Intracranial Chondrosarcoma Research Articles

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review.

Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection. A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described. The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2years (range 2months to 18years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired. IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis. SafeMaximal resection followed by radiotherapy is the preferred treatment strategy.

Survival Analysis of Intracranial Chondrosarcoma: Insights from a Systematic Review

Survival Analysis of Intracranial Chondrosarcoma: Insights from a Systematic Review

A multicenter retrospective analysis of clinical outcomes of intracranial chondrosarcoma in 26 patients

Intracranial chondrosarcoma is a rare tumor with limited reports. We reviewed the clinical outcomes, imaging findings, and pathological characteristics at three centers to improve the diagnosis and treatment of intracranial chondrosarcoma. We retrospectively analyzed 26 patients with intracranial chondrosarcoma who had undergone surgical treatment at Tangdu Hospital of Air Force Military Medical University, Mianyang Central Hospital, and Nanchong Central Hospital from January 2010 to July 2022. Clinical manifestations, imaging features, surgical treatment, prognosis, and overall survival (OS) were analyzed. All 26 chondrosarcomas were located at the skull base. Gross total resection (GTR), subtotal resection (STR), and partial resection (PR) were performed in 14, 10, and 2 cases, respectively. Four cases underwent endoscopic transnasal surgery, while the remaining cases underwent craniotomy. The clinical symptoms were evaluated 1 week after surgery, and 15 cases were relieved to varying degrees. Postoperative complications included pulmonary infection, subcutaneous hydrops, dysphagia and choking, facial numbness, abducens paralysis, and intracranial infection (ICI). Fifteen cases received postoperative adjuvant radiotherapy. Seven cases showed recurrence: two with PR, four with STR, and one with GTR. Six cases received reoperation or radiotherapy after tumor progression, and one untreated patient died 5 months after tumor recurrence. The extent of tumor resection (HR 21.74, 95% CI 1.25–376.6, P = 0.03) and pathological grading (HR 131.99, 95% CI 4.05–4300.5, P = 0.006) were associated with improved OS. We presented our experience in the treatment of intracranial chondrosarcoma at three centers in the past 12 years. Intracranial chondrosarcoma lacked typical imaging features and are difficult to differentiate from other skull base lesions. Maximum extent of tumor resection with minimal injury to neurological function remains the most important treatment strategy. The extent of surgical resection and pathological grading were found to be predictors for OS.

Challenges with Adjuvant Radiation for Intracranial Chondrosarcoma in Pregnancy

Background Managing a brain tumor during pregnancy is a highly confusing and challenging situation, complicated by several technical, medical, ethical, and sociocultural concerns. The interests of the mother and child are often pitted against each other, for which legal opinion may occasionally be needed. Case Report We present the report of a young lady with intracranial well-differentiated chondrosarcoma who was determined to be pregnant in the immediate postoperative period. We discuss the management of challenges and dilemmas in devising optimum therapy, and the modifications and care required at each step to help safeguard maternal and fetal health. Risks with therapeutic radiation and measures to assess and pre-empt fetal doses that may assist decision-making are also discussed. Conclusion Radiation therapy during pregnancy is challenging and requires multidisciplinary involvement and psychosocial support for the patient and family.

Papilledema associated unilateral juxtafoveal choroidal neovascularization in a patient of intracranial chondrosarcoma

Skull base chondrosarcomas are rare intracranial neoplasms. Although peripapillary choroidal neovascularization (CNV) has infrequently been noted in papilledema secondary to other pathologies, juxtafoveal CNV following intracranial chondrosarcomas have never been reported earlier. We report a case of a 50-year-old lady with operated intracranial chondrosarcoma who presented with progressive diminution of vision in the right eye. Detailed ophthalmic evaluation and ancillary investigations confirmed the diagnosis of papilledema associated juxtafoveal CNV in the right eye, and she received two intravitreal bevacizumab injections with guarded outcome. We have also described the probable differentials of the current case and highlighted the need for a high index of suspicion among neurologists as well as ophthalmologists for this rare but vision-threatening complication.

Skull base chondrosarcoma presenting with parenchymal hemorrhage: A case report

Chondrosarcoma (CS) is a locally invasive, slow growing malignant neoplasm. Intracranial chondrosarcoma are extradural osseous lesions and intradural location with intratumoral hemorrhage is very rare. We report a rare case of 65-year-old male presenting with a small mass in left posterior skull base and later, suddenly deteriorating due to large cerebellar hemorrhage. A brief review of literature of such cases is also presented.

