Neoplasia maligna rara de tecidos moles, de diferenciação incerta, caracterizada pela presença de células semelhantes a condroblastos em um estroma mixóide e um padrão de crescimento multinodular. Os locais mais comuns de envolvimento são os tecidos moles profundos das extremidades, particularmente a coxa. Geralmente se apresenta como uma massa crescente de tecidos moles. Os pacientes podem ter sobrevida longa, mas recorrências locais e metástases ocorrem em aproximadamente metade dos casos. O local mais comum de metástase são os pulmões.
Introdução
O que você precisa saber de cara
Neoplasia maligna rara de tecidos moles, de diferenciação incerta, caracterizada pela presença de células semelhantes a condroblastos em um estroma mixóide e um padrão de crescimento multinodular. Os locais mais comuns de envolvimento são os tecidos moles profundos das extremidades, particularmente a coxa. Geralmente se apresenta como uma massa crescente de tecidos moles. Os pacientes podem ter sobrevida longa, mas recorrências locais e metástases ocorrem em aproximadamente metade dos casos. O local mais comum de metástase são os pulmões.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 2 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
5 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
Binds to ssRNA containing the consensus sequence 5'-AGGUAA-3' (PubMed:21256132). Might normally function as a transcriptional repressor (PubMed:10767297). EWS-fusion-proteins (EFPS) may play a role in the tumorigenic process. They may disturb gene expression by mimicking, or interfering with the normal function of CTD-POLII within the transcription initiation complex. They may also contribute to an aberrant activation of the fusion protein target genes
NucleusCytoplasmCell membrane
Ewing sarcoma
A highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue that affects children and adolescents. It belongs to the Ewing sarcoma family of tumors, a group of morphologically heterogeneous neoplasms that share the same cytogenetic features. They are considered neural tumors derived from cells of the neural crest. Ewing sarcoma represents the less differentiated form of the tumors.
Transcriptional activator that binds to regulatory elements in promoter regions in a cell- and response element (target)-specific manner. Induces gene expression by binding as monomers to the NR4A1 response element (NBRE) 5'-AAAAGGTCA-3' site and as homodimers to the Nur response element (NurRE) site in the promoter of their regulated target genes (By similarity). Plays a role in the regulation of proliferation, survival and differentiation of many different cell types and also in metabolism and
Nucleus
Ewing sarcoma
A highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue that affects children and adolescents. It belongs to the Ewing sarcoma family of tumors, a group of morphologically heterogeneous neoplasms that share the same cytogenetic features. They are considered neural tumors derived from cells of the neural crest. Ewing sarcoma represents the less differentiated form of the tumors.
Transcriptional regulator. Involved in the initiation of neuronal differentiation. Activates transcription by binding to the E box (5'-CANNTG-3') (By similarity). May be involved in the functional network that regulates the development of the GnRH axis (PubMed:32620954)
Nucleus
Craniosynostosis 3
A primary abnormality of skull growth involving premature fusion of one or more cranial sutures. The growth velocity of the skull often cannot match that of the developing brain resulting in an abnormal head shape and, in some cases, increased intracranial pressure, which must be treated promptly to avoid permanent neurodevelopmental disability.
The TFIID basal transcription factor complex plays a major role in the initiation of RNA polymerase II (Pol II)-dependent transcription (PubMed:33795473). TFIID recognizes and binds promoters with or without a TATA box via its subunit TBP, a TATA-box-binding protein, and promotes assembly of the pre-initiation complex (PIC) (PubMed:33795473). The TFIID complex consists of TBP and TBP-associated factors (TAFs), including TAF1, TAF2, TAF3, TAF4, TAF5, TAF6, TAF7, TAF8, TAF9, TAF10, TAF11, TAF12 an
Nucleus
Plays a role in the normal dynamic function of the endoplasmic reticulum (ER) and its associated microtubules (PubMed:23479643, PubMed:27813252). Required for secretory cargo traffic from the endoplasmic reticulum to the Golgi apparatus (PubMed:21478858)
Endoplasmic reticulum
Medicamentos e terapias
Mecanismo: DNA topoisomerase II alpha inhibitor
Mecanismo: DNA topoisomerase II alpha inhibitor
Mecanismo: Vascular endothelial growth factor receptor inhibitor
Variantes genéticas (ClinVar)
348 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 2 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
20 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Condrossarcoma mixoide extraesquelético
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Pesquisa e ensaios clínicos
22 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Endovascular Thrombectomy in a Patient with Acute Ischemic Stroke due to Tumor Emboli Associated with Cardiac Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.
Cardioembolic stroke caused by tumor emboli is a rare form of cancer-associated stroke. We report a case of a patient with cardiac metastasis of extraskeletal chondrosarcoma who developed left internal carotid artery occlusion due to embolic stroke. Extraskeletal chondrosarcoma is an extremely rare subtype of soft tissue sarcoma characterized by a tendency for local recurrence and metastasis. Endovascular thrombectomy was performed using a combined technique, achieving complete revascularization. Histopathological analysis of the retrieved cell mass confirmed that the embolic source was the preexisting extraskeletal chondrosarcoma.
