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Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients

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Abstract

Previous studies of chordoma have focused on either surgery, radiotherapy, or particular tumor locations. This paper reviewed the outcomes of surgery and proton radiotherapy with various tumor locations. Between 2001 and 2008, 40 patients with chordomas of the skull base and cervical spine had surgery at our hospital. Most patients received proton therapy. Their clinical course was reviewed. Age, sex, tumor location, timing of surgery, extent of resection, and chondroid appearance were evaluated in regard to the progression-free survival (PFS) and overall survival (OS). The primary surgery (PS) group was analyzed independently. The extensive resection rate was 42.5%. Permanent neurological morbidity was seen in 3.8%. Radiotherapy was performed in 75% and the mean dose was 68.9 cobalt gray equivalents. The median follow-up was 56.5 months. The 5-year PFS and OS rates were 70% and 83.4%, respectively. Metastasis was seen in 12.5%. The tumor location at the cranio-cervical junction (CCJ) was associated with a lower PFS (P = 0.007). In the PS group, a younger age and the CCJ location were related to a lower PFS (P = 0.008 and P < 0.001, respectively). The CCJ location was also related to a lower OS (P = 0.043) and it was more common in young patients (P = 0.002). Among the survivors, the median of the last Karnofsky Performance Scale score was 80 with 25.7% of patients experiencing an increase and 11.4% experiencing a decrease. Multimodal surgery and proton therapy thus improved the chordoma treatment. The CCJ location and a younger age are risks for disease progression.

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Acknowledgements

We sincerely express our gratitude to Dr. Akiko Tamakoshi at the Department of Public Health in Aichi Medical University for technical support in statistical analysis and Dr. Giuseppe Mirone, Ms. Karin Aouabed, Ms. Aline Le Borne, and Ms. Haruko Kobayashi for technical support in writing this paper.

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The authors report no conflicts of interest concerning the materials or methods used in this study or the findings specified in this paper.

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Authors and Affiliations

  1. Department of Neurosurgery, Lariboisiere Hospital (AP-HP), Paris, France

    Muneyoshi Yasuda, Damien Bresson, Salvatore Chibbaro, Jan F. Cornelius & Bernard George

  2. Department of Pathology, Lariboisiere Hospital (AP-HP), Paris, France

    Marc Polivka

  3. Department of Radiotherapy, Pitie Salpetriere Hospital (AP-HP), Paris, France

    Loic Feuvret

  4. Institute Curie, Protontherapy Center of Orsay, Orsay, France

    Loic Feuvret

  5. Department of Neurosurgery, Aichi Medical University, Nagakute, Aichi, 480-1195, Japan

    Muneyoshi Yasuda & Masakazu Takayasu

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  1. Muneyoshi Yasuda
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Correspondence to Muneyoshi Yasuda.

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Venelin Gerganov, Ladislau Steiner, Hannover, Germany

Yasuda et al. report a series of 17 skull base, 13 cranio-cervical, and 10 spinal chordomas, managed between 2001 and 2008 in the Department of Neurosurgery of Lariboisiere Hospital, in the Protontherapy Center of Orsay and in the Radiotherapy Department of Pitie Salpetriere Hospital in Paris. The notochordial origin that determines the spread of the tumor, the lack of malignant histopathological characteristics in contrast to its aggressive behavior, its incidence, and the strategy of treatment, including radical surgical resection and radiation techniques not used prior to the last decade, are described in an occasionally crowded text with pertinent details providing a glimpse of the state of the art of chordoma management.

The authors emphasize that no chondrosarcomas are included in their series. Light microscopic findings and immunohistological staining secured the pathological diagnosis of chordoma. However, concerning localization, the tumors present a wide range of distribution. This makes the reading laborious. A homogeneous material would have excluded the difficulty to relate the provided information to the tumor to which it did belong.

The “basic strategy” of surgery was to achieve as radical resection as possible without inacceptable morbidity. If this was not feasible, the purpose of surgery was to achieve decompression of the brain stem, spinal cord, and optic pathways and to reduce the tumor size to one appropriate for radiation. Major vessels involved in the tumor were embolized before surgery or ligated during surgery if necessary and possible. Navigation and endoscopy were used if required. Electrophysiological monitoring and stimulation—a useful technique in tumors adjacent to eloquent structures of the brain—are not mentioned.

The extent of tumor resection was estimated by using the surgical records and postoperative magnetic imaging. An extensive radical resection was achieved in 17 (42.5%) cases. A subtotal resection, defined as 95% of tumor reduction, was achieved in 19 (47.5%) patients. Four (10.0%) patients had partial resection, defined as 80–95% of tumor reduction.

In the primary surgery group, nine (39.1%) patients had extensive radical resection, ten (43.5%) patients had a subtotal resection, and four (17.4%) patients had a partial resection. In the recurrency group, eight (47.1%) patients had extensive radical resection and nine (58.9%) patients had subtotal resection.

Yasuda et al. do not provide information about the method used for volumetry of tumor resection. In our experience, there is not one of them without flows. The 90% figure therefore should be considered as an approximation. Hence, any comparison of the volumetric data should be assessed stringently.

The complication rate was 22.6% considering all operations. In the primary surgery group, the complication incidence was 34.8% (eight patients). In the recurrency surgery group, the incidence of complications was 23.5% (four patients). Complications included CSF leakage in six (11.3%). Meningitis occurred in five (9.4%) patients. Two cases of hydrocephalus occurred after meningitis. A skull base meningocele occurred in one (1.9%) patient. Two patients developed postoperative hematoma. Permanent neurological deficits were observed in two (3.8%) patients. No mortality due to surgery occurred.