Assessment of Multimodality Imaging for Target Definition of Intracranial Chondrosarcomas

Assessment of Multimodality Imaging for Target Definition of Intracranial Chondrosarcomas

The Differences Between Intracranial Mesenchymal Chondrosarcoma and Conventional Chondrosarcoma in Clinical Features and Outcomes

To report differences in clinical features and outcomes between intracranial mesenchymal chondrosarcoma (MCS) and conventional chondrosarcoma (CCS). Clinical data of patients with primary intracranial MCS and CCS were retrospectively extracted and analyzed to compare differences between MCS and CCS. Seventy-four patients with intracranial chondrosarcoma (61 cases with MCS and 13 cases with CCS) were included. Compared with patients with CCS, patients with MCS presented at a younger mean age (21.1 years vs. 34.5 years, P < 0.001) and had a poor mean preoperative Karnofsky performance scale score (64.6 vs. 77.8, P= 0.014). Compared with CCS, MCS was less often located in the skull base (38.5% vs. 96.7%, P < 0.001) and had a larger tumor volume (87.8 cm3 vs. 26.7 cm3, P < 0.001). Rates of gross total resection in MCS and CCS subgroups were 41.1% (n= 25) and 46.2% (n= 6), respectively; rates of adjuvant radiotherapy postoperatively were 44.2% (n= 27) and 46.2% (n= 6), respectively. After mean follow-up of 41.7 months, 1-year, 3-year, and 5-year overall survival and progression-free survival of MCS were significantly shorter than overall survival and progression-free survival of CCS. Multivariate analysis revealed that tumor pathology and extent of surgery were independent predictors for tumor recurrence. Clinical features of MCS are quite different from CCS. Treatment strategies used for CCS do not yield satisfactory outcomes for MCS. Treatment of MCS should be aggressive and individualized.

Primary Intracranial Mesenchymal Chondrosarcoma: A Case Report

Mesenchymal chondrosarcoma is a rare malignancy characterized by a biphasic pattern of small undifferentiated round cells intermixed with island of well-differentiated cartilaginous matrix. Although this type of tumor exhibits widespread distribution in the body, intracranial mesenchymal chondrosarcoma is rare. Radical excision of intracranial mesenchymal chondrosarcoma is important for obtaining a positive prognosis but the effectiveness of chemotherapy and radiotherapy remains uncertain. An 18-year-old patient presented with generalized seizure with magnetic resonance imaging showing a large dura-attached solid mass in the frontal convexity, which was presumptively diagnosed as a meningioma. Gross total resection of tumor was achieved without postoperative deficit. The final histological diagnosis was extra-skeletal mesenchymal chondrosarcoma, grade I. There was no adjuvant treatment after surgery. Five years after surgery, the prognosis was good after gross total resection only; however, long-term follow-up is necessary in the future. J Med Cases. 2018;9(5):135-138 doi: https://doi.org/10.14740/jmc3040w

Proposed Treatment Paradigm for Intracranial Chondrosarcomas Based on Multidisciplinary Coordination

Objectives There was no consensus regarding the treatment of intracranial chondrosarcoma (CSA). The study aimed to evaluate the adverse factors for progression-free survival (PFS) and overall survival (OS), and propose a treatment strategy for CSA.

Primary Intracranial Myxoid Chondrosarcoma: A case report

Objective: Intracranial extraskeletal myxoid chondrosarcoma is a rare tumor; up to date, only ten cases have been reported. Case report: We present a case report of a 65-year-old female admitted for right side hemiplegia of gradual onset and progressive course over three weeks. Magnetic resonance imaging (MRI) revealed left intra-axial parieto-occipital space occupying lesion, hypodense with ring enhancement surrounded by diffuse edema. The patient underwent subtotal resection of the mass using sonar guided craniotomy. The histopathological and immunohistochemical evaluation confirmed a diagnosis of extraskeletal myxoid chondrosarcoma. One month after initial discharge, the patient underwent radiotherapy. A follow-up examination 6 months after surgery revealed that the patient was deteriorated and died. Conclusion: intracranial extraskeletal myxoid chondrosarcoma is a rare malignant cartilaginous tumor. Pathological diagnosis is the gold standard and radical excision is the standard treatment

Proposed Treatment Paradigm for Intracranial Chondrosarcomas Based on Multidisciplinary Coordination.

