Skull base chondrosarcoma

doi:10.1016/j.jocn.2015.10.029

Journal of Clinical Neuroscience

Volume 24, February 2016, Pages 1-5

https://doi.org/10.1016/j.jocn.2015.10.029 Get rights and content

Highlights

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We present two illustrative cases of intracranial chondrosarcoma.
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We review the literature regarding skull base chondrosarcomas with summaries.
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We advocate maximal safe resection, plus radiotherapy for grade II and III tumours.

Abstract

Intracranial chondrosarcoma are rare tumours, accounting for approximately one in 1000 intracranial neoplasms. Although more common in the axial or appendicular skeleton, intracranial tumours present a challenging surgical and oncological problem. Chondrosarcoma have a predilection for the skull base and although commonly slow growing, Grade II and III lesions do occur. We present two illustrative patients from the Royal Melbourne Hospital, Australia, demonstrating dramatically differing presentation and clinical outcome and the diagnostic difficulties that may arise. A review of the literature regarding skull base chondrosarcoma is presented. We summarise the clinical, radiological and histological features. The evidence for surgical resection, radiotherapy and chemotherapy is presented and critically evaluated. Based on the available evidence, we advocate maximal safe resection, followed by radiotherapy for Grade II and III tumours. There is no current role for chemotherapy. Radical excision should not be attempted at the expense of neurological function.

Introduction

Chondrosarcomas are rare malignant tumours composed of cells derived from transformed chondrocytes and account for approximately 20% of skeletal system cancers [1]. They are a heterogeneous group of tumours that have diverse morphological features and clinical behavior. They commonly occur within the pelvis and shoulder or along the superior metaphysial and diaphysial areas of the appendicular skeleton. Only 2% of all chondrosarcomas occur within the skull base [2], accounting for 0.1–0.2% of all intracranial tumours [3]. They are generally very slow growing and erode the bones of the skull base; however higher grade tumours occur, characterised by rapid growth and early metastasis.

Section snippets

Patient 1

J.E. is a 68-year-old woman who presented with headaches over a period of 12 months. The headaches were not consistent with any particular pattern and were not suggestive of raised intracranial pressure. Neurological examination was normal. Neuroimaging revealed a contrast-enhancing lesion eroding through the petrous bone into the cerebellopontine angle (Fig. 1, Fig. 2, Fig. 3). A retrosigmoid craniotomy was performed with the intracranial component being resected and a significant amount of the

Epidemiology/presentation

Skull base chondrosarcoma occurs most commonly between the ages of 40 and 70 years. There may be a slight female predominance (1.1:1 female:male [4]) although many studies demonstrate no sex difference. Presenting symptoms vary according to the precise location of the lesion and the compression and/or displacement of surrounding structures. Symptoms generally include headaches, diplopia, hearing reduction and/or tinnitus, facial sensory disturbances and gait abnormalities. Cranial nerve deficits

Prognosis and treatment

It is important to distinguish chordomas from chondrosarcomas in the skull base as both have similar appearances and radiological characteristics; however, chondrosarcomas carry a much better prognosis. They cannot however be reliably differentiated on the basis of neuroimaging alone. Rosenberg discusses 200 patients with skull base chondrosarcoma of which 74 (37%) were initially referred with a pre-operative diagnosis of chordoma [2].

Bloch and colleagues stated that prognosis is based

Summary

Skull base chondrosarcomas are relatively rare, and it is recommended that treatment occur in specialised centres. Treatment is also best undertaken in a multidisciplinary setting with skull base neurosurgeons and neuro-oncologists. Chondrosarcomas are indolent but invasive tumours that can be at best difficult to treat in view of their location and often extensive nature at the time of diagnosis. Given the available literature and slow growth of most chondrosarcomas, we would argue that whilst

Conflicts of Interest/Disclosures

The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

References (26)

O.G. Bloch et al.
A systematic review of intracranial chondrosarcoma and survival
J Clin Neurosci
(2009)
O. Bloch et al.
Skull base chondrosarcoma: evidence-based treatment paradigms
Neurosurg Clin N Am
(2013)
M.S. Richardson
Pathology of skull base tumors
Otolaryngol Clin North Am
(2001)
A.D. Rapidis et al.
Chondrosarcomas of the skull base: review of the literature and report of two cases
J Craniomaxillofac Surg
(1997)
A.L. Weber et al.
Cartilaginous tumors and chordomas of the cranial base
Otolaryngol Clin North Am
(1995)
D.J. Papachristou et al.
Comparison of allelic losses in chondroblastoma and primary chondrosarcoma of bone and correlation with fluorescence in situ hybridization analysis
Hum Pathol
(2006)
J. Debus et al.
Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base
Int J Radiat Oncol Biol Phys
(2000)
E.B. Hug et al.
Proton radiation therapy for chordomas and chondrosarcomas of the skull base
Neurosurg Clin N Am
(2000)
S.H. Landis et al.
Cancer statistics, 1999
CA Cancer J Clin
(1999)
A.E. Rosenberg et al.
Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma
Am J Surg Pathol
(1999)

A.G. Korten et al.

