Sarcoma indiferenciado de partes moles caracterizado pela presença de infiltrado celular maligno pleomórfico. Também é conhecido como histiocitoma fibroso maligno.
Introdução
O que você precisa saber de cara
Sarcoma indiferenciado de partes moles caracterizado pela presença de infiltrado celular maligno pleomórfico. Também é conhecido como histiocitoma fibroso maligno.
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
Partes do corpo afetadas
+ 4 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 9 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
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 — Sarcoma pleomórfico indiferenciado
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Outros ensaios clínicos
95 ensaios clínicos encontrados, 21 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.008
Severe mitral stenosis due to primary cardiac intimal sarcoma: case report.
Primary cardiac tumours are exceedingly rare with intimal sarcomas being the rarest subtype of malignant cardiac sarcomas. These tumours are characterized by late presentation, haemodynamic compromise, and poor prognosis due to their rapid progression and metastatic potential. Multimodal management is essential given the lack of standardized guidelines. A 64-year-old female presented with heart failure and atrial fibrillation. An echocardiogram revealed a 48 mm obstructive intra-cardiac mass in the left atrium causing severe functional mitral stenosis. Given recurrent episodes of flash pulmonary oedema secondary to mitral stenosis, a multi-disciplinary decision was made to proceed with high-risk surgical debulking. The patient underwent resection with pathology showing an undifferentiated pleomorphic sarcoma with evidence of MDM2 amplification, in keeping with an intimal sarcoma. She was discharged home in stable condition but had progression of disease despite treatment with doxorubicin. She died 6 months after from a haemorrhagic stroke. Intimal sarcomas carry a dismal prognosis due to late-stage detection and challenges with complete resection. While anthracycline-based chemotherapy and surgical resection may modestly extend survival, recurrence and metastases remain common. This case underscores the aggressive nature of cardiac intimal sarcoma and the vital role of early diagnosis and coordinated, multi-disciplinary care. Additionally, the psychosocial toll of such a devastating illness necessitates early integration of supportive services to address patient and family needs during a rapidly evolving clinical course.
A Phase 1 Study of Neoadjuvant Cabozantinib in Combination With Radiation Therapy for Sarcomas of the Extremities.
Cabozantinib demonstrates activity in multiple soft tissue sarcoma (STS) subtypes, but use with concurrent radiation therapy (RT) has been limited by concern for risk of fistula or perforation. This phase 1 trial evaluated the safety of concurrent cabozantinib and RT as neoadjuvant therapy in patients with extremity STS. Adults with newly diagnosed localized extremity STS planned for neoadjuvant RT and surgical resection were eligible. Participants received radiation with 5000 to 5040 cGy with conventional fractionation and cabozantinib 40 mg or 60 mg daily. Patients were observed for dose-limiting toxicity (DLT) up to 28 days after completion of concurrent cabozantinib/RT. The primary objective was to identify a recommended phase 2 dose (RP2D) of cabozantinib for combination with RT, and secondary objectives included estimating rates of treatment-related adverse event (TRAE), margin positivity, and objective response. Six patients were enrolled with histologic subtypes of undifferentiated pleomorphic sarcoma, myxofibrosarcoma, leiomyosarcoma, and myxoid liposarcoma. No DLT due to the combination of cabozantinib and radiation was observed, but 3/6 (50%) patients required dose-reduction due to TRAE of cabozantinib alone. No grade ≥3 toxicities were attributed to RT. The RP2D was cabozantinib 60 mg. Six (100%) patients demonstrated stable disease at 12 weeks, and 5 (83%) underwent R0 resection. Two (33%) patients experienced metastatic relapse, and 1 (17%) died without relapse; 3 (50%) patients survived without relapse by last contact. No local recurrences occurred. In this phase 1 trial, concurrent cabozantinib/RT was feasible and demonstrated an acceptable safety profile for patients with extremity STS.
Single-Cell RNA Sequencing Reveals the Cellular and Molecular Differences Between Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Objective: Myxofibrosarcoma (MXF) and undifferentiated pleomorphic sarcoma (UPS) are common and aggressive subtypes of cancer differing by clinical characteristics and prognosis; however, their differential diagnosis is difficult. Elucidation of cellular and transcriptomic discrepancies between these diseases that could improve their identification was the aim of our study. Methods: We applied single-cell RNA sequencing to compare MXF and UPS by tumor cell clusters and cell-cell ligand-receptor interactions, using five tumor samples of both subtypes. Results: We identify nine major cell types in all tumors analyzed. Any significant differences in their proportions between MXF and UPS were not found. Further reclusterization of lymphoid cells showed that cytotoxic CD8+ T cell proportion was higher in the MXF samples. In UPS cancer cells, the pathways maintaining extracellular matrix components (including collagens, proteoglycans, and other proteins) were highly active, while MXF cells were characterized by high activity of growth factors and angiogenesis pathways. The ligand-receptor interactions between cancer cells and the microenvironment differed significantly between MXF and UPS. In UPS, CD80 of dendritic cells and macrophages prominently interacted with T cell co-inhibitory CTLA-4 receptors, whereas the activating CD80-CD28 interaction was predominant in MXF. Moreover, in UPS, CD44 and integrins of cytotoxic CD8+ T cells prominently interacted with COL1A1/2, while in MXF CD44, interaction with FN1, COL6A1, and LAMC1 prevailed. Conclusions: Differences were identified between UPS and MFS in the composition of lymphoid cell populations and in the intercellular interactions. This proposes deeper understanding of the biological differences between these sarcoma subtypes and may be important for the development of new therapeutic approaches, although further validation of the findings is required.