A series of extraskeletal myxoid chondrosarcomas with rare morphological and molecular variations.
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma characterised by myxoid matrix, multilobular architecture, eosinophilic ovoid to short spindle cells arranged in cords, clusters or reticular patterns and NR4A3 gene rearrangement. However, some EMCs show morphological variations or non-EWSR1::NR4A3 fusion. We described herein five cases of variant EMCs. The five patients included two females and three males, aged 24-59 years (median: 49 years). The tumours were located in the dorsal region, buttock, thigh, paravertebral region and elbow, respectively. Grossly, the tumour sizes ranged from 4.0 to 16.0 cm (median: 6.5 cm) in greatest dimension. Morphological examination revealed tumours with varied growth patterns, including solid variants with eosinophilic proteinaceous fluid (n = 2), classic EMC morphology with rhabdoid cells (n = 1), a biphasic variant, composed of fibroblastic/myofibroblastic-like cells and oval to short spindle-shaped cells (n = 1), and a spindle-cell morphology with a myxoid stroma and prominent haemorrhagic cystic spaces (n = 1). Immunohistochemically, all cases showed variable expression of CD117. Next-generation sequencing (NGS) identified an EWSR1::NR4A3 fusion, a novel FUS::NR4A2 fusion, a novel ACTB::NR4A3 fusion, and two FUS::NR4A3 fusions. Follow-up for all five patients showed no signs of local recurrence or distant metastasis. These five cases of EMC highlight the continuous morphological spectrum of this tumour, demonstrating significantly greater histological diversity than classically described. The identification of novel fusion partners further expands its genetic landscape.
Reconstruction after unplanned excisions of soft tissue sarcomas using intraoperative extracorporeal autogenous irradiated bone and tendon grafts: a report of three cases.
Wide resection of soft tissue sarcomas (STS) of the hand and foot is challenging due to limited soft tissue and complex anatomy. Reconstruction often requires tissue from healthy donor sites. To address this, we used intraoperative extracorporeal irradiation (ECI) of tumor-affected bones and tendons for reconstruction after wide resection. Herein, we report the oncological and functional outcomes using this technique. Three patients who underwent unplanned excision of the hand or foot at another institution referred to our hospital for additional treatment. Case 1: A 46-year-old man with extraskeletal myxoid chondrosarcoma on the dorsal aspect of the left hand underwent wide resection followed by ECI bone and extensor tendon reconstruction with free flap coverage, and subsequently required tenolysis. At 5 years, he showed no recurrence and excellent function (Musculoskeletal tumor society (MSTS) score, 100%; Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH) score, 20.5). Case 2: A 27-year-old woman with synovial sarcoma of the dorsal right hand underwent wide resection and ECI bone and extensor tendon reconstruction. Delayed bone union required secondary bone grafting. She remained disease-free at 6 years with mild functional impairment (MSTS, 80%; QuickDASH, 29.5). Case 3: A 19-year-old woman with synovial sarcoma of the dorsal aspect of the right hallux underwent en bloc resection and ECI bone and extensor tendon reconstruction. At 4 years, she had no recurrence and excellent functional recovery (MSTS, 97%). Reconstruction using ECI-treated autogenous bone and tendon grafts allows effective oncological resection while preserving limb function and avoiding donor-site morbidity. Although mild degenerative changes were observed after osteoarticular reconstruction, all patients reported satisfactory outcomes and returned to their occupations. ECI is a valuable limb-salvage reconstruction option for STS of the hands and feet.
[Clinicopathological and molecular characteristics of extraskeletal myxoid chondrosarcoma: an analysis of sixteen cases].
