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Central nervous system mesenchymal chondrosarcoma. Salvati M,Caroli E,Frati A,Piccirilli M,Agrillo A,Brogna C,Occhiogrosso G,Giangaspero F Journal of experimental & clinical cancer research : CR Central nervous system mesenchymal chondrosarcomas are rare malignant tumors that constitute a separate entity from the classical chondrosarcoma and myxoid variant. Clinical behaviour of central nervous system chondrosarcomas is still unknown. We describe two rare examples of intracranial mesenchymal chondrosarcoma with a review of the literature, in an attempt to clarify the clinical characteristics, prognosis and treatment of choice of these unusual tumors. Among the 55 reported cases, 23 had postoperative radiotherapy. Although there is no statistical significance according to the Log-Rank test (p=0.7), the patients treated with radiation therapy seem to have a better chance of survival. Patients who had adjuvant chemotherapy (only 5) showed survival times similar to those patients who had none. Although clinical behaviour of central nervous system chondrosarcomas remains to be defined, data from our series as well as literature show that radical removal is the best therapeutic choice. In addition, patients treated with postoperative radiotherapy seem to show a trend toward increased survival.
Proposed Treatment Paradigm for Intracranial Chondrosarcomas Based on Multidisciplinary Coordination. Li Da,Weng Jian-Cong,Zhang Gui-Jun,Hao Shu-Yu,Tang Jie,Zhang Li-Wei,Wang Liang,Wu Zhen,Jia Wang,Zhang Jun-Ting World neurosurgery 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 to propose a treatment strategy for CSA. METHODS: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. RESULTS: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. CONCLUSIONS: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. 10.1016/j.wneu.2017.10.013
Intracranial Metastasis of Extracranial Chondrosarcoma: Systematic Review With Illustrative Case. Brain tumor research and treatment BACKGROUND:Cerebral chondrosarcoma metastases are rare and aggressive neoplasms. The rarity of presentation has precluded rigorous analysis of diagnosis, risk factors, treatment, and survival. We analyzed every reported case through exhaustive literature review. We further present the first case with Maffucci syndrome. METHODS:Three databases, PubMed, Embase, and Google Scholar, and crossed references were queried for cerebral chondrosarcoma metastases. Extracted variables included demographics, risk factors, tumor characteristics, interventions, and outcomes. Univariate and multivariate analyses were performed. RESULTS:Fifty-six patients were included from 1,489 literature results. The average age at brain metastasis was 46.6±17.6 years and occurred at a median of 24±2.8 months from primary diagnosis. Primary tumor histology (dedifferentiated 5.0±1.5 months, mesenchymal 24±3.0 months, conventional 41±7.4 months, <0.05) and grade (low grade 54±16.7 months vs. high-grade 10±6.4 months, <0.001) correlated with time interval until brain metastasis. A multiple enchondromatosis syndrome occurred in 13.2% of cases. At time of brain metastases diagnosis, extracranial metastases were identified in 76.2% of cases. Median survival after the development of brain metastasis was 2.0±0.78 months with a 1-year survival of 10.0%. On regression analysis, surgery reduced brain metastasis mortality risk and radiation trended towards reduced mortality risk (surgery: hazard ratio [HR] 0.22, 95% confidence interval [CI] 0.064-0.763, =0.017; radiation: HR 0.31, 95% CI 0.091-1.072, =0.064). CONCLUSION:We present a systematic review of cerebral chondrosarcoma metastases. Primary tumor histology and grade correlate with time until cerebral metastasis. Following cerebral metastasis, these tumors have poor prognosis and modestly benefit from surgery. 10.14791/btrt.2023.0003
Primary central nervous system sarcomas in adults: A systematic review. Clinical neurology and neurosurgery BACKGROUND:Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics, treatment strategies, and survival are scarce. We comprehensively reviewed management strategies and outcomes of primary CNS sarcomas in adults. METHODS:PubMed, Scopus, and Cochrane were search following the PRISMA guidelines to include studies on primary CNS sarcomas in adults. Clinical features, management strategies, and survival were analyzed. RESULTS:We included 9 studies comprising 78 patients. Primary CNS sarcomas were mostly intracranial (87.2%), frequently located in the parietal (17.9%), frontal (14.1%), and temporal (14.1%) lobes. Spinal CNS sarcomas were found in 10 patients (12.8%). The most common tumor histology were fibrosarcoma (16.7%), intracranial synovial sarcoma (12.8%), extraosseous mesenchymal chondrosarcoma (11.5%), perivascular sarcoma (11.5%), reticulum cell sarcoma (11.5%), and myeloid sarcoma (9%). Partial resection (57.7%) was preferred over complete resection (42.3%), and 43 patients (55.1%) received adjuvant treatments: radiotherapy (51.3%) and/or systemic chemotherapy (20.5%). 21 patients experienced CNS sarcomas recurrences, with a median progression-free survival of 9 months (range, 4-48). At last follow-up, 60 patients (76.9%) were dead, with a median overall survival of 9 months (0.1-396). Overall survival was significantly longer in patients with fibrosarcoma (p = 0.001). CONCLUSION:Surgical resection coupled with adjuvant chemotherapy or radiation has historically been the cornerstone treatment for CNS sarcoma but showed poor local control and dismal survival. A better understanding of the CNS sarcoma microenvironment may favor the development of tailored strategies aimed at improving survival. 10.1016/j.clineuro.2022.107127