Immunoreactivity for CD34, Desmin, Keratins, KIT, Alpha-Smooth Muscle Actin, S100, and Vimentin in Malignant Mesenchymal Neoplasms in Guinea Pigs: A Series of 62 Cases From a Single Institution.
There has been a lack of research regarding diagnostic immunohistochemistry in soft tissue pathology in rodents. We aimed to analyse the immunohistochemical expression of CD34, keratin AE1/AE3, keratin 8/18, desmin, KIT, SMA, S100, and vimentin in various malignant mesenchymal tumours in guinea pigs. A total of 62 tumours included 10 fibrosarcomas, 8 myxosarcomas, 8 undifferentiated pleomorphic sarcomas, 6 undifferentiated round cell sarcomas, 6 dedifferentiated liposarcomas (involving deep soft tissue, skin or subcutis), 12 uterine leiomyosarcomas, 4 hemangiosarcomas (involving skin and spleen), and 8 gastrointestinal stromal tumours (GIST). Desmin and SMA positivity was present in tumours of various lineages, including hemangiosarcoma. CD34 positivity was restricted to hemangiosarcomas, GISTs and one case of undifferentiated pleomorphic sarcoma, whereas KIT positivity was detected only in GISTs. Lesser amounts of keratins positivity were observed in fibrosarcoma, myxosarcoma, undifferentiated pleomorphic sarcoma, and dedifferentiated liposarcoma. S100 positivity was identified in myxosarcoma, undifferentiated pleomorphic sarcoma, and dediffentiated liposarcoma and vimentin positivity was detected in all tumour types. The strong and diffuse staining pattern of desmin and SMA can be very helpful in distinguishing leiomyosarcoma from its spindle cell mimics. A novel finding is that SMA positivity was identified in malignant endothelium. Immunolabelling for CD34 and KIT provides reliable markers for vascular neoplasms and GISTs. Vimentin does not allow to distinguish between different mesenchymal malignancies in guinea pigs. Occasional positivity for keratins and S100 points to potential pitfalls and emphasised the need for a panel of immunohistochemical stains in the investigation of mesenchymal tumours.
A Japanese Multicenter Database Study of Retroperitoneal Sarcoma: Outcomes of Patients Receiving Eribulin Therapy.
Clinical evidence on disease characteristics and outcomes of retroperitoneal sarcoma (RPS) remains limited. Although eribulin was approved for soft tissue sarcomas in Japan in 2016, real-world data on its use for advanced RPS are scarce. We investigated treatment patterns and survival outcomes of patients with RPS, with a particular focus on those receiving eribulin. The database included 118 patients pathologically diagnosed with RPS between January 2000 and August 2025. Clinicopathologic data, including inflammation/nutrition markers and survival outcomes, were analyzed. Among them, 102 (86%) underwent surgical resection of the primary tumor, with a median recurrence-free survival of 20 months and a median overall survival of 9.2 years. Well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, and undifferentiated pleomorphic sarcoma were the most frequent histologic subtypes. Non-well-differentiated liposarcoma histology, Stage IIIA-IIIB, lower C-reactive protein-albumin-lymphocyte index, and higher modified neutrophil-platelet score were associated with poor postoperative prognosis. Of 44 patients with unresectable RPS who received systemic therapy, 23 (52%) were treated with eribulin. In this group, complete response, partial response, and stable disease rates were 4.3%, 26%, and 35%, respectively. The most common adverse events were decreased neutrophil count (40%), peripheral neuropathy (30%), and dysgeusia (20%). Multivariate analysis showed that non-use of eribulin and undifferentiated pleomorphic sarcoma were independently associated with worse survival in unresectable disease. We separately described outcomes and treatment patterns of patients with resectable and unresectable RPS. Our findings suggest that eribulin should be considered a treatment option for unresectable RPS in clinical practice.
Publicações recentes
ATRX loss in sarcomas is associated with dysregulated gene and transposable element expression, loss of DNA methylation, and worse survival.
Mesenchymal Tissue-Driven Gene Programs Identify EMP3 as a Key Biomarker of Aggressiveness in Undifferentiated Sarcomas.
Targeting eIF4A-dependent translation in genetically complex sarcoma.
Gene Expression Profile (GEP) Comparison of Atypical Fibroxanthoma (AFX) and Pleomorphic Dermal Sarcoma (PDS).
Tumor Size Modifies the Survival Benefit of Chemotherapy in Localized Soft Tissue Sarcomas: A Propensity-Matched Cohort Study.
📚 EuropePMC445 artigos no totalmostrando 200
Wide resection and reconstruction using the sandwich technique for primary undifferentiated pleomorphic sarcoma of the sternum: a case report.
General thoracic and cardiovascular surgery casesTreatment paradigms in undifferentiated pleomorphic sarcoma of bone: lessons learned from osteosarcoma and questions that remain commentary on: Bielack SS et al. J Cancer Res Clin Oncol (2026).