Objective: To investigate the clinicopathological features, molecular characteristics and differential diagnosis of extraskeletal myxoid chondrosarcoma (EMC). Methods: A total of 16 cases of EMC diagnosed from January 2016 to February 2025 were collected from Luoyang Orthopedic-Traumatological Hospital of Henan Province (Henan Provincial Orthopedic Hospital, 3 cases) and Beijing Jishuitan Hospital, Capital Medical University (13 cases) for clinicopathological, immunohistochemical, fluorescence in situ hybridization (FISH) and next generation sequencing analyses and follow-up. Results: There were 11 males and 5 females, with a median age of 51(30, 73) years. The tumor sites included extremities (n=12), trunk (n=3), and sacrum (n=1). Histologically, EMC showed two distinct subtypes. Fourteen cases (14/16) were classic subtype with pale-blue myxoid or chondromyxoid matrix. The cells characteristically interconnected with one another to form cords, small clusters, and complex trabecular or cribriform patterns. The tumor cells were round, ovoid, short spindle or star-shaped; some may be rhabdoid with eosinophilic cytoplasm and eccentrically placed nuclei. Two cases were cellular subtype, demonstrating solid sheets of epithelioid cells with minimal intervening myxoid matrix, large vesicular nuclei, prominent nucleoli, and brisk mitotic activity. Immunohistochemistry showed that the cells diffusely or partially expressed vimentin, CD117, Syn, INSM-1, CD34, SMA and NSE, but were negative for pan-keratin (AE1/AE3), S-100, calponin, desmin and brachyury. INI1 expression was not deleted. The proliferation index of Ki-67 ranged from 2% to 15% in the classic subtype and 5% in the cellular subtype. NR4A3 gene rearrangement was detected by FISH in 15 cases (15/16), and EWSR1::NR4A3 fusion was confirmed by next-generation sequencing in 4 cases. Follow-up data were available in 14 patients (1-104 months), of whom 7 (7/14) developed local recurrence and 2 (2/14) developed distant metastases. Conclusions: EMC is a rare mesenchymal malignancy that arises not only in soft tissues but also in bone. The predominant histological subtype is classical EMC, with a minority presenting as cell-rich EMC. FISH detection of NR4A3 gene rearrangements provides a crucial value for the diagnosis. It needs to be differentiated from myoepithelial tumors, chordomas and myxoid liposarcomas. 目的： 探讨骨外黏液样软骨肉瘤（extraskeletal myxoid chondrosarcoma，EMC）的临床病理学特征、分子特征及鉴别诊断。 方法： 收集河南省洛阳正骨医院（河南省骨科医院，3例）及首都医科大学附属北京积水潭医院（13例）2016年1月至2025年2月确诊的16例EMC患者，分析其临床资料、组织病理学特点、免疫组织化学标志物表达情况及分子病理学特征，并进行随访。 结果： 16例患者中男性11例，女性5例，年龄51（30，73）岁。12例发生于四肢，3例发生于躯干，1例发生于骶骨。EMC呈分叶状生长，组织学形态可分为2种类型。14例（14/16）为经典型，丰富的黏液背景下较一致的圆形、卵圆形、短梭形或星形细胞稀疏排列，瘤细胞相互连接成索状、小梁状或网格状。瘤细胞也可呈横纹肌样，胞质嗜伊红核偏位。2例（2/16）为富细胞型，大部分区域瘤细胞丰富呈片状或实性排列，含有少量黏液样基质。瘤细胞上皮样、核呈空泡状，核仁明显，核分裂活跃。瘤细胞不同程度表达波形蛋白（15/15）、CD117（12/14）、突触素（6/13）、INSM-1（3/6），少数病例表达CD34、平滑肌肌动蛋白、神经元特异性烯醇化酶，INI1未缺失，广谱细胞角蛋白（AE1/AE3）、S-100蛋白、Calponin、结蛋白、brachyury均阴性，Ki-67阳性指数经典型为2%~15%，富细胞型约5%。15例荧光原位杂交检测到NR4A3基因重排（15/16），其中4例二代测序检测到EWSR1：：NR4A3融合基因，1例（例13）同时检测到EWSR1基因和NR4A3基因断裂。14例获得随访，随访时间1~104个月，7例（7/14）出现局部复发，2例（2/14）远处转移。 结论： 骨外黏液样软骨肉瘤是一种罕见的间叶性恶性肿瘤，除发生于软组织外，也可发生于骨。病理组织学类型主要为经典型，少数为富细胞型。FISH法检测NR4A3基因重排对诊断具有重要价值。需与软组织肌上皮瘤、脊索瘤、黏液样脂肪肉瘤等鉴别。.
A Case Report of a Patient Presenting With Extra-skeletal Myxoid Chondrosarcoma.
Extra-skeletal myxoid chondrosarcoma (EMC) is a type of rare sarcoma of uncertain differentiation. This malignancy is marked by the growth of primitive chondroid cells forming multiple nodules within a rich myxoid matrix. It is distinguished by a specific translocation resulting in the fusion gene EWSR1::NR4A3, which is a distinctive molecular hallmark of EMC. EMC is categorised as an intermediate-grade tumour and is distinguished by a prolonged clinical course with a high likelihood of local recurrence and distant metastasis. Surgical treatment is the only option for a cure for EMC, while non-surgical treatments are typically considered for recurrent or distant disease. This case report discusses an interesting case of a patient with EMC and further elaborates on the history, examination, imaging, pathological findings, and management of EMC.
Publicações recentes
Small Round Cell Tumors of Soft Tissue and Skeleton in Children and Youth: A Narrative and TruSight-Based Illustrative Review of Diagnostic Cases.
Endovascular Thrombectomy in a Patient with Acute Ischemic Stroke due to Tumor Emboli Associated with Cardiac Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.
A series of extraskeletal myxoid chondrosarcomas with rare morphological and molecular variations.
Reconstruction after unplanned excisions of soft tissue sarcomas using intraoperative extracorporeal autogenous irradiated bone and tendon grafts: a report of three cases.
[Clinicopathological and molecular characteristics of extraskeletal myxoid chondrosarcoma: an analysis of sixteen cases].
📚 EuropePMC281 artigos no totalmostrando 175
Endovascular Thrombectomy in a Patient with Acute Ischemic Stroke due to Tumor Emboli Associated with Cardiac Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.
NMC case report journalA series of extraskeletal myxoid chondrosarcomas with rare morphological and molecular variations.
