Skull Base Chondrosarcomas Research Articles

Randomised trial of proton vs. carbon ion radiation therapy in patients with low and intermediate grade chondrosarcoma of the skull base, clinical phase III study

BackgroundLow and intermediate grade chondrosarcomas are relative rare bone tumours. About 5-12% of all chondrosarcomas are localized in base of skull region. Low grade chondrosarcoma has a low incidence of distant metastasis but is potentially lethal disease. Therefore, local therapy is of crucial importance in the treatment of skull base chondrosarcomas. Surgical resection is the primary treatment standard. Unfortunately the late diagnosis and diagnosis at the extensive stage are common due to the slow and asymptomatic growth of the lesions. Consequently, complete resection is hindered due to close proximity to critical and hence dose limiting organs such as optic nerves, chiasm and brainstem. Adjuvant or additional radiation therapy is very important for the improvement of local control rates in the primary treatment. Proton therapy is the gold standard in the treatment of skull base chondrosarcomas. However, high-LET (linear energy transfer) beams such as carbon ions theoretically offer advantages by enhanced biologic effectiveness in slow-growing tumours.Methods/DesignThe study is a prospective randomised active-controlled clinical phase III trial. The trial will be carried out at Heidelberger Ionenstrahl-Therapie (HIT) centre as monocentric trial.Patients with skull base chondrosarcomas will be randomised to either proton or carbon ion radiation therapy. As a standard, patients will undergo non-invasive, rigid immobilization and target volume definition will be carried out based on CT and MRI data. The biologically isoeffective target dose to the PTV (planning target volume) in carbon ion treatment will be 60 Gy E ± 5% and 70 Gy E ± 5% (standard dose) in proton therapy respectively. The 5 year local-progression free survival (LPFS) rate will be analysed as primary end point. Overall survival, progression free and metastasis free survival, patterns of recurrence, local control rate and morbidity are the secondary end points.DiscussionUp to now it was impossible to compare two different particle therapies, i.e. protons and carbon ions, directly at the same facility in connection with the treatment of low grade skull base chondrosarcomas.This trial is a phase III study to demonstrate that carbon ion radiotherapy (experimental treatment) is not relevantly inferior and at least as good as proton radiotherapy (standard treatment) with respect to 5 year LPFS in the treatment of chondrosarcomas. Additionally, we expect less toxicity in the carbon ion treatment arm.Trial RegistrationClinicalTrials.gov identifier: NCT01182753

Skull Base Chondrosarcoma: Is there a Role of Endoscopic Excision?

Abstract Skull base chondrosarcomas are rare and account for approximately 0.15% of all intracranial tumors. Management of these tumors is challenging because of their potential to recur, and their proximity to vital structures such as major vessels and cranial nerves. We describe five patients with chondrosarcoma of the skull base who were managed at our institute between January 2002 and December 2009.

Nasal endoscopic surgical treatment for chondrosarcoma of paranasal sinus and the skull base

To discuss the clinical characteristics and treatments for chondrosarcoma of paranasal sinus and the skull base. The clinical characteristics of chondrosarcoma of paranasal sinus and skull base in 7 patients underwent endoscopic surgeries between 2001 and 2008 were analyzed. Of the patients, 4 men and 3 women. The patients' age ranged from 18 to 47 years, with a median of 31 years. stuffy, nose bleeding, runny, headache, diplopia, eye outreach limited, blurred vision and even blindness. Surgery methods: under nasal endoscopy, after the attachment sites of the tumors to normal tissues were confirmed, the tumors were peeled off along the clear boundary between the tumors and normal tissues, and the potential residual tumor tissues on bones were cleared by a drill. The patients were followed up postoperatively for 24 to 108 months, with a median of 36 months. Five of 7 patients were no recurrence, 2 were alive with tumor. Chondrosarcoma of paranasal sinus and skull base can be treated by nasal endoscopic surgery, with good clinical outcome.