Journal of cancer research and clinical oncologyDistinct immunologic patterns of response and resistance to anti-PD-1/PD-L1-based immunotherapy in patients with soft tissue sarcoma.
Frontiers in immunologyProteomic landscape analysis of undifferentiated pleomorphic sarcoma.
Genome medicineSevere mitral stenosis due to primary cardiac intimal sarcoma: case report.
European heart journal. Case reportsCase Report: A rare case of primary undifferentiated pleomorphic sarcoma of the renal pelvis with high PD-L1 expression and a misleading positive urine FISH.
Frontiers in immunologyRevisiting the Immune Frontier in Soft Tissue Sarcomas.
Current oncology reportsSafety and Efficacy of Epirubicin, Ifosfamide, and Nivolumab as First-line treatment for patients with Undifferentiated Pleomorphic Sarcoma.
Clinical cancer research : an official journal of the American Association for Cancer ResearchRadiation-Associated Sarcoma in a Resource-Constrained Setting: A Decade of Real-World Outcomes From a Tertiary Care Sarcoma Center in India.
JCO global oncologyUndifferentiated testicular pleomorphic sarcoma: A case report.
World journal of clinical casesRed Bumps, Red Flags: A Case Series of Four Neoplasms Masquerading as Inflamed Cutaneous Cysts.
CureusPerformance of deep learning-based segmentation of soft tissue sarcoma by MRI sequence, tumor type and location.
Skeletal radiologyA Phase 1 Study of Neoadjuvant Cabozantinib in Combination With Radiation Therapy for Sarcomas of the Extremities.
American journal of clinical oncologyCase Report: Complete response to the concurrent neoadjuvant radiation therapy and pembrolizumab in a locally recurrent, chemotherapy-refractory undifferentiated pleomorphic sarcoma of bone.
Frontiers in oncologyClinical Characteristics of Complex Karyotype Soft Tissue Sarcomas: A Single-Institution Cohort Study.
Medicina (Kaunas, Lithuania)Molecular epidemiology of the expression of urokinase plasminogen activator receptor-associated protein (uPARAP) in mesenchymal malignancies.
Translational oncologyUndifferentiated High-Grade Pleomorphic Sarcoma of the Posterior Mandible: A Report of a Rare Case.
CureusCommentary: Undifferentiated pleomorphic sarcoma of the adrenal gland: a case report and literature review.
Frontiers in oncologySingle-Cell RNA Sequencing Reveals the Cellular and Molecular Differences Between Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
Medical sciences (Basel, Switzerland)Malignant transformation of fibrous dysplasia in long bones: a clinicopathological and molecular study of 19 cases.
Journal of clinical pathologyPATIENTS WITH SOFT TISSUE SARCOMA AFTER TREATMENT BY NON ORTHOPEDIC ONCOLOGIC SURGEONS: EPIDEMIOLOGICAL PROFILE, STAGING, AND THERAPEUTIC CHALLENGES.
Acta ortopedica brasileiraPathological Study of Rare Malignant Cardiac Tumors: A Case Series of Five Patients.
Case reports in pathologyTranscriptomic subgroups in soft tissue tumors correlate with morphologic subtype, genomic features, and outcome.
Clinical cancer research : an official journal of the American Association for Cancer ResearchAdenosine A2B Receptor Promotes Tumor Progression and Metastases in Undifferentiated Pleomorphic Sarcoma.
Clinical cancer research : an official journal of the American Association for Cancer ResearchImmunoreactivity for CD34, Desmin, Keratins, KIT, Alpha-Smooth Muscle Actin, S100, and Vimentin in Malignant Mesenchymal Neoplasms in Guinea Pigs: A Series of 62 Cases From a Single Institution.
Veterinary medicine and scienceIntraoperative MRI for giant cell tumor of bone after denosumab and other selected tumors: A pilot study.
Acta radiologica openA Japanese Multicenter Database Study of Retroperitoneal Sarcoma: Outcomes of Patients Receiving Eribulin Therapy.
Asia-Pacific journal of clinical oncologyRetroperitoneal undifferentiated pleomorphic sarcoma with aortic and inferior vena cava involvement presenting as right common iliac artery rupture.
Journal of vascular surgery cases and innovative techniquesReal-World Implementation of Next-Generation Sequencing in Sarcoma: Molecular Insights and Therapeutic Outcomes.
Medical sciences (Basel, Switzerland)Undifferentiated pleomorphic sarcoma of bone (UPSB) treated in the German-speaking countries. A report of 132 unselected patients from the Cooperative Osteosarcoma Study Group (COSS).
Journal of cancer research and clinical oncologyA Rare Case of Recurrent Sarcoma Presenting as an Anterior Mediastinal Mass: Case Report.
Case reports in oncologyA retrospective study of post-radiation sarcoma from three institutions in Japan with a review of the literature.
Nagoya journal of medical sciencePathologic Assessment of Retroperitoneal Sarcomas: A Position Paper By the Transatlantic Australasian Retroperitoneal Sarcoma Working Group.
The American journal of surgical pathologyTargeted RNA sequencing enhances the integrated diagnosis of bone and soft tissue tumors.
Human pathologyCombined Use of a Pedicled Medial Sural Artery Perforator Flap and a V-Y Advancement Flap Based on an Anterior Tibial Artery Perforator for Pretibial Defect Reconstruction: A Case Report.