HistopathologyReconstruction after unplanned excisions of soft tissue sarcomas using intraoperative extracorporeal autogenous irradiated bone and tendon grafts: a report of three cases.
World journal of surgical oncology[Clinicopathological and molecular characteristics of extraskeletal myxoid chondrosarcoma: an analysis of sixteen cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyA Case Report of a Patient Presenting With Extra-skeletal Myxoid Chondrosarcoma.
CureusPrimary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review.
Oncology lettersExcellent Response and Persistent Local Control of Metastatic Extraskeletal Myxoid Chondrosarcoma Repeatedly Treated with Surgical Excision or Stereotactic Radiotherapy Alone: A Case Report.
Case reports in oncologySpontaneous regression of metastatic disease after palliative debulking surgery for heavily pre-treated extraskeletal myxoid chondrosarcoma: A case report.
Rare tumorsNovel HSPA8-NR4A2 rearrangement in extraskeletal myxoid chondrosarcoma: a sarcoma mimicker of neuroendocrine neoplasia.
Virchows Archiv : an international journal of pathologyIncidence and prognostic impact of regional lymph node metastasis at the time of diagnosis in soft-tissue sarcoma : a population-based cohort study.
The bone & joint journalClinical and magnetic resonance imaging features of soft tissue extraskeletal myxoid chondrosarcoma: A retrospective observational cohort study.
Skeletal radiologyFrom pathogenesis to the patient's bedside: a comprehensive review of extraskeletal myxoid chondrosarcoma.
Journal of cancer research and clinical oncologyResection of Primary Lesion for Patients With Metastatic Soft Tissue Sarcoma at Initial Presentation.
Anticancer researchThe role of radiotherapy and chemotherapy in extraskeletal myxoid chondrosarcoma.
Journal of orthopaedic surgery and researchSecondary Genetic Alterations in Extraskeletal Myxoid Chondrosarcoma.
Genes, chromosomes & cancerExtraskeletal Myxoid Chondrosarcoma With Rhabdoid Features in a Pediatric Patient.
CureusEstablishment and characterization of NCC-EMC1-C1: a novel patient-derived cell line of extraskeletal myxoid chondrosarcoma.
Human cellExtraskeletal myxoid chondrosarcoma mimicking mucinous ovarian cancer: A case report.
The journal of obstetrics and gynaecology researchNon-myxoid solid variant of extraskeletal myxoid chondrosarcoma: An underrecognized subtype.
Human pathologyLaparoscopic-Assisted, Percutaneous Cryoablation: A Novel Technique for the Treatment of Abdominal Wall Soft Tissue Tumors.
Annals of surgical oncologyClinical, imaging and pathological features of extraskeletal myxoid chondrosarcoma.
Skeletal radiologyWhole genome sequencing for metastatic mutational burden in extraskeletal myxoid chondrosarcoma.
Frontiers in molecular medicinePrimary pulmonary myxoid sarcoma in the interlobar fissure of the left lung lobe: a case report.
BMC pulmonary medicine[Detection of EWSR1 gene rearrangement by fluorescence in situ hybridization in bone and soft tissue tumors: clinical application evaluation and atypical signal analysis].
Zhonghua bing li xue za zhi = Chinese journal of pathologyExtraskeletal myxoid chondrosarcoma metastasis to a Meckel's diverticulum adenocarcinoma.
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de CitologiaFDG PET/CT and MRI Findings of Extraskeletal Myxoid Chondrosarcoma Showing a Nonmyxoid Cellular Variant.
Clinical nuclear medicineCHRNA6 RNA In Situ Hybridization Is a Useful Tool for the Diagnosis of Extraskeletal Myxoid Chondrosarcoma.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncExtraskeletal Myxoid Chondrosarcoma of Floor of Mouth-A Rare Case Report and Review of Literature.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaIdentification of Novel/Rare EWSR1 Fusion Partners in Undifferentiated Mesenchymal Neoplasms.
International journal of molecular sciences18 F-FDG PET/CT Findings of Ovarian Myxoid Chondrosarcoma.
Clinical nuclear medicineCorrigendum to "Juxta-articular extraskeletal myxoid chondrosarcoma mistaken for a benign cyst presenting with multiple lung metastases" [Radiology Case Reports 19 (2024) 684-690].
Radiology case reportsExtraskeletal myxoid chondrosarcoma of the buttock: a case report and literature review.
Frontiers in oncologyJuxta-articular extraskeletal myxoid chondrosarcoma mistaken for a benign cyst presenting with multiple lung metastases.
Radiology case reportsSMARCB1/INI1-deficient epithelioid and myxoid neoplasms in paratesticular region: Expanding the clinicopathologic and molecular spectrum.
Annals of diagnostic pathologyExtraskeletal Myxoid Chondrosarcoma Identified in a Traumatic Fracture of the Toe: A Case Report.
Journal of orthopaedic case reportsExtraskeletal myxoid chondrosarcoma of the gingival: a rare case report and review of the literature.
Diagnostic pathologyExtraskeletal myxoid chondrosarcoma: a case report.