Highlights in surgery of brain tumours

The largest possible and safe removal of the lesion (also malignant) is the best first step of treatment. The extra-axial benign lesions do require the best possible surgical treatment, ideally a complete resection of the lesion without endangering the surrounding neural and/or vascular structures. The most frequent extra-axial intracranial tumours are: pituitary tumours, craniopharyngiomas, central skull base meningiomas, chordomas, chondrosarcomas, trigeminal schwannomas, and schwannomas of the CNs VIII. The outmost surgical reduction of the size of the lesion is highly welcome regardless of whether the tumour is intra-axial and/or extra-axial. Complementary treatment can be much more effective following a successful surgical resection of the lesion. There is no doubt that biological treatments will eventually replace the present day surgical interventions. But at the moment, this is still most effective and the only treatment which can be offered to the patients with intracranial tumours.

Spinal drop metastasis from grade I skull base chondrosarcoma

Chondrosarcoma of the skull base is a rare tumour with a good prognosis following surgical resection. We describe a patient with low-grade chondrosarcoma of the skull base with intradural extramedullary spinal metastases. A 31-year-old female with grade 1 chondrosarcoma involving the cavernous sinus, sphenoid wing and clivus presented at age 19. The tumour was subtotally excised at initial surgery and over the following 4 years, 3 subsequent resections were undertaken for tumour progression followed by proton beam radiotherapy to the residual tumour. The patient re-presented with cervical radiculopathy 7 years later. MRI showed multiple, intradural extramedullary spinal drop metastases. Following surgical excision of the symptomatic lesion, histological diagnosis was confirmed as a mixed hyaline/myxoid grade 1 chondrosarcoma. Patients with skull base chondrosarcoma with intradural extension should have whole spine imaging as part of long-term monitoring to exclude drop metastases, particularly after intradural surgery.

Intensity-Modulated Radiation Therapy for Skull Base Chordomas and Chondrosarcomas: Outcomes in the Image-Guided Era

Intensity-Modulated Radiation Therapy for Skull Base Chordomas and Chondrosarcomas: Outcomes in the Image-Guided Era

Outcomes of a Modular Approach to the Paramedian Plane Using the Expanded Endoscopic Endonasal Approach for Resection of Skull Base Chondrosarcomas

Outcomes of a Modular Approach to the Paramedian Plane Using the Expanded Endoscopic Endonasal Approach for Resection of Skull Base Chondrosarcomas

Traitement des chondrosarcomes de la base du crâne

Skull base chondrosarcomas are rare. Gross total removal is the treatment of choice, but can be difficult depending on the closeness of noble structures. Proton beam therapy can be associated in most cases. Retrospective study of five cases treated in 13 years and study of the literature. Median age of patients was 34 years [28-46]. Cranial nerve palsy was the common clinical presentation. Tumor location was variable but always off midline. Treatment was surgical in all patients with a maximal resection and proton beam therapy associated for two cases. Surgical complications were rare with cranial nerve palsy as the main side effect. Outcomes were good with a median follow-up of 12.4 years [4.3-16.2]. The review of the literature showed that chondrosarcomas of skull base are rare. The best outcome is achieved with total surgical resection. Medical imaging can only give clues to the diagnosis. Pathology is required to obtain a precise immunohistochemistry diagnosis. Multidisciplinary treatment using proton beam therapy and surgical removal enables a good local control (90-100%) at 5 years with good quality-of-life. It is difficult to determine how many cases have been published (around 220 cases in the literature) since many surgical or radiotherapy series included the same patients.

Clinical Outcomes Using the Expanded Endoscopic Endonasal Approach for Resection of Skull Base Chondrosarcomas

Clinical Outcomes Using the Expanded Endoscopic Endonasal Approach for Resection of Skull Base Chondrosarcomas

Skull base chondrosarcoma: a case treated by the CyberKnife system

Skull base chondrosarcomas represent 2% of all chondrosarcomas. They usually have a low grade of malignity and a complete resection may be curative. However, often they are considered irresectable because of the difficult approach and another kind of treatment is needed. We present a 54-year-old male case who, after weeks suffering from diplopia, was diagnosed with skull base chondrosarcoma and treated with the CyberKnife system. A review of radiosurgery techniques for skull base neoplasms is carried out.