MicrosurgeryManagement of intracranial undifferentiated pleomorphic sarcoma: a systematic review.
Neurosurgical reviewDual role of IFNγ in reprogramming the undifferentiated pleomorphic sarcoma cell line JBT19 towards cytotoxic chemotherapy and antitumor immunity.
Oncology lettersPrimary Cardiac Sarcomas: Clinical Characteristics, Management, and Outcomes at a Spanish National Reference Center.
CancersProminent repair-like changes that mimic atypical squamous cells after radiation treatment in the lung: A report of two cases.
CytoJournalApplying Radiomics to Predict Outcomes in Patients with High-Grade Retroperitoneal Sarcoma Treated with Preoperative Radiotherapy.
Journal of imagingOsteogenesis Imperfecta Type V With Undifferentiated Pleomorphic Sarcoma: A Rare Occurrence.
Pediatric blood & cancerClinicopathological and genomic profiling in undifferentiated pleomorphic sarcoma: Small series, clear message.
Journal of applied geneticsThe prognostic value of systemic inflammatory response index (SIRI) and CD8 + tumor-infiltrating lymphocytes for patients with localized undifferentiated pleomorphic sarcoma of soft tissue.
BMC cancerDelayed Treatment Associated With Worse Survival in High-grade Undifferentiated Pleomorphic Sarcoma.
The Journal of surgical researchDistal Upper Extremity Sarcoma Epidemiology: A Single-Institution Review.
Hand (New York, N.Y.)Pericardial Sarcoma of Uncertain Origin in a Young Cat: A Case Report Highlighting Diagnostic Challenges and Thoracic Drainage in Palliative Management.
Animals : an open access journal from MDPIThe Immune Landscape of Canine Soft Tissue Sarcomas as a Model for Human Soft Tissue Sarcomas.
CancersSingle-Cell Sequencing Reveals Novel Tumor Populations and Their Interplay with the Immune Microenvironment in a Pleomorphic Rhabdomyosarcoma.
International journal of molecular sciencesATRX loss in sarcomas is associated with dysregulated gene and transposable element expression, loss of DNA methylation, and worse survival.
medRxiv : the preprint server for health sciencesHigh-throughput screening identifies the activity of histone deacetylase inhibitors in patient-derived models of soft tissue sarcoma.
Cancer biology & therapyGenetic Heterogeneity of Undifferentiated Pleomorphic Sarcoma: Is There Potential for Targeted Therapy?
CancersProfiling the genomic landscape and evolutionary history of polyploid giant cancer cells in undifferentiated pleomorphic sarcomas.
Cancer lettersPembrolizumab combined with surgical treatment for spontaneously ruptured undifferentiated pleomorphic sarcoma of the liver: a case report and literature review.
Frontiers in immunologyPeriacetabular Resection for Bone Tumors: Is There Still a Role for Massive Allograft-prosthesis Composite Reconstructions?
Clinical orthopaedics and related researchDistinct Sarcoma Microenvironments Predict Benefit from Addition of Pembrolizumab to Preoperative Radiotherapy and Surgery in SU2C-SARC032.
medRxiv : the preprint server for health sciencesA signal-seeking phase 2 study of tremelimumab in advanced cancers with high tumour mutational burden.
NPJ precision oncologyASO Visual Abstract: Advancing Prognostics in Oncology: Developing a Machine Learning Model for Predicting 2-Year and 5-Year Survival Rates in Patients with Undifferentiated Pleomorphic Sarcoma.
Annals of surgical oncology[Effectiveness evaluation of three-dimensional printed customized hemi-pelvic prosthesis for pelvic reconstruction after resection of massive pelvic tumor].
Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgeryIntracranial undifferentiated pleomorphic sarcoma of right frontal lobe: a case report.
Discover oncologyOncolytic measles virotherapy encoding the neutrophil-activating protein is effective in synovial sarcoma.
Molecular therapy. OncologyEctopic Adrenocorticotropic Hormone Syndrome Secondary to Undifferentiated Pleomorphic Sarcoma of the Thigh: A Rare Paraneoplastic Manifestation.
CureusDeep-Learning Tool ScVital Enables Species-Agnostic Integration of Cancer Cell States.
Cancer researchImaging Findings of Atypical Spindle Cell/Pleomorphic Lipomatous Tumors: A Case Series and Systematic Review.
CureusA Rare Case of High-Grade Undifferentiated Pleomorphic Sarcoma of the Terminal Ileum in a 40-Year-Old Man: Successful Outcome and Prolonged Survival After Surgical Resection and Adjuvant Chemotherapy.
CureusHigh-Grade Undifferentiated Pleomorphic Sarcoma of the Tongue: A Case Report.
CureusImpact of Proximity to an Academic Health Center on Adherence to the National Comprehensive Cancer Network (NCCN) Guidelines in Patients With Soft Tissue Sarcomas.
CureusIncidence 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 journalOrthopedic Surgical Management of Soft Tissue Sarcomas in a Referral Center: 5-Year Oncological Outcomes.
Orthopaedics & traumatology, surgery & research : OTSRA rare case of cellular angiofibroma with sarcomatous transformation occurred in the popliteal fossa.
Discover oncologySalvage resection after immunotherapy for giant undifferentiated pleomorphic sarcoma of the chest wall.