MESENCHYMAL CHONDROSARCOMA. DOWLING E A The Journal of bone and joint surgery. American volume
A mesenchymal chondrosarcoma with aberrant nuclear expression of STAT6: a potential diagnostic pitfall. Broggi Giuseppe,Mazzucchelli Manuel,Covello Renato,Casini Beatrice,Barbagallo Giuseppe Maria Vincenzo,Salvatorelli Lucia,Magro Gaetano Pathology, research and practice STAT6 is usually considered to be a very sensitive and specific immunomarker for diagnosis of solitary fibrous tumor (SFT), being a surrogate of the NAB2-STAT6 fusion gene identified in most cases of this tumor. STAT6 expression has also been reported in rare cases of other soft tissue tumors, such as low-grade fibromyxoid sarcoma, myxoid/round cell liposarcoma, dedifferentiated liposarcoma and deep fibrous histiocytoma. The aim of this study was to report, for the first time, a case of mesenchymal chondrosarcoma showing diffuse aberrant immunohistochemical expression of STAT6. Molecular biology, showing the HEY1-NCOA2 fusion gene, was crucial to rule out SFT. 10.1016/j.prp.2022.153803
The Differences Between Intracranial Mesenchymal Chondrosarcoma and Conventional Chondrosarcoma in Clinical Features and Outcomes. Ma Xiujian,Meng Guolu,Wang Ke,Li Da,Wang Liang,Li Huan,Zhang Junting,Zhang Liwei,Wu Zhen World neurosurgery OBJECTIVE:To report differences in clinical features and outcomes between intracranial mesenchymal chondrosarcoma (MCS) and conventional chondrosarcoma (CCS). METHODS:Clinical data of patients with primary intracranial MCS and CCS were retrospectively extracted and analyzed to compare differences between MCS and CCS. RESULTS: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 cm vs. 26.7 cm, 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. CONCLUSIONS: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. 10.1016/j.wneu.2018.10.230
Intracranial Mesenchymal Chondrosarcoma: Report of 16 Cases. Wang Ke,Ma Xiu-Jian,Guo Teng-Xian,Wang Liang,Li Da,Hao Shu-Yu,Jia Gui-Jun,Jia Wang,Zhang Jun-Ting,Zhang Li-Wei,Wu Zhen World neurosurgery OBJECTIVES:Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS. METHODS:Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. RESULTS:This study included 11 male and 5 female patients with a mean age of 22.9 ± 14.4 years. The most common presentations were headache (n = 10; 62.5%), followed by cranial deficits (n = 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients. CONCLUSIONS:Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments. 10.1016/j.wneu.2018.05.069
Metastasis from Intracranial Mesenchymal Chondrosarcoma: Report of a Rare Case. Dutta Gautam,Singh Daljit,Saran Ravindra Kumar,Singh Hukum,Srivastava Arvind Kumar,Jagetia Anita Journal of neurological surgery. Part A, Central European neurosurgery Chondrosarcoma is a rare malignant tumor originating from cartilaginous tissue with a tendency to localize in the epiphysis of long and pelvic bones. Only 7% of all chondrosarcomas originate in the craniocervical region. Metastasis from intracranial chondrosarcoma is extremely rare with only two previously reported cases. We report on a young patient with intracranial chondrosarcoma who presented with extracranial metastasis 2 years after surgical excision of the primary lesion. Gross total excision combined with radiotherapy so far has led to a favorable outcome. 10.1055/s-0038-1655733
Mesenchymal chondrosarcoma: a clinicopathologic and flow cytometric study of 13 cases presenting in the central nervous system. Rushing E J,Armonda R A,Ansari Q,Mena H Cancer BACKGROUND:Mesenchymal chondrosarcomas arising in the central nervous system are extremely rare. Morphologic features have not been found to correlate reliably with prognosis. METHODS:Eight intracranial and five intraspinal mesenchymal chondrosarcomas were reviewed with regard to location, treatment, and long term follow-up data. The histopathologic and immunohistochemical results, including Ki-67 nuclear staining frequency, were critically reviewed, and deoxyribonucleic acid content was analyzed by flow cytometry. RESULTS:Microscopically, all 13 cases were remarkably similar. Immunoreactivity in the small cell component included vimentin in 100% and cytokeratin and glial fibrillary acidic protein in 25% of cases. S-100 immunoreactivity was noted in the cartilaginous component of 100% of cases, and in rare cells in the small cell component along the interface. Flow cytometry of the eight tumors studied revealed a diploid pattern in six, aneuploidy in two, and a wide range of S-phase fractions (0-36.5%). CONCLUSIONS:Review of the literature and the findings of the current series indicates that mesenchymal chondrosarcomas presenting in the brain and spinal cord pursue a progressive course that correlates most reliably with extent of surgical resection. This limited retrospective study also suggests that survival may be shorter for those patients with a high S-phase fraction and a high Ki-67 staining frequency. 10.1002/(SICI)1097-0142(19960501)77:9<1884::AID-CNCR19>3.0.CO;2-W