Intracranial chondrosarcoma: review of the literature and report of 15 cases

J Neurol Neurosurg Psychiatry

(1998)

L. Arpino et al.

Parasellar myxoid chondrosarcoma: a rare variant of cranial chondrosarcoma

J Neurosurg Sci

(2011)

M. Hassounah et al.

Primary cranial and intracranial chondrosarcoma. A survey

Acta Neurochir (Wien)

(1985)

Cited by (37)

Radiomic analysis of multiparametric magnetic resonance imaging for differentiating skull base chordoma and chondrosarcoma
2019, European Journal of Radiology
Citation Excerpt :
However, skull base surgery is highly challenging owing to the inaccessibility of the tumors and their close proximity to vital structures such as the brainstem, cranial nerves, and major vessels. Therefore, some studies have suggested that maximal safe surgical resection followed by adjuvant radiotherapy may result in a relatively good prognosis for chondrosarcoma of the skull base [6,7]. For chordoma, owing to its high recurrence rate [8], gross total resection followed by adjuvant radiotherapy is advocated to achieve better long-term survival [9].
Patients with skull base chordoma and chondrosarcoma have different prognoses and are not readily differentiated preoperatively on imaging. Multiparametric magnetic resonance imaging (MRI) is a routine diagnostic tool that can noninvasively characterize the salient characteristics of tumors. In the present study, we developed and validated a preoperative multiparametric MRI-based radiomic signature for differentiating these tumors.
This retrospective study enrolled 210 patients and consecutively divided them into the primary and validation cohorts. A total of 1941 radiomic features were acquired from preoperative T1-weighted imaging, T2-weighted imaging and contrast-enhanced T1-weighted imaging for each patient. The most discriminative features were selected by minimum-redundancy maximum-relevancy and recursive feature elimination algorithms in the primary cohort. The multiparametric and single-sequence MRI signatures were constructed with the selected features using a support vector machine model in the primary cohort. The ability of the novel radiomic signatures to differentiate chordoma from chondrosarcoma were assessed using receiver operating characteristic curve analysis in the validation cohort.
The multiparametric radiomic signature, which consisted of 11 selected features, reached an area under the receiver operating characteristic curve of 0.9745 and 0.8720 in the primary and validation cohorts, respectively. Moreover, compared with each single-sequence MRI signature, the multiparametric radiomic signature exhibited better classification performance with significant improvement (p <  0.05, Delong’s test) in the primary cohorts.
By combining features from three MRI sequences, the multiparametric radiomics signature can accurately and robustly differentiate skull base chordoma from chondrosarcoma.
Long term outcome of skull-base chondrosarcoma patients treated with high-dose proton therapy with or without conventional radiation therapy
2018, Radiotherapy and Oncology
Skull-base chondrosarcoma (ChSa) is a rare bone tumor and the outcome of patients with this malignancy has been documented only in a limited number of series with a restricted number of patients.
This study was conducted to assess the outcome and prognostic factors of a large cohort of ChSa patients treated with radiotherapy in two proton therapy centers.
From 1996 to 2015, 251 (male, 43.4%) patients (mean age, 42.0 ± 16.2 years) were treated with protons with (n = 135; 53.8%) or without photons (n = 116; 46.2%). Median delivered dose was 70.2 Gy_RBE. Failure-free survival (FFS), overall survival (OS) and CTCAE grade ≥3 toxicity free survival (TFS) were calculated using the Kaplan–Meier method.
After a median follow-up of 88.0 months for surviving patients, local and distant failures were observed in 12 (4.8%) and 4 (1.6%) patients, respectively. Late failures >6 years were observed in 4 (33.3%) patients. The estimated 7-year FFS was 93.1%. Twenty-five (10%) patients died. The estimated 7-year OS was 93.6%. Tumor volume (p = 0.006) and optic pathway compression (p = 0.027) were significantly associated with the risk of treatment failure on univariate analysis. Treatment failure was significantly associated with a higher risk of death (hazard ratio = 126). The estimated 7-year TFS was 84.2%.
The outcome of skull-base ChSa patients treated with high-dose protons with or without photons is excellent, particularly for patients with small tumors with no optic pathway compression. Treatment failure was however associated with a significantly increased risk of death.
Assessing the feasibility of the transmastoid infralabyrinthine approach without decompression of the jugular bulb to the extradural part of the petrous apex and petroclival junction prior to surgery
2024, Acta Neurochirurgica
A systematic review and meta-analysis of radiotherapy and particle beam therapy for skull base chondrosarcoma: TRP-chondrosarcoma 2024
2024, Frontiers in Oncology
CT and MRI Features of Chondrosarcoma in the Mastoid Involving the Facial Nerve in Comparison to Facial Nerve Schwannoma
2024, Current Medical Imaging
Imaging of the Craniocervical Junction: A Pictorial Review
2023, Seminars in Musculoskeletal Radiology

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ReviewSkull base chondrosarcoma

Highlights

Abstract

Introduction

Section snippets

Patient 1

Epidemiology/presentation

Prognosis and treatment

Summary

Conflicts of Interest/Disclosures

J Clin Neurosci

Neurosurg Clin N Am

Otolaryngol Clin North Am

J Craniomaxillofac Surg

Otolaryngol Clin North Am

Hum Pathol

Int J Radiat Oncol Biol Phys

Neurosurg Clin N Am

Cancer statistics, 1999

CA Cancer J Clin

Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma

Am J Surg Pathol