Surgical treatment of skull base chondrosarcomas

Skull base chondrosarcomas are rare tumors and individual experience with their management is limited. We present a series of such tumors treated at our institution. Particular attention was paid to their extension pattern, choice of surgical approach, and outcome. Twenty-five patients were operated consecutively over a period of 19 years. Their clinical presentation, radiological features, surgical treatment, early and late treatment outcome, as well as survival rate were analyzed. The most frequent initial symptom was abducent palsy. The typical bone destruction of the petrous apex was found in 83%. Chondrosarcomas extended in 92% to the posterior cranial fossa. Total number of surgeries was 39. The operative approach was tailored to each case. The retrosigmoid approach was used in 30.8%, the pterional in 23%, and the transethmoid in 15.4%. Total tumor removal was achieved in 19 of the surgeries. New neurological deficits immediately after surgery appeared in 33.3%. The perioperative mortality was 0%. The average Karnofsky performance score at last follow-up was 91%, and 5- and 10-year survival rates were 95%. Individually tailored approach in skull base chondrosarcomas allows radical removal with low morbidity rates and without mortality. Given the good long-term prognosis, surgical treatment should not deteriorate significantly patients' quality of life.

Spot Scanning-Based Proton Radiotherapy for Paraspinal and Skull Base Chordomas and Chondrosarcomas: Presentation of the Next-Generation Proton Delivery System and 5-year Clinical Data from the First Spot Scanning-Based Proton Facility

Spot Scanning-Based Proton Radiotherapy for Paraspinal and Skull Base Chordomas and Chondrosarcomas: Presentation of the Next-Generation Proton Delivery System and 5-year Clinical Data from the First Spot Scanning-Based Proton Facility

Anaplastic glioma after high-dose proton–photon radiation treatment for low-grade skull base chondrosarcoma

We report the case of a 63-year-old man who developed an anaplastic oligoastrocytoma of the brain stem and midbrain 13 years after postoperative high-dose proton-photon radiation therapy for a recurrent low-grade chondrosarcoma of the skull base. To our knowledge, this is the first reported case of an anaplastic glioma after proton-photon irradiation.

Recurrent Chondrosarcoma of the Right Skull Base in a Patient with Maffucci Syndrome

Maffucci syndrome is a rare, sporadic disease characterized by the development of multiple enchondromas and subcutaneous hemangiomas. Patients with Maffucci syndrome have a 23-37% risk of malignancy, with chondrosarcomas being the most common. Although the development of a chondrosarcoma in a patient with Maffucci syndrome may be expected, intracranial chondrosarcomas are rare. We present a patient with Maffucci syndrome who was diagnosed with an intracranial chondrosarcoma after presenting with hearing loss and vomiting. After three craniotomies and two recurrences of chondrosarcoma of the right skull base, the patient demonstrated a positive outcome to treatment with external radiation therapy.

Radiosurgery for chordomas and chondrosarcomas of the skull base

Radiosurgery for chordomas and chondrosarcomas of the skull base

Endoscopic Expanded Endonasal Approach for Resection of Skull Base Chondrosarcomas

Endoscopic Expanded Endonasal Approach for Resection of Skull Base Chondrosarcomas

Chondrosarcoma of the Skull Base

Chondrosarcoma of the skull base is an uncommon neoplasm comprising 0.15% of all intracranial tumors and 6% of skull base neoplasms. The outcome of treatment is difficult to assess because the slow growth rate means that there is a long interval before detecting the recurrence. We describe the use of lateral skull base techniques for these lesions and examine the long-term outcomes. The pathological features, radiological findings, and radiotherapy options are also discussed. Retrospective case review with current follow-up where possible. Tertiary referral neurotologic private practice. Eight patients with histologically confirmed skull base chondrosarcoma operated on since 1979. At the time of the surgery, the five women and three men ranged in age from 31 to 63 years, with a mean of 42.9 years. The follow-up ranged from 9 months to 25 years and 5 months. All patients underwent surgical removal; the earliest patient underwent surgical removal via transcochlear and retrosigmoid approaches, and seven via an infratemporal fossa approach, with or without exenteration of the otic capsule. Number of patients with residual and recurrent tumors. In the patient who underwent surgery via the transcochlear approach, recurrence was noted within 3 months, and new exploration using the retrosigmoid approach was performed for the removal of the recurrent lesion. This patient showed no signs of recurrence for 25 years since last surgery. Of the seven patients who underwent surgery via the infratemporal fossa approach, one had residual tumor found at 1 year, but remained alive and well for 21 years after the surgery. The other six had gross total resection and showed no signs of recurrence at the last known follow-up. One patient died as a result of an unrelated cause 18 years after surgery for the chondrosarcoma. The gross total resection of these lesions is possible because of the evolution of lateral skull base techniques and can be routinely accomplished with the infratemporal fossa approach. The improved exposure afforded by this approach seems to have resulted in more complete extirpation of the tumor and a decrease in the recurrence rate.