Journal of surgical case reportsIs the Thickness of the Margin Associated with Local Recurrence and Survival in Patients with Undifferentiated Pleomorphic Sarcoma?
Medicina (Kaunas, Lithuania)Succinate Dehydrogenase B (SDHB) Overexpression with Enzymatic Dysfunction Defines a Distinct Subtype of Undifferentiated Pleomorphic Sarcoma.
Cancer research communicationsAtypical Fibroxanthoma: Trends and Insights From a Single Tertiary Care Institution Over a Ten-Year Span.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Aortic Pleomorphic Sarcoma Presenting With Claudication: A Rare Diagnostic Challenge.
Vascular and endovascular surgeryThe prognostic potential of circulating biomarkers for sarcoma patients with pleural dissemination.
Pathology oncology research : PORThe value of morphology: osteoclast-like cells in soft tissue tumours.
Pathology oncology research : PORImaging findings for differentiating and predicting prognosis in undifferentiated pleomorphic sarcoma and myxofibrosarcoma.
European journal of radiologyASO Author Reflections: Advancing Prognostics in Oncology: Developing a Machine Learning Model for Predicting 2-Year and 5-Year Survival Rates in Patients with Undifferentiated Pleomorphic Sarcoma.
Annals of surgical oncologyThe clinical outcomes of joint-preserving endoprosthesis reconstruction for primary sarcomas about the knee.
Journal of orthopaedic surgery and researchDeconstructing the prevalence of undifferentiated pleomorphic sarcoma across socio-demographic groups: a potential launchpad for future research.
Dermatology online journalCharacteristics and Outcomes of Soft Tissue Sarcoma in Saudi Arabia: A 10-Year Experience.
Cancer management and researchThe levels of circulating tumor DNA and inflammatory proteins depict the clinical response in a patient with metastatic undifferentiated pleomorphic sarcoma, a case report.
Acta oncologica (Stockholm, Sweden)Advancing Prognostics in Oncology: Developing a Machine Learning Model for Predicting 2-Year and 5-Year Survival Rates in Patients with Undifferentiated Pleomorphic Sarcoma.
Annals of surgical oncologyEpidemiology and Outcomes of Retroperitoneal Sarcoma: An Analysis of the Population-Based Cancer Registry in Japan 2016-2019.
Journal of surgical oncologyImmunotherapy in the Treatment of Undifferentiated Pleomorphic Sarcoma and Myxofibrosarcoma.
Current treatment options in oncologyPrimary Cardiac Sarcoma: A Rare Case With Superior Vena Cava Syndrome.
In vivo (Athens, Greece)Priming the tumor microenvironment with pembrolizumab and radiotherapy for durable cures in high-risk localized undifferentiated pleomorphic sarcoma and pleomorphic/dedifferentiated liposarcoma.
Journal for immunotherapy of cancerA real-world study on the expression and clinical application of IMP3 immunohistochemistry in bone tumors.
Human pathologyCase Report: A rare case of hepatic undifferentiated pleomorphic sarcoma: diagnostic dilemmas and multidisciplinary management strategies.
Frontiers in oncologyCase Report: Three-year follow-up of TACE for recurrent undifferentiated pleomorphic sarcoma: sustained local control and no systemic progression.
Frontiers in oncologyGTSE1-expressed osteoblastic cells facilitate formation of pro-metastatic tumor microenvironment in osteosarcoma.
Genes & diseasesPrimary undifferentiated pleomorphic sarcoma of biceps femoris.
Indian journal of cancerA rare cause of gastrointestinal bleeding and intussusception in a patient with undifferentiated pleomorphic sarcoma.
Polish archives of internal medicineBudd-Chiari syndrome from pleomorphic sarcoma compressing the inferior vena cava: A case report.
Radiology case reportsCase Report: Malignant transformation of maxillary giant cell tumor of bone from EURACAN reference center.
Frontiers in oncologyA case of cardiac undifferentiated pleomorphic sarcoma treated with post-operative radiotherapy followed by heart transplantation.
Cardiovascular pathology : the official journal of the Society for Cardiovascular PathologyUndifferentiated Pleomorphic Sarcoma of Laryngeal Ventricle: A Case Report.
Oman medical journalOral Undifferentiated Pleomorphic Sarcoma: A Novel SPECC1L::TERT Gene Fusion and a Comprehensive Literature Review.
GenesTransoral Videolaryngoscopic Surgery for an Undifferentiated Pleomorphic Sarcoma of the Tongue Base: A Case Report.
Reports (MDPI)A Review of Intracardiac Mass Cases Encountered Over a Two-Year Period.
CureusUndifferentiated Pleomorphic Sarcoma: A Diagnosis of Exclusion.
CureusOptimal Factors That Influence the Establishment of Successful Primary Human Soft-Tissue Sarcoma Cell Lines.
Clinical cancer research : an official journal of the American Association for Cancer ResearchPrimary leiomyosarcoma of bone: a clinicopathologic and immunohistochemical study of 142 cases.
PathologicaA rare presentation of a cutaneous metastasis from an undifferentiated pleomorphic sarcoma.
Journal of cancer research and therapeuticsDivergent therapeutic and prognostic impacts of immunogenic features in undifferentiated pleomorphic sarcoma and myxofibrosarcoma.
