The Experts below are selected from a list of 1578 Experts worldwide ranked by ideXlab platform
Hideaki Murata - One of the best experts on this subject based on the ideXlab platform.
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Intracranial extraskeletal Myxoid Chondrosarcoma with special reference to lamellar inclusions in the rough endoplasmic reticulum
Acta Neuropathologica, 1993Co-Authors: Kazufumi Sato, Toshihiko Kubota, Kazuhiko Yoshida, Hideaki MurataAbstract:An extraskeletal Myxoid Chondrosarcoma in the pineal region was studied by light and electron microscopy. The immunohistochemical positivity for S-100 protein, vimentin and collagen type II favored a chondrocytic origin of the tumor. In addition to the well-described ultrastructural features suggestive of a cartilagenous nature, this tumor had unusual lamellar inclusions in the rough endoplasmic reticulum. To the best of our knowledge, this is the first report of these special inclusion bodies in a Myxoid Chondrosarcoma.
Silvia Stacchiotti - One of the best experts on this subject based on the ideXlab platform.
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International single-arm phase II trial of pazopanib in advanced extraskeletal Myxoid Chondrosarcoma: A Collaborative Spanish (GEIS), Italian (ISG) and French (FSG) Sarcoma Groups study.
Journal of Clinical Oncology, 2017Co-Authors: Silvia Stacchiotti, Stefano Ferrari, Andrés Redondo, Emanuela Palmerini, Nadia Hindi, M. Angeles Vaz, Anna Maria Frezza, Antonio Gutierrez, Antonio Lopez-pousa, Giovanni GrignaniAbstract:11062Background: Extraskeletal Myxoid Chondrosarcoma (EMC) is an exceedingly rare sarcoma, marked by a specific translocation involving the gene NR4A3 that can be rearranged with different partners...
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activity of sunitinib in extraskeletal Myxoid Chondrosarcoma
European Journal of Cancer, 2014Co-Authors: Silvia Stacchiotti, Gianpaolo Dagrada, Tiziana Negri, Carlo Morosi, Alessandro Gronchi, Annalisa Astolfi, Maria Abbondanza Pantaleo, Valentina Indio, A Dei P Tos, Chiara ColomboAbstract:Abstract Background Extraskeletal Myxoid Chondrosarcoma (EMC) is a rare soft tissue sarcoma, marked by NR4A3 rearrangement. Herein we report on the activity of sunitinib in a series of 10 patients, strengthening what initially observed in two cases. Patients and methods From July 2011, 10 patients with progressive metastatic translocated EMC have been consecutively treated with sunitinib 37.5 mg/day, on a named-use basis. In an attempt to interpret the activity of sunitinib in EMC, genotype/phenotype correlations were carried out by fluorescence in situ hybridization (FISH) analyses. Moreover, transcriptome, immunohistochemical and biochemical analyses of a limited set of samples were performed focusing on some putative targets of sunitinib. Results Eight of 10 patients are still on therapy. Six patients had a Response Evaluation Criteria in Solid Tumours (RECIST) partial response (PR), two were stable, two progressed. Positron emission tomography (PET) was consistent in 6/6 evaluable cases. One patient underwent surgery after sunitinib, with evidence of a pathologic response. At a median follow-up of 8.5 months (range 2–28), no secondary resistance was detected. Median progression free survival (PFS) has not been reached. Interestingly, all responsive cases turned out to express the typical EWSR1–NR4A3 fusion, while refractory cases carried the alternative TAF15–NR4A3 fusion. Among putative sunitinib targets, only RET was expressed and activated in analysed samples. Conclusions This report confirms the therapeutic activity of sunitinib in EMC. Genotype/phenotype analyses support a correlation between response and EWSR1–NR4A3 fusion. Involvement of RET deserves further investigation.
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anthracycline based chemotherapy in extraskeletal Myxoid Chondrosarcoma a retrospective study
Clinical sarcoma research, 2013Co-Authors: Silvia Stacchiotti, Gianpaolo Dagrada, Roberta Sanfilippo, Tiziana Negri, Isabella Vittimberga, Stefano Ferrari, Federica Grosso, Gaetano Apice, Marco Tricomi, Chiara ColomboAbstract:Background Extraskeletal Myxoid Chondrosarcoma (EMC) is a rare subgroup within soft tissue sarcomas. Its sensitivity to chemotherapy is reported to be low.