The Pan African medical journalPRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
Virchows Archiv : an international journal of pathologyExtraskeletal Myxoid Chondrosarcoma of the Jugular Foramen in a Pediatric Patient: A Case Report and Comprehensive Review of the Literature.
Pediatric neurosurgeryTAF15::NR4A3 gene fusion identifies a morphologically distinct subset of extraskeletal myxoid chondrosarcoma mimicking myoepithelial tumors.
Genes, chromosomes & cancerEWSR1::NR4A3 gene fusion in a cutaneous atypical myoepithelial neoplasm.
Journal of cutaneous pathologyExtraskeletal Myxoid Chondrosarcomas: The Uncommon Clinicopathologic Manifestations and Significance of TAF15::NR4A3 Fusion.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncExtraskeletal Myxoid Chondrosarcoma: Retrospective Case Series Examining Prognostic Factors, Treatment Approaches, and Oncologic Outcomes.
American journal of clinical oncologyKeratin-positive fibrotic extraskeletal myxoid chondrosarcoma: a close mimic of myoepithelial tumour.
HistopathologyExtraskeletal myxoid chondrosarcoma in the pelvis successfully treated with proton beam radiotherapy.
BMJ case reportsExtraskeletal Myxoid Chondrosarcoma of the Vulva: A Case Report.
CureusA nonsteroidal anti-inflammatory drug, zaltoprofen, inhibits the growth of extraskeletal chondrosarcoma cells by inducing PPARγ, p21, p27, and p53.
Cell cycle (Georgetown, Tex.)Intra-muscular extraskeletal myxoid chondrosarcoma of the thigh: a case report.
International journal of clinical and experimental pathologyTranscription regulators and ultra-rare and other rare translocation-related sarcomas treated with trabectedin: A proof of principle from a post-hoc analysis.
Frontiers in oncologyExtraskeletal myxoid chondrosarcoma: A study of 17 cases focusing on the diagnostic utility of INSM1 expression and presenting rare morphological variants associated with non-EWSR1::NR4A3 fusions.
Human pathologyExtraskeletal myxoid chondrosarcoma: p53 and Ki-67 offer prognostic value for clinical outcome - an immunohistochemical and molecular analysis of 31 cases.
Virchows Archiv : an international journal of pathologySurgical outcomes of extraskeletal myxoid chondrosarcoma.
Turkish journal of medical sciencesEstablishment, characterization and functional testing of two novel ex vivo extraskeletal myxoid chondrosarcoma (EMC) cell models.
Human cell[Cellular-rich extraskeletal myxoid chondrosarcoma: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyIdentification of Novel PGR-NR4A3 Fusion in Extraskeletal Myxoid Chondrosarcoma and Resultant Patient Benefit From Tamoxifen Therapy.
JCO precision oncologyMetastatic extraskeletal myxoid chondrosarcoma presenting as a forehead mass.
JAAD case reportsImmunosuppressive tumor microenvironment in extraskeletal myxoid chondrosarcoma: A case of pleural metastases.
Thoracic cancerExtraskeletal myxoid chondrosarcoma: Clinicopathological features and outcomes from the United States sarcoma collaborative database.
Journal of surgical oncologyA novel fusion variant LSM14A::NR4A3 in extraskeletal myxoid chondrosarcoma.
Genes, chromosomes & cancerCase Report: Gene Heterogeneity in the Recurrent and Metastatic Lesions of a Myxoid Chondrosarcoma Patient With Aggressive Transformation.
Frontiers in geneticsMusculoskeletal Tumors in Extremities Diagnosed During the Gestational Period: Case Series and Literature Review.
Acta chirurgiae orthopaedicae et traumatologiae CechoslovacaExtraskeletal myxoid chondrosarcoma of the penis: Description of a rare tumor arising in an unusual location.
Urology case reportsDifferential diagnosis of myxoid soft tissue tumors. Experience in the Clinical University Hospital of Valencia.
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de CitologiaPrimary extraskeletal myxoid chondrosarcoma of the breast: report of a case and literature review.
PathologicaMagnetic Resonance Imaging of Unusual Neoplasms Related to Foramen of Luschka: A Review for Differential Diagnosis.
The Indian journal of radiology & imagingPrimary intracranial extraskeletal myxoid chondrosarcoma: A case report and review of literature.
World journal of clinical casesGRM1 Immunohistochemistry Distinguishes Chondromyxoid Fibroma From its Histologic Mimics.
The American journal of surgical pathologyHigh-dose-rate interstitial brachytherapy as a suitable option for metastatic extraskeletal myxoid chondrosarcoma - a case report.
Journal of contemporary brachytherapyMutation of KIT in cellular extraskeletal myxoid chondrosarcoma: a case report and literature review.
Diagnostic pathologyExtraskeletal myxoid chondrosarcoma: A case series and review of the literature.
Rare tumorsExtraskeletal myxoid chondrosarcoma: A rare but important differential of a retroperitoneal mass.
Journal of medical imaging and radiation oncologyExtraskeletal myxoid chondrosarcoma: Clinical features and overall survival.