Cancer immunology, immunotherapy : CIIPerioperative Systemic Therapy in Rare, Chemosensitive Subtypes of Retroperitoneal Sarcoma: A Hospital-Based Propensity Score-Matched Analysis.
CancersHyalinization-based pathologic response and immune infiltration following neoadjuvant radiotherapy with or without immune-checkpoint blockade in localized undifferentiated pleomorphic sarcoma.
ESMO openSynergistic Effects of ATR Inhibition and Lurbinectedin in Soft-Tissue Sarcomas: The Predictive Role of SLFN11 Expression.
Clinical cancer research : an official journal of the American Association for Cancer ResearchEvaluation of PRDM10 gene rearrangement by immunohistochemistry and molecular methods in unclassifiable undifferentiated soft tissue tumors.
Medical molecular morphologyIsolated Limb Perfusion Can Avert Amputation Indication in Initially Nonsalvageable Sarcomas of the Extremities.
Clinical orthopaedics and related researchOral Soft Tissue and Jawbone Sarcomas: A Retrospective Clinicopathologic Analysis of 128 Cases from Two Institutions and Comprehensive Literature Review.
Head and neck pathologyMolecular Characterization of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma.
CancersThe role of whole-genome sequencing for guiding systemic therapy in patients with soft tissue sarcoma.
ESMO open[Gastric Undifferentiated Pleomorphic Sarcoma Presenting as a Rapidly Growing Subepithelial Tumor].
The Korean journal of helicobacter and upper gastrointestinal researchPrognostic factors and the impact of neoadjuvant chemotherapy on the development of metastasis in patients with undifferentiated pleomorphic sarcoma of soft tissue.
International journal of clinical oncologySevere immunotherapy-related thrombocytopenia in metastatic bone cancer: a multicenter retrospective case series highlighting early recognition and management.
Frontiers in oncologyReal-world data on immune checkpoint inhibitors in advanced sarcomas across multiple European institutions.
Acta oncologica (Stockholm, Sweden)Incidence, risk factors, and prognostic impact of regional lymph node metastasis in bone sarcoma: a population-based cohort study.
Japanese journal of clinical oncologyUndifferentiated pleomorphic sarcoma invading thoracic aorta treated by thoracic endovascular aortic repair: a case report.
Journal of medical case reportsUndifferentiated Pleomorphic Sarcoma With Mismatch Repair Deficiency Arising From the Heart.
JACC. Case reportsEfficacy of toripalimab in combination with anlotinib in recurrent undifferentiated pleomorphic sarcoma of the sinonasal region: a case report with biomarker analysis.
Frontiers in immunologyGenomics-led approach to drug testing in models of undifferentiated pleomorphic sarcoma.
Molecular oncologyTransdifferentiation of Hairy Cell Leukemia to Lytic and Mass-Producing Histiocytic Sarcoma: A Novel Head and Neck Example and Review of the Literature.
Head and neck pathologyDoes Size Outweigh Number in Predicting Survival After Pulmonary Metastasectomy for Soft Tissue Sarcoma? Insights from a Retrospective Multicenter Study.
Annals of surgical oncologyCurrent and emerging systemic treatment options for malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma.
International journal of clinical oncologyRare Case of Grade 3 Undifferentiated Pleomorphic Sarcoma in Left Atrium, Mitral Valve, and Papillary Muscle.
Journal of clinical medicineTwo Simultaneous Primary Cancers: A Case Report.
CureusUndifferentiated Pleomorphic Sarcoma of the Prostate, a Rare Cause of Genitourinary Tumour in Men.
Archivos espanoles de urologiaBuilding a pre-surgical multiparametric-MRI-based morphologic, qualitative, semiquantitative, first and high-order radiomic predictive treatment response model for undifferentiated pleomorphic sarcoma to replace RECIST.
Cancer imaging : the official publication of the International Cancer Imaging SocietyDiagnosis and Management of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma in a Large Institutional Cohort.
Journal of cutaneous medicine and surgeryStatistics of Primary Malignant Spine Tumors in Japan: A Retrospective Study Using the Bone and Soft Tissue Tumor Registry in Japan.
SpineThe outcomes and treatment strategies in metastatic soft tissue sarcoma treated with immunotherapy-based therapy: a three-center study.
Frontiers in immunologyUndifferentiated Pleomorphic Sarcoma in Children and Young Adults: A Comprehensive Clinicopathologic, Genomic, and Epigenetic Comparison With Adult Counterparts.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncRecurrence Screening of Undifferentiated Pleomorphic Sarcoma with Giant Cells of the Breast.
Clinical laboratoryA Rare Intruder of the Liver: A Case of Undifferentiated Pleomorphic Sarcoma.
CureusRadiation-induced sarcoma after radiotherapy for breast cancer: A retrospective case series.
JPRAS openDNA Mismatch Repair Deficiency as a Biomarker in Sarcoma.
Surgical oncology insightMethylation Analysis Reveals Epigenetic Congruence Between Bone Sarcomas With H3-3A Mutations and Malignant Giant Cell Tumors of Bone.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncCRE25-037: Paraneoplastic Syndrome in Undifferentiated Pleomorphic Sarcoma Demonstrates Acquired Antithrombin III Deficiency as Risk Factor for Cancer-Related Morbidity.