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Anthracycline-based chemotherapy in extraskeletal Myxoid Chondrosarcoma: a retrospective study
'Springer Science and Business Media LLC', 2013Co-Authors: Silvia Stacchiotti, Gianpaolo Dagrada, Roberta Sanfilippo, Tiziana Negri, Isabella Vittimberga, Stefano Ferrari, Federica Grosso, Gaetano Apice, Marco Tricomi, Chiara ColomboAbstract:BACKGROUND: Extraskeletal Myxoid Chondrosarcoma (EMC) is a rare subgroup within soft tissue sarcomas. Its sensitivity to chemotherapy is reported to be low. METHODS: We retrospectively reviewed a series of 11 EMC patients treated as from 2001 within the Italian Rare Cancer Network (RCN) with anthracycline-based chemotherapy. Pathologic diagnosis was centrally reviewed in all cases and confirmed by the presence of the specific chromosomal rearrangements, involving the NR4A3 gene locus on chromosome 9. RESULTS: Eleven patients treated with anthracycline-based chemotherapy were included (M/F: 9/2 - mean age: 52 years - site of primary: lower limb/other\u2009=\u20099/2 - metastatic\u2009=\u200911 - front line/ further line\u2009=\u200910/1 - anthracycline as single agent/ combined with ifosfamide\u2009=\u20091/10). Ten patients are evaluable for response. Overall, best response according to RECIST was: partial response (PR)\u2009=\u20094 (40 %), stable disease (SD)\u2009=\u20093, progressive disease (PD)\u2009=\u20093 cases. Median PFS was 8 (range 2-10) months. CONCLUSIONS: By contrast to what reported so far, anthracycline-based chemotherapy is active in a distinct proportion of EMC patients
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extraskeletal Myxoid Chondrosarcoma tumor response to sunitinib
Clinical sarcoma research, 2012Co-Authors: Silvia Stacchiotti, Gianpaolo Dagrada, Tiziana Negri, Carlo Morosi, Antonella Romanini, Silvana Pilotti, Alessandro Gronchi, Paolo G CasaliAbstract:Background Extraskeletal Myxoid Chondrosarcoma (EMCS) is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein EWSR1-CHN (the latter even called NR4A3 or TEC). EMCS is marked by >40% incidence of metastases in spite of its indolent behaviour. It is generally resistant to conventional chemotherapy, and, to the best of our knowledge, no data have been reported to date about the activity of tirosin-kinase inhibitor (TKI) in this tumor. We report on two consecutive patients carrying an advanced EMCS treated with sunitinib.
Kazufumi Sato - One of the best experts on this subject based on the ideXlab platform.
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Intracranial extraskeletal Myxoid Chondrosarcoma with special reference to lamellar inclusions in the rough endoplasmic reticulum
Acta Neuropathologica, 1993Co-Authors: Kazufumi Sato, Toshihiko Kubota, Kazuhiko Yoshida, Hideaki MurataAbstract:An extraskeletal Myxoid Chondrosarcoma in the pineal region was studied by light and electron microscopy. The immunohistochemical positivity for S-100 protein, vimentin and collagen type II favored a chondrocytic origin of the tumor. In addition to the well-described ultrastructural features suggestive of a cartilagenous nature, this tumor had unusual lamellar inclusions in the rough endoplasmic reticulum. To the best of our knowledge, this is the first report of these special inclusion bodies in a Myxoid Chondrosarcoma.
Michael J Wagner - One of the best experts on this subject based on the ideXlab platform.