Yasuda et al. state that proton beam therapy and radical surgery are the gold standards for chordoma care. The advantage of proton beam due to the Bragg peak—its physical property of dose delivery—is well known. Delivered fractionated, pure, or combined with photon radiotherapy, its efficiency in chordomas is documented in hundreds of cases (see reference list of the current report).

Given the fact that SL has some experience with the Gamma knife radiosurgery, we mention that small volume postsurgery chordoma residuals respond well to the highly conformal single high dose of narrow ionizing cobalt beams of Leksell’s Gamma unit (1). However, its beneficial effect in chordoma is up to date documented in only a small series of chordomas. The five or more years of outcomes are comparable to those of proton beam treatment. Their reproducibility remains to be proved in a larger series.

In a well-written discussion, Yasuda et al. compare their resection rates, surgical complications, outcome of proton therapy, progression-free and overall survival, and functional outcome of survivors with the results of previously published series.

Tumor control in the recurrency group was better in the present study as compared with previous studies. Colli reported a 5-year progress-free survival rate of 32.5% in his recurrency surgery group, while the survival study demonstrated an actuarial 5-year progress-free survival of 81.8% and an actuarial 5-year overall survival of 77.1% in the recurrency group.

The influence of age on the progression of chordoma is still controversial. There are those who contend that patients younger than 20 years have a favorable clinical course. Others have suggested the possibility of a poor prognosis in children. In the primary surgery group of the current study, patients younger than 42 years old experienced a progress-free survival rate shorter than 5 years. Most of the rapid relapses and tumor-related deaths were observed among patients younger than 25 years old. Based on this fact, the authors of the current study strongly suspect that there is a poor prognosis in young patients with chordoma.

It seems that a type of chordoma has a tendency to invade both the skull and the cervical spine. The authors propose that this type should be classified as “malignant chordoma” or “fulminant chordoma.” Imatinib mesylate, a tyrosine kinase inhibitor, is reported to have a therapeutic effect on chordoma. Yasuda et al. conclude that the majority of patients in the current series kept or restored their ability to perform daily life and social life activities. The favorable outcome according to the authors was due to the radical extirpation of the tumor. However, the extent of required resection is difficult to determine when cranial nerves are entrapped in the tumor. In some situations, aggressive tumor resection may be more important than conservation of paralyzed nerves. One of the most important benefits of surgery was the decompression of important neural structures. Most of the patients with KPS score improvement underwent a decompressive surgical procedure for brain stem or spinal cord. With the progress in the survival rates of chordoma patients, it is becoming more important to maintain or promote their functional outcome.

The report of Yasuda et al. provides its reader plenty of useful information about chordoma and its management.

1. Steiner L, Yen CP. Comment to: Amichetti M, Cianchetti M, et al. Proton therapy in chordoma of the base of the skull: a systematic review. Neurosurg Rev. 2009; 32:403–416.

Benedicto Oscar Colli, Ribeirão Preto, Brasil

The authors performed a detailed and extensive retrospective analysis of the clinical outcome of 40 patients with intracranial chordomas primarily or secondary treated at the Lariboisiere Hospital (Paris).

They analyzed six factors aiming to establish its involvement in the prognosis of these patients: age, sex, tumor location, timing of surgery (primary or reoperation), extent of surgical removal and pathological findings (presence of condroid component).

A high percentage of radical or subtotal resection with less postoperative complication rates with operative mortality were got in this study and it is comparable to the best results reported in the literature. This is more expressive considering that 100% with a previous surgery had radical or subtotal resection with a similar rate of complication and no mortality. This improvement was attributed to predominance of these tumors in the cervical region or due or to the use of endoscope and neuronavigation.

An interesting finding of this study was the evidence of a better global progression-free survival (PFS) and overall survival (OS) rates for all patients and for patients that had a previous surgery, in comparison with the previous report. It is well known for patients with chordomas of the skull base that as longer is the follow-up, greater is the recurrence rates and differences in the follow-up time can explain in part the discrepancy observed in literature regarding the PFS and OS for these patients. The median follow-up in is study was 56.5 months that is not so long for patients with chordomas and many patients of this group had tumors of the cervical spine. Another important fact was that tumor control in the recurrence surgery group was also much more favorable in this study than in previous studies.

Regarding the factors that can influence the prognosis of these patients, the authors found that patients younger than 42 years-old had a worst 5-years PFS and that most and more precocious relapses occurred in patients with less than 25 year-old, suggesting a correlation of poor prognosis for young patients. Nevertheless, this is a controversial topic in the literature. Location in the craniocervical junction was found correlated with poor prognosis and, decompression of the brainstem and spinal cord was imputed to be one of the most important reasons for improvement in the postoperative KPS scores.

The good results of this study were attributed to the multidisciplinary surgical approaches (reduced surgical complications) and to the combination with proton-based therapy (satisfactory outcomes in the control of disease).

I would like to congratulate the authors by this laborious and good work that certainly contributed for better understanding of this complex disease.

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Yasuda, M., Bresson, D., Chibbaro, S. et al. Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients. Neurosurg Rev 35, 171–183 (2012). https://doi.org/10.1007/s10143-011-0334-5

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