Cancer treatment and research communicationsAnalysis of clinicopathologic features and expression of NR4A3 in sinonasal acinic cell carcinoma.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncIntracranial Extraskeletal Myxoid Chondrosarcoma in Fourth Ventricle.
Brain tumor research and treatmentClinical Activity of Single-Agent Cabozantinib (XL184), a Multi-receptor Tyrosine Kinase Inhibitor, in Patients with Refractory Soft-Tissue Sarcomas.
Clinical cancer research : an official journal of the American Association for Cancer ResearchFine-needle aspiration cytopathology of soft tissue myoepithelioma: an analysis of seven cases.
Journal of the American Society of CytopathologyPeriosteal Myxoid Leiomyosarcoma Histologically Mimicking Extraskeletal Myxoid Chondrosarcoma: Report of a Case with Histopathological and Cytopathological Comparison with Extraskeletal Myxoid Chondrosarcoma.
Acta cytologicaPrimary intracranial extra-skeletal myxoid chondrosarcoma of right lateral ventricle with EWSR1 gene fusion: a case report and review of literature.
EcancermedicalscienceAnti-Hu-mediated paraneoplastic chronic intestinal pseudo-obstruction associated with extraskeletal myxoid chondrosarcoma.
Revista espanola de enfermedades digestivasA rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues.
Autopsy & case reportsSustained response to imatinib in patient with extraskeletal myxoid chondrosarcoma and novel KIT mutation.
BMJ case reports[Clinicopathological and molecular features of small round cell sarcoma of bone and soft tissue: a study of 72 cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyUncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations. Spanish Group for Sarcoma research (GEIS -GROUP). Part II.
Cancer treatment reviewsNovel RGAG1-BCOR gene fusion revealed in a somatic soft tissue sarcoma with a long follow-up.
Virchows Archiv : an international journal of pathologyBone and soft tissue sarcomas in cerebrospinal fluid and effusion: A 20-year review at our institution.
Cancer cytopathologySMARCA2-NR4A3 is a novel fusion gene of extraskeletal myxoid chondrosarcoma identified by RNA next-generation sequencing.
Genes, chromosomes & cancerWide en bloc resection of an extraskeletal myxoid chondrosarcoma about the knee with single-stage knee extensor mechanism reconstruction: A case report.
The KneeMyxoepithelioid tumour with chordoid features: a clinicopathological, immunohistochemical and genetic study of 14 cases of SMARCB1/INI1-deficient soft-tissue neoplasm.
HistopathologyExtraskeletal myxoid chondrosarcoma: a case report with adjuvant intraoperative treatment.
Journal of surgical case reportsLocally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response.
Clinical sarcoma researchDetection of Structural Variants in Circulating Cell-Free DNA from Sarcoma Patients Using Next Generation Sequencing.
CancersExtraskeletal Myxoid Chondrosarcoma of the Vulva Confirmed by EWSR1 FISH: A Case Report and Review of the Literature.
International journal of gynecological pathology : official journal of the International Society of Gynecological PathologistsExtraskeletal Myxoid Chondrosarcoma: State of the Art and Current Research on Biology and Clinical Management.
CancersExtraskeletal Myxoid Chondrosarcoma: Long-Term Survival in the Setting of Metastatic Disease.
Case reports in orthopedicsRelevance of the combination of external beam radiotherapy with the hypoxia-activated prodrug ICF05016 in an experimental model of extraskeletal myxoid chondrosarcoma.
Investigational new drugsCurability of patients with lymph node metastases from extremity soft-tissue sarcoma.
CancerLong-term Outcomes for Extraskeletal Myxoid Chondrosarcoma: A SEER Database Analysis.
Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive OncologyExtraskeletal myxoid chondrosarcoma: combining cytopathology with molecular testing to achieve diagnostic accuracy.
Journal of the American Society of CytopathologyExtraskeletal myxoid chondrosarcoma: A high incidence of metastatic disease to lymph nodes.
Journal of surgical oncologySWI/SNF complex-deficient soft tissue neoplasms: An update.
Seminars in diagnostic pathologyExtraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions.
Frontiers in oncologyASO Author Reflections: Extraskeletal Myxoid Chondrosarcoma: A Rare Sarcoma Associated With Prolonged Survival.
Annals of surgical oncologyExtraskeletal Myxoid Chondrosarcoma with Molecularly Confirmed Diagnosis: A Multicenter Retrospective Study Within the Italian Sarcoma Group.
Annals of surgical oncologyApatinib for Treatment of Inoperable Metastatic or Locally Advanced Chondrosarcoma: What We Can Learn About the Biological Behavior of Chondrosarcoma from a Two-Center Study.
Cancer management and researchDesign, synthesis and evaluation of targeted hypoxia-activated prodrugs applied to chondrosarcoma chemotherapy.
Bioorganic chemistryThe Extracompartmental Tumoral Invasion of Extraskeletal Myxoid Chondrosarcoma Induces Distant Metastasis.