There was no consensus regarding the treatment of intracranial chondrosarcoma (CSA). The study aimed to evaluate the adverse factors for progression-free survival (PFS) and overall survival (OS) and to propose a treatment strategy for CSA. The clinical chart and radiographic data of 106 consecutive cases (mesenchymal and conventional CSA in 18 and 88 patients, respectively) of surgically treated CSAs were retrospectively reviewed. Gross total resection was achieved in 43 patients (40.6%), and adjuvant radiotherapy was administered in 45 patients. After a mean follow-up duration of 47.8 months, 38 patients (37.3%) experienced recurrence. PFS and disease-specific OS at 5 years was 57.7% and 74.4%. Independent adverse factors for PFS were previous surgery (hazard ratio [HR] 2.261; P= 0.028), increased lesion size (HR, 1.298; P= 0.026), extent of surgical resection (HR, 3.226; P < 0.001), malignant pathology (HR, 2.018; P= 0.003), and postoperative radiotherapy (HR, 3.246; P= 0.001). The stereotactic radiosurgery subgroup presented best 5-year PFS of 88.9%, and a linear accelerator prolonged the mean PFS time (57.0 months) compared with no radiation (38.1 months). In the incomplete resection subgroup (n=63), radiotherapy significantly benefited tumor control (HR, 2.101; P= 0.016). Extent of surgical resection (HR, 1.797; P= 0.026) and malignant disease (HR, 1.717; P=0.030) were associated with OS. Intracranial CSAs were not completely amendable by surgery alone. Gross total resection as far as possible plus radiation were necessary for mesenchymal CSA and conventional CSA with active growth or residual tumor. Stereotactic radiosurgery was an alternative if proton therapy was unavailable. A future study with a large cohort is required to verify our findings.

Primary Intracranial Myxoid Chondrosarcoma: A case report

Objective: Intracranial extraskeletal myxoid chondrosarcoma is a rare tumor; up to date, only ten cases have been reported. Case report: We present a case report of a 65-year-old female admitted for right side hemiplegia of gradual onset and progressive course over three weeks. Magnetic resonance imaging (MRI) revealed left intra-axial parieto-occipital space occupying lesion, hypodense with ring enhancement surrounded by diffuse edema. The patient underwent subtotal resection of the mass using sonar guided craniotomy. The histopathological and immunohistochemical evaluation confirmed a diagnosis of extraskeletal myxoid chondrosarcoma. One month after initial discharge, the patient underwent radiotherapy. A follow-up examination 6 months after surgery revealed that the patient was deteriorated and died. Conclusion: intracranial extraskeletal myxoid chondrosarcoma is a rare malignant cartilaginous tumor. Pathological diagnosis is the gold standard and radical excision is the standard treatment

Botulinum Toxin Type A Injection for Neuropathic Pain in a Patient With a Brain Tumor: A Case Report.

Neuropathic pain is usually managed pharmacologically, rather than with botulinum toxin type A (BTX-A). However, medications commonly fail to relieve pain effectively or have intolerable side effects. We present the case of a 62-year-old man diagnosed with an intracranial chondrosarcoma, which was removed surgically and treated with radiation therapy. He suffered from neuropathic pain despite combined pharmacological therapy with gabapentin, amitriptyline, tramadol, diazepam, and duloxetine because of adverse effects. BTX-A (100 units) was injected subcutaneously in the most painful area in the posterior left thigh. Immediately after the injection, his pain decreased significantly from 6/10 to 2/10 on a visual analogue scale. Pain relief lasted for 12 weeks. This case report describes intractable neuropathic pain caused by a brain tumor that was treated with subcutaneous BTX-A, which is a useful addition for the management of neuropathic pain related to a brain tumor.

Primary intracranial chondrosarcoma: A report of 2 cases and literature review

Primary intracranial chondrosarcoma: A report of 2 cases and literature review

Classical intracranial chondrosarcoma: A case report.