Journal of the National Comprehensive Cancer Network : JNCCNExpression of Cancer-Testis Antigens MAGE-A1, MAGE-A4, NY-ESO-1 and PRAME in Bone and Soft Tissue Sarcomas: The Experience From a Single Center in China.
Cancer medicineBlue Light Emitting Diode Suppresses Sarcoma Cell Proliferation via the Endogenous Apoptotic Pathway Without Damaging Normal Cells.
Cancer medicine[The expert consensus on the diagnosis and treatment of skull base and head-neck sarcomas (2024 edition)].
Zhonghua zhong liu za zhi [Chinese journal of oncology]LDH and hemoglobin outperform systemic inflammatory indices as prognostic factors in patients with soft tissue sarcoma undergoing neoadjuvant treatment.
BMC cancerComplete Resection of Mediastinal Undifferentiated Pleomorphic Sarcoma.
Annals of thoracic surgery short reportsParadoxical Response to Neoadjuvant Therapy in Undifferentiated Pleomorphic Sarcoma: Increased Tumor Size on MRI Associated with Favorable Pathology.
CancersRadiation-Induced Undifferentiated Pleomorphic Sarcoma in Thyroid: A Rare Occurrence.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaSubcutaneous undifferentiated pleomorphic sarcoma is more aggressive than pleomorphic dermal sarcoma: Prognosis from a Danish nationwide registry-based cohort.
European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical OncologyRedefining radiologic responses in high-risk soft-tissue sarcomas treated with neoadjuvant chemotherapy: final results of ISG-STS 1001, a randomized clinical trial.
ESMO openImpact of Tertiary Lymphoid Structure on Prognosis and Tumor Microenvironment in Undifferentiated Pleomorphic Sarcoma.
Cancer scienceLaparoscopic Abdominoperineal Resection of Undifferentiated Spindle Cell Sarcomas of the Rectum with Lymph Node Metastases: A Rare Case Report.
Surgical case reportsMalignant fibrous histiocytoma of the mandible: A rare case report with diagnostic challenge and treatment approach.
Journal of oral and maxillofacial pathology : JOMFPGlucose Transporter 1 (GLUT1) Positivity in Ganglion Cysts: A Diagnostic Caveat Due to Overlap With Perineuriomas and Other Fibromyxoid Lesions.
CureusAnlotinib versus Placebo as Adjuvant Therapy for Localized High-Grade Soft-Tissue Sarcomas: A Phase II, Double-Blinded, Randomized Controlled Trial.
Clinical cancer research : an official journal of the American Association for Cancer ResearchClinicopathological background of local recurrence in high grade sarcoma of the extremity with preoperative chemotherapy: a supplementary analysis of JCOG0304.
Japanese journal of clinical oncologySpontaneous Undifferentiated Pleomorphic Sarcoma With Mast Cell Infiltration in a Sprague-Dawley Rat: A Case Report.
Veterinary medicine and scienceNeglected Giant Scalp Tumors: A Report of Two Cases With an Analysis of Delay Factors.
CureusLarge retroperitoneal mass: A case of an undifferentiated pleomorphic sarcoma.
Radiology case reportsHistological Type-Specific Behavior in Sarcomas: Analysis of Head and Neck Cancer Registry of Japan.
The LaryngoscopeNovel Use and Value of Contrast-Enhanced Susceptibility-Weighted Imaging Morphologic and Radiomic Features in Predicting Extremity Soft Tissue Undifferentiated Pleomorphic Sarcoma Treatment Response.
JCO clinical cancer informaticsUndifferentiated pleomorphic sarcoma in the maxillary sinus: a case report.
Journal of medical case reportsUndifferentiated Pleomorphic Cardiac Sarcoma: A Rare and Often Fatal Diagnosis.
JACC. Case reportsEvolutionary Pressures Shape Undifferentiated Pleomorphic Sarcoma Development and Radiotherapy Response.
Cancer researchNovel Therapeutics in Soft Tissue Sarcoma.
CancersTreatment of a Radiation-Associated Femur Fracture with an Intercalary Endoprosthesis: A Case Report.
JBJS case connectorAssessing the Utility of DNA Methylation Profiling in the Classification of Myogenic Sarcomas of Bone.
International journal of surgical pathologySarcomas developed in patients with Lynch Syndrome are enriched in pleomorphic soft-tissue sarcomas and are sensitive to immunotherapy.
European journal of cancer (Oxford, England : 1990)Monitoring Response Using Circulating Tumor DNA in Undifferentiated Pleomorphic Sarcoma: A Case Report.
CureusDurvalumab and tremelimumab in patients with advanced rare cancer: a multi-centre, non-blinded, open-label phase II basket trial.
EClinicalMedicineAmong the Masses: Multiple Left Atrial Undifferentiated Pleomorphic Sarcomas.
Ochsner journalHeterogeneity in response to neoadjuvant radiotherapy between soft tissue sarcoma histotypes: associations between radiology and pathology findings.
European radiologyHistologic and Immunologic Factors Associated with Response to Immune Checkpoint Inhibitors in Advanced Sarcoma.
Clinical cancer research : an official journal of the American Association for Cancer ResearchDecoding Chemotherapy Resistance of Undifferentiated Pleomorphic Sarcoma at the Single Cell Resolution: A Case Report.