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long term outcomes for extraskeletal Myxoid Chondrosarcoma a seer database analysis
Cancer Epidemiology Biomarkers & Prevention, 2020Co-Authors: Michael J Wagner, Bonny Chau, Elizabeth T Loggers, Seth M Pollack, Teresa S Kim, Edward Y Kim, Matthew J ThompsonAbstract:Background: Extraskeletal Myxoid Chondrosarcoma (EMCS) is a rare tumor that typically has an indolent course but high rate of recurrence. We queried the Surveillance, Epidemiology, and End Results (SEER) database to assess factors associated with metastasis, treatment, and survival. Methods: We queried the SEER 1973–2016 database for patients with Myxoid Chondrosarcoma (ICD-O-3: 9231/3). Kaplan–Meier analyses and Cox proportional hazard models assessed effects on overall survival (OS) of demographics and clinical characteristics. Logistic regression assessed associations between tumor location and distant disease. Primary analysis was a complete case analysis; multiple imputation (MI) was used in a sensitivity analysis. Results: Locoregional disease (LRD) was found in 373 (85%) of patients. In univariate analysis with LRD, surgery correlated with superior OS [HR = 0.27; 95% confidence interval (CI), 0.16–0.47]; chemotherapy and radiotherapy associated with inferior OS (HR = 1.90; 95% CI, 1.11–3.27 and HR = 1.45; 95% CI, 1.03–2.06, respectively). No treatment modality associated with OS in the adjusted, complete case model. In the adjusted sensitivity analysis, surgery associated with superior outcomes (HR = 0.36; 95% CI, 0.19–0.69). There was no OS difference by primary tumor site. 10-year OS with distant disease was 10% (95% CI, 2%–25%). Conclusions: Surgery in LRD associated with improved OS in univariate analysis and adjusted models correcting for missing data. There was no OS benefit with chemotherapy or radiotherapy. Impact: This represents the largest report of EMCS with long-term follow-up. Despite the reputedly indolent nature of EMCS, outcomes with metastatic disease are poor. We provide OS benchmarks and guidance for stratification in future prospective trials.
Antonio G Nascimento - One of the best experts on this subject based on the ideXlab platform.
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extraskeletal Myxoid Chondrosarcoma a light microscopic immunohistochemical ultrastructural and immuno ultrastructural study indicating neuroendocrine differentiation
Histopathology, 2001Co-Authors: Yw Goh, Andre M Oliveira, Dominic V Spagnolo, Michael A Platten, P Caterina, Cyril Fisher, Antonio G NascimentoAbstract:Extraskeletal Myxoid Chondrosarcoma: a light microscopic, immunohistochemical, ultrastructural and immuno-ultrastructural study indicating neuroendocrine differentiation. Aims: Extraskeletal Myxoid Chondrosarcoma is a rare low-grade soft-tissue sarcoma with locally aggressive and metastasizing potential. Extraskeletal Myxoid Chondrosarcoma has distinctive clinical. light microscopic, immunophenotypic, cytogenetic and ultrastructural features. Evidence that extraskeletal Myxoid Chondrosarcoma often shows neuroendocrine features was first provided by Chhieng et al.(9) on the basis of an immunohistochemical and ultrastructural study of seven cases. Our study aims to further confirm by immunohistochemistry and ultrastructural studies, including immunoelectron microscopy. that extraskeletal Myxoid Chondrosarcoma indeed may show neuroendocrine differentiation. Methods and results: Fifteen cases of extraskeletal Myxoid Chondrosarcoma and seven control cases of skeletal Chondrosarcomas were studied. Extensive immunohistochemical analysis was performed in all cases and ultrastructural studies were done in I I extraskeletal Myxoid Chondrosarcomas and three skeletal Chondrosarcomas. Immunoelectron microscopy was performed on one case each of extraskeletal Myxoid Chondrosarcoma and skeletal Chondrosarcoma. Extraskeletal Myxoid Chondrosarcomas expressed neuron-specific enolase (100%,), synaptophysin (87%), S100 (50%). PGP 9.5 (40%), and epithelial membrane antigen (25%). Co-expression of synaptophysin and PGP 9.5 was observed in six tumours. Skeletal Chondrosarcomas showed expression of S100 protein, vimentin and neuron-specific enolase in all cases. Synaptophysin. chromogranin and PGP 9.5 were not expressed in any skeletal Chondrosarcoma case. Ultrastructurally. extraskeletal Myxoid Chondrosarcoma was characterized by distinct cords of cells immersed in a glycosaminoglycan-rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In three cases there were easily found 140-180 nm diameter membrane-bound dense-core granules in cell bodies and in processes, unrelated to the Golgi, compatible with neurosecretory granules. Fewer such granules were present in the remaining extraskeletal Myxoid Chondrosarcoma cases, three of which also contained intracisternal tubules typical of extraskeletal Myxoid Chondrosarcoma. The skeletal Chondrosarcomas had scalloped cell surfaces. prominent rough endoplasmic reticulum focally distended with secretory product, and lacked neurosecretory granules. Intermediate filaments were prominent in both extraskeletal Myxoid Chondrosarcoma and skeletal Chondrosarcomas. Immunoelectron microscopy showed synaptophysin expression in the extraskeletal Myxoid Chondrosarcoma but not in the skeletal Chondrosarcoma case. Conclusions: This study confirms that a substantial proportion of extraskeletal Myxoid Chondrosarcomas show immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation, and are unlikely to be related to conventional skeletal Chondrosarcomas.