Intracranial chondrosarcoma is a rare malignant cartilage-forming tumor, with only a small number of cases in the posterior cranial fossa reported previously. The present study reports the case of a 40-year-old male patient who was admitted to Tianjin Huanhu Hospital with a progressive headache and dizziness that had lasted for 2 years. Physical and neurological examinations were normal. Radiography of the skull identified an opaque lesion in the left frontal region of the brain. Cranial computed tomography and magnetic resonance imaging revealed a lesion with calcification and homogenous contrast enhancement in the left frontal region. Subsequently, the patient underwent bicoronal craniotomy and gross total resection of the tumor. Pathological examination confirmed the diagnosis of classical intracranial chondrosarcoma. The patient was discharged 10 days after surgery, with no neurological deficit. One month after initial discharge, the patient underwent γ-knife treatment. A follow-up examination 9 months after surgery revealed that the patient was still alive and had returned to work, with no obvious symptoms or evidence of recurrence.

Primary Intracranial Myxoid Chondrosarcoma

Primary Intracranial Myxoid Chondrosarcoma

Falcine and parasagittal chondrosarcomas

Intracranial chondrosarcomas are primary cartilaginous neoplasms that represent 6% of all skull base tumors. Intracranial extraskeletal chondrosarcomas are more rare, often arising from the meninges at the falx, tentorium, or cerebral convexity. They are generally characterized as classical or mesenchymal, with the latter associated with worse outcomes. We present our institutional series of falcine and parasagittal chondrosarcomas along with a review of the literature. Although skull base chondrosarcomas pose significant challenges due to their invasive biology and proximity to vital brainstem structures and cranial nerves, intracranial extraskeletal chondrosarcomas are generally associated with a good prognosis. Our review of the literature identified 29 patients with falcine and parasagittal chondrosarcomas. There were six recurrences, five among patients with the mesenchymal subtype and one in a patient with the classical subtype. All deaths occurred in patients with the mesenchymal subtype. Management of skull base chondrosarcomas is controversial but extraskeletal intracranial tumors can generally be managed by surgical resection alone. Treatment should be tailored to the biology of the tumor, with radiation therapy reserved for patients with the mesenchymal subtype.

High-grade intracranial chondrosarcoma presenting with haemorrhage

Chondrosarcomas are rare sarcomas that produce malignant cartilage, infrequently arising as a primary intracranial tumour. We present a patient with intracranial chondrosarcoma with intratumoural haemorrhage arising in an unusual location and with unusual imaging findings. A 46-year-old man presented with headache, nausea, and vomiting over the previous 24hours. Physical and neurological examinations were normal. Cranial CT scans and MRI revealed a large right pre-frontal (subdural) and interhemispheric heterogeneous density associated with a frontal, partially calcified mass and midline shift. An awake craniotomy was performed. With the intra-operative quick section favouring subdural hematoma, the lesion was subtotally resected. Follow-up imaging confirmed residual mass. Pathology examination revealed a high-grade malignant neoplasm with chondroid differentiation, diagnosed as conventional Grade III chondrosarcoma. The patient was referred to oncology for follow-up and radiation therapy. Intracranial chondrosarcoma was first reported in 1899, and since then continues to be an extremely rare malignancy of the brain. These tumours commonly present as extra-axial masses, originating from the skull base, and produce symptoms due to progressive enlargement and compression of local structures. Unusual presentations of these tumours, such as vascularity, intratumoural haemorrhage, and intra-axial location, may complicate pre-surgical decision making by altering the provisional diagnosis prior to intervention. This patient emphasises the importance of careful analysis and incorporation of imaging findings into surgical decision making. Specific imaging characteristics that, in such unusual situations, are suggestive of chondrosarcoma should motivate an aggressive surgical approach to optimise adjuvant interventions.

Proton Beam Radiotherapy: Report of the First Patient Treated at the Centro NAzionale di Adroterapia Oncologica (CNAO)

Proton beam radiotherapy, an innovative treatment modality, allows delivery of high radiation doses to the target while sparing surrounding healthy structures. The Centro Nazionale di Adroterapia Oncologica (CNAO), equipped with a synchrotron and capable of using both protons and ions, initiated its clinical activity in September 2011. The first treatment of a skull base tumor with protons is reported here. The case of a 26-year-old man with an intracranial low-grade chondrosarcoma of the right petroclival junction is discussed with emphasis on technical and clinical details. Two previous surgical interventions had achieved partial removal of the tumor and the patient was treated with protons for residual disease. The prescribed dose was 70 GyE in 35 fractions of 2 GyE. Treatment was completed with minimal acute toxicity consisting of grade 1 alopecia and nausea. Nine months after treatment the disease is locally controlled. Use of high-energy protons at CNAO is a safe and effective means of treating a tumor located near critical normal structures.