Journal of clinical medicineDual inhibition of BET and EP300 has antitumor activity in undifferentiated pleomorphic sarcomas and synergizes with ferroptosis induction.
Translational oncologyMultimodality imaging challenge: differentiating a pleiomorphic sarcoma of the left atrial appendage from a thrombus.
European heart journal. Imaging methods and practiceHigh-grade undifferentiated pleomorphic sarcoma of the left elbow diagnosed by ultrasound: A case report.
Asian journal of surgery[Clinical characteristics of undifferentiated pleomorphic sarcoma from the head and neck].
Zhonghua yi xue za zhiAbscopal effect-induced spontaneous regression of distant metastases in malignant mesenchymal tumor: a case report.
Frontiers in oncologyUndifferentiated Pleomorphic Sarcoma: A Case Report.
CureusTwo Cases of Intraocular Undifferentiated Pleomorphic Sarcoma.
Ocular oncology and pathologyA unique tripartite collision tumor of the esophagus: a case report and literature review.
Frontiers in oncologyThe Association of Leser-Trélat Sign with Cutaneous Undifferentiated Pleomorphic Sarcoma.
Indian journal of dermatologySarcoma: Last Year's Practice Changing Papers.
Annals of surgical oncology[A Case of Retroperitoneal Primary Undifferentiated Pleomorphic Sarcoma with a Rapid Course].
Hinyokika kiyo. Acta urologica JaponicaUndifferentiated pleomorphic sarcoma of the adrenal gland: a case report and literature review.
Frontiers in oncologyBrain Metastases in Sarcomas: A Multicenter Retrospective Cohort Study.
CancersDysregulation of miRNAs in Soft Tissue Sarcomas.
CellsUndifferentiated Pleomorphic Sarcoma of the Proximal Thigh With Neoplastic Fever and Metachronous Sternal Metastases: A Case Report and Review of the Literature.
CureusDescriptive analysis of incidence, demographic characteristics, and survival outcomes of soft-tissue sarcoma of the extremities in Saudi Arabia.
Scientific reportsNeoadjuvant immunotherapy in the evolving landscape of sarcoma treatment.
Journal for immunotherapy of cancerThe survival, mechanisms of failure, and function after total humeral replacement in patients with primary sarcomas.
Journal of shoulder and elbow surgeryResults of an Integrated Phase I/II Prospective Clinical Trial (NEXIS) for Neoadjuvant Anti-PD-L1 (Durvalumab) and Anti-CTLA-4 (Tremelimumab) With Radiation for High-Risk Soft-Tissue Sarcoma of the Trunk and Extremities.
CureusLow-Level MDM2 Amplification by FISH: An Institutional Experience With a Diagnostic Dilemma.
International journal of surgical pathologySingle-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma.
Molecular cancerSafety and efficacy of pembrolizumab, radiation therapy, and surgery versus radiation therapy and surgery for stage III soft tissue sarcoma of the extremity (SU2C-SARC032): an open-label, randomised clinical trial.
Lancet (London, England)Nivolumab and sunitinib in patients with advanced bone sarcomas: A multicenter, single-arm, phase 2 trial.
CancerMalignant sarcomatous transformation of calvarial fibrous dysplasia: illustrative case.
Journal of neurosurgery. Case lessonsIs intercalary frozen autograft augmented with intramedullary cement and bridging plates fixation a durable reconstruction?
Journal of orthopaedic surgery and researchA Rare Case of Primary Hepatic Undifferentiated Pleomorphic Sarcoma: Exploring Cancer-related Gene Mutations.
Internal medicine (Tokyo, Japan)PTEN pathogenic variants are associated with poor prognosis in patients with advanced soft tissue sarcoma.
BJC reportsCT and MR imaging of a huge retroperitoneal synovial sarcoma: A case report.
Radiology case reportsAssociações
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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.
- Severe mitral stenosis due to primary cardiac intimal sarcoma: case report.
- A Phase 1 Study of Neoadjuvant Cabozantinib in Combination With Radiation Therapy for Sarcomas of the Extremities.
- Single-Cell RNA Sequencing Reveals the Cellular and Molecular Differences Between Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma.
- Immunoreactivity for CD34, Desmin, Keratins, KIT, Alpha-Smooth Muscle Actin, S100, and Vimentin in Malignant Mesenchymal Neoplasms in Guinea Pigs: A Series of 62 Cases From a Single Institution.
- A Japanese Multicenter Database Study of Retroperitoneal Sarcoma: Outcomes of Patients Receiving Eribulin Therapy.
- ATRX loss in sarcomas is associated with dysregulated gene and transposable element expression, loss of DNA methylation, and worse survival.
- Mesenchymal Tissue-Driven Gene Programs Identify EMP3 as a Key Biomarker of Aggressiveness in Undifferentiated Sarcomas.
- Targeting eIF4A-dependent translation in genetically complex sarcoma.
- Gene Expression Profile (GEP) Comparison of Atypical Fibroxanthoma (AFX) and Pleomorphic Dermal Sarcoma (PDS).
- Tumor Size Modifies the Survival Benefit of Chemotherapy in Localized Soft Tissue Sarcomas: A Propensity-Matched Cohort Study.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:2023(Orphanet)
- MONDO:0002142(MONDO)
- GARD:6963(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q1887348(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