Skull Base Chondrosarcoma Originating from the Petroclival Junction

To define the presentation of patients with skull base chondrosarcoma, to elucidate surgical strategies, and to identify the role of postoperative radiotherapy. Retrospective review. Tertiary referral center. All patients (n = 33) with skull base chondrosarcoma managed at our institution. The average follow-up time was 7.7 years (range, 0-20 years). Tumor location, presenting symptoms, presence of residual or recurrent tumor, and mortality. The most common tumor location was the petroclival junction (n = 29). Common presenting symptoms were diplopia (48%) and headache (45%). Surgical approaches included retrosigmoid, transtemporal, transfacial, and frontotemporal craniotomies. Biopsy only was performed in four patients, subtotal resection in 19 patients, and total resection in nine patients. Most patients received postoperative radiotherapy (82%). Follow-up revealed residual, stable disease in 28% of patients and recurrent disease in 24% of patients. The mean time to recurrence was 3.0 +/- 2.8 years. The lack of postoperative radiation was significantly correlated with an increased risk of recurrence (odds ratio, 28; p = 0.007) but incomplete tumor resection was not (p = 0.6). Life-table analysis revealed that the 5-year survival rate was 85% and the 10-year survival rate was 77%. Five patients died; four of the deaths attributable to recurrent disease. The characteristic growth pattern of skull base chondrosarcoma is tumor eroding the petroclival junction. Current therapeutic strategy is resection through an extradural subtemporal craniotomy with removal of the petrous apex and clivus. Radical resection of uninvolved structures is often not necessary. Nonetheless, gross total removal is often achievable. Postoperative radiotherapy reduces the chance of tumor recurrence.

The subtemporal and preauricular infratemporal approach for removal of skull base chordomas and chondrosarcomas

The subtemporal and preauricular infratemporal fossa approach provides direct access to the middle cranial fossa, petrous bone, clivus, and cavernous sinus. This approach is ideally suited for use in the resection of chordomas and chondrosarcomas because most are extradural tumors. This approach requires a thorough knowledge of the surgical anatomy of the middle cranial fossa, cavernous sinus, temporal bone, and clivus. A frontotemporal craniotomy is made, and an orbitozygomatic osteotomy is performed to provide a low approach to this area. Dissection is performed extradurally under the temporal lobe to identify the petrous segment of the internal carotid artery. The artery is exposed completely in its entire course through the temporal bone and displaced laterally. The temporal bone and clivus are drilled medial to the petrous internal carotid artery. The surgeon approaches the area in front of the brainstem from an anterolateral direction.

A multidisciplinary team approach to skull base chondrosarcomas.

The authors review their experience with treating skull base chondrosarcomas, which are much rarer than skull base chordomas and differ from them in prognosis and treatment. Seventeen patients (12 male and five female patients) with histologically verified chondrosarcomas were followed up prospectively over a 12-year period. The mean age at presentation was 35.9 years. Most patients presented with cranial nerve palsies. Seven had undergone surgery prior to referral to the authors' unit. All underwent maximum surgical cytoreduction by the most direct surgical approach; only the two patients harboring the mesenchymal variant underwent radiotherapy. One patient died of a pulmonary embolus; the patients harboring mesenchymal chondrosarcomas died at 20 and 36 months, respectively, after treatment. Of the remaining patients, 93% were alive 5 years postsurgery and had a projected 10-year survival rate of 84% (mean survival time 9.3 years). These data emphasize the very slow progression of this tumor compared with skull base chordoma.