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extraskeletal Myxoid Chondrosarcoma
Clinical Orthopaedics and Related Research, 2001Co-Authors: James E Mcgrory, Michael G Rock, Antonio G Nascimento, Andre M OliveiraAbstract:The medical records and histologic material of 16 patients with extraskeletal Myxoid Chondrosarcoma were reviewed. The mean age of the patients was 52 years. Thirteen tumors arose in the lower extremity. Thirteen patients presented with primary, localized disease, whereas three presented with pulmonary metastases. Treatment of the primary site included wide excision or amputation in 13 patients and marginal or intralesional resections with radiation in three patients. The mean followup was 7.4 years. Five patients were continuously disease free (5- and 10-year event free survival 43% and 14%, respectively). Local recurrence developed in four, and metastases developed in six of 13 patients presenting with localized disease. Of six patients who received chemotherapy for systemic disease, four had disease progression and died, and two had a response to chemotherapy (one partial, one complete). The mean survival after onset of metastases was 45 months. Overall 5- and 10-year survival was 87% and 63%, respectively. The current series suggests that extraskeletal Myxoid Chondrosarcoma is an intermediate-grade neoplasm with a tendency toward recurrence and metastasis. Survival after relapse may be prolonged. More effective therapy for systemic disease is needed.
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extraskeletal Myxoid Chondrosarcoma a clinicopathologic immunohistochemical and ploidy analysis of 23 cases
Modern Pathology, 2000Co-Authors: Andre M Oliveira, James E Mcgrory, Michael G Rock, Thomas J Sebo, Thomas A Gaffey, Antonio G NascimentoAbstract:Twenty-three cases of extraskeletal Myxoid Chondrosarcoma, evaluated at the Mayo Clinic between 1968 and 1996, were studied for clinicopathologic features, immunohistochemical profile, Ki-67 activity, and ploidy status to identify adverse prognostic factors. Females and males were equally affected, and the median age at diagnosis was 50 years. The tumors were located mainly in the lower extremities (83%), and the median tumor size was 9.5 cm. Sixteen tumors showed low cellularity (70%), and eight tumors had high mitotic activity (more than two per 10 high-power fields). The tumors were immunoreactive for vimentin (89%), synaptophysin (72%), epithelial membrane antigen (28%), and S-100 protein (17%). Nine tumors were diploid, three aneuploid, and one tetraploid. Mean Ki-67 activity was 11% (range, 1 to 45%). The 10-year overall survival rate was 78%. On univariate analysis, tumor size > or = 10 cm, high cellularity, presence of anaplasia or rhabdoid features, mitotic activity more than two per 10 high-power fields, Ki-67 > or = 10%, and Ki-67 "hot spot" > or = 25% were associated with decreased metastasis-free or overall survival. Ploidy status was not associated with any adverse outcome. The presence of any of these adverse prognostic factors can indicate the possibility of a more aggressive behavior in extraskeletal Myxoid Chondrosarcoma, and a closer follow-up is suggested.
