clin N Am 11:6276382000

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    Jho HDCarrau RLMcLaughlin MRSomaza SC: Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. Acta Neurochir (Wien) 139:3433481997

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    Jiang GLTucker SLGuttenberger RPeters LJMorrison WHGarden AS: Radiation-induced injury to the visual pathway. Radiother Oncol 30:17251994

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    Kassam ASnyderman CHMintz AGardner PCarrau RL: Expanded endonasal approach: the rostrocaudal axis. Part II Posterior clinoids to the foramen magnum. Neurosurg Focus 19:1E42005

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    Kassam ABGardner PSnyderman CMintz ACarrau RL: Expanded endonasal approach: fully endoscopic, completely trans-nasal approach to the middle third of the clivus, petrous bone, middle cranial fossa, and infratemporal fossa. Neurosurg Focus 19:1E62005

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    Kondziolka DLunsford LDFlickinger JC: The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base. Neurosurgery 29:38461991

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    Krishnan SFoote RLBrown PDPollock BELink MJGarces YI: Radiosurgery for cranial base chordomas and chondrosarcomas. Neurosurgery 56:7777842005

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    Muthukumar NKondziolka DLunsford LDFlickinger JC: Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences. Int J Radiat Oncol Biol Phys 41:3873921998

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    Pamir MNKiliç TTüre UOzek MM: Multimodality management of 26 skull-base chordomas with 4-year mean follow-up: experience at a single institution. Acta Neurochir (Wien) 146:3433542004

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    Raffel CWright DCGutin PHWilson CB: Cranial chordomas: clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery 17:7037101985

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    Sekhar LNPranatartiharan RChanda AWright DC: Chordomas and chondrosarcomas of the skull base: results and complications of surgical management. Neurosurg Focus 10:3E22001

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    Sen CNSekhar LNSchramm VLJanecka IP: Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery 25:9319411989

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Article Information

Address correspondence to: L. Dade Lunsford, M.D., Suite B-400, University of Pittsburgh Medical Center–Presbyterian, 200 Lothrop Street, Pittsburgh, Pennsylvania 15213. email: lunsfordld@upmc.edu.

© AANS, except where prohibited by US copyright law."

Headings

Figures

  • View in gallery

    Axial contrast-enhanced T1-weighted MR images showing a residual chondrosarcoma at the time of radiosurgery (left) and significant regression of the treated lesion at 78 months (right). This patient died of malignant metastatic melanoma.

  • View in gallery

    Kaplan–Meier plot showing tumor control after radiosurgery for skull base chondrosarcomas.

  • View in gallery

    Axial (A and C) and coronal (B and D) T1-weighted contrast-enhanced MR images. A and B: A recurrent chordoma is revealed in a patient who had third, fourth, and sixth cranial nerve palsies and upper-extremity Grade 3 weakness, related to a left cavernous sinus and upper clivus lesion at the time of radiosurgery. C and D: Images obtained at 144-month follow-up showing regression of the tumor. This patient had complete resolution of cranial nerve deficits and improvement in right upper-extremity motor function.

  • View in gallery

    Kaplan–Meier plot showing tumor control after a single SRS procedure for skull base chordomas.

  • View in gallery

    Kaplan–Meier plot showing tumor control and survival after single or repeated SRS (three patients) for skull base chordomas.

References

1

al-Mefty OBorba LA: Skull base chordomas: a management challenge. J Neurosurg 86:1821891997

2

Arnautovic KIAl-Mefty O: Surgical seeding of chordomas. J Neurosurg 95:7988032001

3

Austin JPUrie MMCardenosa GMunzenrider JE: Probable causes of recurrence in patients with chordoma and chondrosarcoma of the base of skull and cervical spine. Int J Radiat Oncol Biol Phys 25:4394441993

4

Bosma JJKirollos RWBroome JEldrige PR: Primary intradural classic chondrosarcoma: case report and literature review. Neurosurgery 48:4204232001

5

Crockard HACheeseman ASteel TRevesz THolton JPlowman N: A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg 95:1841892001

6

Crockard HASteel TPlowman NSingh ACrossman JRevesz T: A multidisciplinary team approach to skull base chordomas. J Neurosurg 95:1751832001

7

Debus JHug EBLiebsch NJO'Farrel DFinkelstein DEfird J: Brainstem tolerance to conformal radiotherapy of skull base tumors. Int J Radiat Oncol Biol Phys 39:9679751997

8

Debus JSchulz-Ertner DSchad LEssig MRhein BThillmann CO: Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:5915962000

9

Feigl GCBundschuh OGharabaghi ASafavi-Abassi SEl Shawarbi ASamii M: Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas. J Neurosurg 102:Suppl1651702005

10

Gay ESekhar LNRubinstein EWright DCSen CJanecka IP: Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:8878971995

11

Glosser GMcManus PMunzenrider JAustin-Seymour MFullerton BAdams J: Neuropsychological function in adults after high dose fractionated radiation therapy of skull base tumors. Int J Radiat Oncol Biol Phys 38:2312391997

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