Anticancer researchEwing's sarcoma/peripheral primitive neuroectodermal tumor with extraskeletal myxoid chondrosarcoma-like areas: a case report.
International journal of clinical and experimental pathologyExtraskeletal Myxoid Chondrosarcoma of the Parotid Gland.
Annals of maxillofacial surgeryEfficacy and Safety of Nanosomal Docetaxel Lipid Suspension-Based Chemotherapy in Sarcoma: A Multicenter, Retrospective Study.
SarcomaA renal cell carcinoma with EWSR1-TFE3 fusion gene.
Genes, chromosomes & cancerExtraskeletal Myxoid Chondrosarcoma of the Midfoot Presenting as Charcot Arthropathy: A Case Report.
JBJS case connectorResults of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma.
CancerNR4A3 Immunohistochemistry Lacks Sensitivity for the Diagnosis of Extraskeletal Myxoid Chondrosarcoma.
The American journal of surgical pathologyPazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: a multicentre, single-arm, phase 2 trial.
The Lancet. OncologyNR4A3 fusion proteins trigger an axon guidance switch that marks the difference between EWSR1 and TAF15 translocated extraskeletal myxoid chondrosarcomas.
The Journal of pathologyEWSR1-NFATC2 Translocation-associated Sarcoma Clinicopathologic Findings in a Rare Aggressive Primary Bone or Soft Tissue Tumor.
The American journal of surgical pathologyExtraskeletal myxoid chondrosarcoma of the leg in a child: A case report.
MedicineA 5-year follow-up of vulval swelling due to extraskeletal myxoid chondrosarcoma: A rare case report.
Molecular and clinical oncologySystemic manifestations of extraskeletal myxoid chondrosarcoma associated with a novel t(2;22)(q34;q12) EWS translocation in a child and a review of the literature.
Pediatric hematology and oncologySWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis.
Surgical pathology clinicsExtraskeletal myxoid chondrosarcoma with massive pulmonary metastases.
Clinical sarcoma researchFine-needle aspiration features of extraskeletal myxoid chondrosarcoma: A study of cytological and molecular features.
Diagnostic cytopathologyIntraluminal EWSR1-CREB1 gene rearranged, low-grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC).
HistopathologyMyxoid chondrosarcoma extraskeletal in pelvis location : a case report.
La Tunisie medicaleExtraskeletal Chondrosarcoma: Long-term Follow-up of a Patient with Metastatic Disease.
CureusExtraskeletal Myxoid Chondrosarcoma: A Comparative Study of Imaging and Pathology.
BioMed research internationalIdentification of an Actionable Mutation of KIT in a Case of Extraskeletal Myxoid Chondrosarcoma.
International journal of molecular sciencesMolecular and Clinicopathologic Heterogeneity of Intracranial Tumors Mimicking Extraskeletal Myxoid Chondrosarcoma.
Journal of neuropathology and experimental neurology[Primary extraskeletal myxoid chondrosarcoma of the corpus callosum: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyMyxoid and reticular angiomatoid fibrous histiocytoma: a case confirmed by fluorescence in situ hybridization analysis for EWSR1 rearrangement.
International journal of clinical and experimental pathologyComprehensive high-resolution genomic profiling and cytogenetics of human chondrocyte cultures by GTG-banding, locus-specific FISH, SKY and SNP array.
European cells & materialsExtraskeletal Myxoid Chondrosarcoma of Nasopharynx: An Oncologic Entity Rarely Reported.
Oman medical journalExtra-axial chordoma: a clinicopathologic analysis of six cases.
Virchows Archiv : an international journal of pathologyPre- and postoperative radiotherapy for extremity soft tissue sarcoma: Evaluation of inter-observer target volume contouring variability among French sarcoma group radiation oncologists.
Cancer radiotherapie : journal de la Societe francaise de radiotherapie oncologiquePrimary extraskeletal myxoid chondrosarcoma in cerebellum: A case report with literature review: Erratum.
MedicinePrimary extraskeletal myxoid chondrosarcoma in cerebellum: A case report with literature review.
MedicineEWSR1-fusion-negative, SMARCB1-deficient primary pulmonary myxoid sarcoma.
Polish journal of pathology : official journal of the Polish Society of PathologistsINSM1 expression and its diagnostic significance in extraskeletal myxoid chondrosarcoma.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncPrimary pulmonary myxoid sarcoma, a potential mimic of metastatic extraskeletal myxoid chondrosarcoma.
PathologyClinical benefit of antiangiogenic therapy in advanced and metastatic chondrosarcoma.
Medical oncology (Northwood, London, England)Programmed Death Ligand 1 (PD-L1) Expression in Malignant Mesenchymal Tumors.
Turk patoloji dergisi[Histology-Specific Chemotherapy in Soft-Tissue Sarcomas].
Gan to kagaku ryoho. Cancer & chemotherapyExtraskeletal myxoid chondrosarcoma of the masticator space in a pediatric patient.
Journal of clinical and experimental dentistryClaudin 6 expression is useful to distinguish myxofibrosarcomas from other myxoid soft tissue tumors.
