Cranial Base Chordomas Research Articles

Skull base chordomas: overview of disease, management options, and outcome

Cranial base chordomas are locally invasive tumors that, from a midline, clival location, extend in different directions and display various patterns of skull base invasion. Although histologically benign, their invasive nature makes true "oncological" resection virtually impossible to achieve in most cases, despite modern skull base surgical techniques. Moreover, because of the tumor's location and proximity to critical neural and vascular structures, surgery-related morbidity can be significant when an aggressive resection is undertaken. Cytoreductive surgery assumes a critical role in the management of these lesions. The choice of surgical approach and the extent of resection are dependent on several factors: location and extension of the tumor, the surgeon's philosophy and familiarity with a specific approach, and the patient's preexisting clinical status. Proton-beam radiotherapy seems to be effective as an adjunct to surgery in achieving local tumor control. The timing of radiation therapy, however, remains controversial. Gamma knife surgery has been proposed as an adjunctive therapy, but the limited experience and short follow-up periods do not permit formulation of meaningful conclusions at this time. Recurrences are common, although in a subset of patients prolonged disease-free survival is demonstrated.

The Role of Radiosurgery in the Management of Chordoma and Chondrosarcoma of the Cranial Base

Abstract Despite conventional multimodality treatment (surgery and fractionated radiation therapy), recurrence and clinical progression of cranial base chordomas and chondrosarcomas are common. The malignant behavior of these tumors is a result of their critical location, locally aggressive nature, and high recurrence rate. To explore the role of radiosurgery in the treatment of these skull base neoplasms, we assessed its use in four patients with chordoma and two with chondrosarcoma. In five of the patients, radiosurgery was used as adjuvant therapy for residual or recurrent tumors after surgical debulking, and in one patient with a chordoma, it was the primary treatment. No patient received fractionated external beam radiotherapy. All tumors were less than 30 mm in diameter and were treated with 20 Gy to the tumor margin. Skull base computed tomography and magnetic resonance images were essential to define the anatomic relationships between tumor and adjacent basal structures. During follow-up (mean, 22 mo; range, 8-36 mo), we found no progression of the treated tumor volume in any patient. Neurological deficits before treatment improved in three patients; the other three patients remained in stable neurological condition. Serial follow-up imaging studies demonstrated that two patients showed reduction in tumor size and four patients had no tumor growth. In one patient, a metastatic parietal lobe chondrosarcoma developed and was treated by microsurgery. Another patient showed tumor progression outside of the radiosurgical trcatment volume. Our results attest to the value of stereotactic radiosurgery as an adjuvant or primary treatment for selected patients with chordoma or chondrosarcoma and demonstrate its potential advantages over standard fractionated irradiation. Analysis of the long-term clinical and imaging effects after radiosurgery is warranted. (Neurosurgery 29:38-46, 1991)

The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base

Despite conventional multimodality treatment (surgery and fractionated radiation therapy), recurrence and clinical progression of cranial base chordomas and chondrosarcomas are common. The malignant behavior of these tumors is a result of their critical location, locally aggressive nature, and high recurrence rate. To explore the role of radiosurgery in the treatment of these skull base neoplasms, we assessed its use in four patients with chordoma and two with chondrosarcoma. In five of the patients, radiosurgery was used as adjuvant therapy for residual or recurrent tumors after surgical debulking, and in one patient with a chordoma, it was the primary treatment. No patient received fractionated external beam radiotherapy. All tumors were less than 30 mm in diameter and were treated with 20 Gy to the tumor margin. Skull base computed tomography and magnetic resonance images were essential to define the anatomic relationships between tumor and adjacent basal structures. During follow-up (mean, 22 mo; range, 8-36 mo), we found no progression of the treated tumor volume in any patient. Neurological deficits before treatment improved in three patients; the other three patients remained in stable neurological condition. Serial follow-up imaging studies demonstrated that two patients showed reduction in tumor size and four patients had no tumor growth. In one patient, a metastatic parietal lobe chondrosarcoma developed and was treated by microsurgery. Another patient showed tumor progression outside of the radiosurgical treatment volume. Our results attest to the value of stereotactic radiosurgery as an adjuvant or primary treatment for selected patients with chordoma or chondrosarcoma and demonstrate its potential advantages over standard fractionated irradiation. Analysis of the long-term clinical and imaging effects after radiosurgery is warranted.