Pathology, research and practiceNext generation sequencing of extraskeletal myxoid chondrosarcoma.
OncotargetHSPA8 as a novel fusion partner of NR4A3 in extraskeletal myxoid chondrosarcoma.
Genes, chromosomes & cancerCurious case of extraskeletal myxoid chondrosarcoma.
Lung India : official organ of Indian Chest SocietyPrimary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature.
Pathology, research and practiceA rare manifestation of extraskeletal myxoid chondrosarcoma with a huge expanding hematoma.
Journal of orthopaedic science : official journal of the Japanese Orthopaedic AssociationThe spectrum of EWSR1-rearranged neoplasms at a tertiary sarcoma centre; assessing 772 tumour specimens and the value of current ancillary molecular diagnostic modalities.
British journal of cancerUnusual Signal Patterns of Break-apart FISH Probes Used in the Diagnosis of Soft Tissue Sarcomas.
Pathology oncology research : PORDiagnosis of extraskeletal myxoid chondrosarcoma in the thigh using EWSR1-NR4A3 gene fusion: a case report.
Journal of medical case reportsBenefit of Radiotherapy in Extraskeletal Myxoid Chondrosarcoma: A Propensity Score Weighted Population-based Analysis of the SEER Database.
American journal of clinical oncologyExtraskeletal Myxoid Chondrosarcoma of the Foot Clinically Mimicking Plantar Fibromatosis.
The American Journal of dermatopathologyMyxoid chondrosarcoma of the mandible in a 22-year-old man: A case report.
Molecular and clinical oncologyPulmonary extraskeletal myxoid chondrosarcoma: A case report and literature review.
International journal of surgery case reportsResults of sub-analysis of a phase 2 study on trabectedin treatment for extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma.
BMC cancerClinicopathologic and radiologic features of extraskeletal myxoid chondrosarcoma: a retrospective study of 40 Chinese cases with literature review.
Annals of diagnostic pathologyExtraskeletal myxoid chondrosarcoma of maxilla: A rare entity.
Journal of oral and maxillofacial pathology : JOMFPExtraskeletal Myxoid Chondrosarcoma of the Orbit.
Middle East African journal of ophthalmologyPrimary intra-articular sarcoma: a clinicopathological study of 15 cases.
HistopathologyUnresectable Extraskeletal Myxoid Chondrosarcoma of the Neck: Early Tumor Response to Chemoradiotherapy.
CureusInvasive urothelial carcinoma with chordoid features of the ureter: a rare entity and review of literature.
International journal of clinical and experimental pathology[Extraskeletal myxoid chondrosarcoma: a clinicopathologic analysis of seven cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyExtraskeletal Myxoid Chondrosarcoma Presenting as a Plantar Fibroma: Case Report and Review of the Literature.
Anticancer researchParavertebral extraskeletal myxoid chondrosarcoma: a case report and review of the literature.
The Pan African medical journalSpontaneous Regression of Metastatic Extraskeletal Myxoid Chondrosarcoma.
The Annals of thoracic surgeryThe TFG-TEC oncoprotein induces transcriptional activation of the human β-enolase gene via chromatin modification of the promoter region.
Molecular carcinogenesisBreast Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.
The American journal of case reportsSoft tissue myoepithelial carcinoma with rhabdoid-like features and EWSR1 rearrangement: Fine needle aspiration cytology with histologic correlation.
Diagnostic cytopathology[Small cell type (Ewing-like) clear cell sarcoma of soft parts: a case report].
Ceskoslovenska patologie[Cardiac metastase of a soft tissue sarcoma: a case report and a review of the literature].
Revue medicale de BruxellesExtraskeletal myxoid chondrosarcoma presenting as an intradural spinal mass: report of a rare clinical presentation with an emphasis on differential diagnostic considerations.
Rare tumorsPrimary extraskeletal myxoid chondrosarcoma of pulmonary arteries: a rare mimic of acute pulmonary thromboembolism.
Interactive cardiovascular and thoracic surgeryExtraskeletal myxoid chondrosarcoma of the thigh in a child: a case report.
Journal of orthopaedic surgery (Hong Kong)Associações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Endovascular Thrombectomy in a Patient with Acute Ischemic Stroke due to Tumor Emboli Associated with Cardiac Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.
- A series of extraskeletal myxoid chondrosarcomas with rare morphological and molecular variations.
- Reconstruction after unplanned excisions of soft tissue sarcomas using intraoperative extracorporeal autogenous irradiated bone and tendon grafts: a report of three cases.
- [Clinicopathological and molecular characteristics of extraskeletal myxoid chondrosarcoma: an analysis of sixteen cases].
- A Case Report of a Patient Presenting With Extra-skeletal Myxoid Chondrosarcoma.
- Small Round Cell Tumors of Soft Tissue and Skeleton in Children and Youth: A Narrative and TruSight-Based Illustrative Review of Diagnostic Cases.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:209916(Orphanet)
- OMIM OMIM:612237(OMIM)
- MONDO:0012825(MONDO)
- GARD:17105(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q18557267(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
