O tumor desmoplásico de células redondas e pequenas (DSRCT) é um tipo agressivo de câncer de tecido mole que geralmente aparece nas membranas que revestem a barriga e a pelve (chamadas peritônio). Ele se espalha para o omento (uma membrana gordurosa dentro do abdômen), para os gânglios linfáticos e se dissemina pelo sangue, principalmente para o fígado. Em casos muito raros, a doença pode começar em outras partes do corpo, fora dessas membranas.
Introdução
O que você precisa saber de cara
O tumor desmoplásico de células redondas e pequenas (DSRCT) é um tipo agressivo de câncer de tecido mole que geralmente aparece nas membranas que revestem a barriga e a pelve (chamadas peritônio). Ele se espalha para o omento (uma membrana gordurosa dentro do abdômen), para os gânglios linfáticos e se dissemina pelo sangue, principalmente para o fígado. Em casos muito raros, a doença pode começar em outras partes do corpo, fora dessas membranas.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 7 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 18 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 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.
Transcription factor that plays an important role in cellular development and cell survival (PubMed:7862533). Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3' (PubMed:17716689, PubMed:25258363, PubMed:7862533). Regulates the expression of numerous target genes, including EPO. Plays an essential role for development of the urogenital system. It has a tumor suppressor as well as an oncogenic role in tumor formation. Function may be isoform-specific: isoforms lacking the KTS motif may a
NucleusNucleus, nucleolusCytoplasmNucleus speckleNucleus, nucleoplasm
Frasier syndrome
Characterized by a slowly progressing nephropathy leading to renal failure in adolescence or early adulthood, male pseudohermaphroditism, and no Wilms tumor. As for histological findings of the kidneys, focal glomerular sclerosis is often observed. There is phenotypic overlap with Denys-Drash syndrome. Inheritance is autosomal dominant.
Variantes genéticas (ClinVar)
1,677 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 2 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
4 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 — Tumor de células pequenas redondas desmoplásicas
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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
🟢 Recrutando agora
11 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
58 ensaios clínicos encontrados, 17 ativos.
Publicações mais relevantes
Update on pediatric soft tissue sarcomas.
The purpose of this review is to highlight recent findings in the diagnosis, biology, risk-stratification, and treatment of soft tissue sarcomas (STS) in children. In rhabdomyosarcoma (RMS), FOXO1 fusion status has been confirmed as an important prognostic factor. Among fusion-negative RMS, TP53 and MYOD1 mutations and detectable circulating tumor DNA at diagnosis are associated with inferior event-free survival in intermediate-risk disease. Delayed primary excision is associated with a reduced risk of local failure whereas radiotherapy dose escalation in large tumors has not improved local control. Maintenance therapy with vinorelbine and oral cyclophosphamide following induction chemotherapy in the RMS2005 trial led to improved survival. In non-rhabdomyosarcoma soft tissue sarcomas, the addition of pazopanib, a multitargeted receptor tyrosine kinase inhibitor, to upfront therapy did not improve survival. Atezolizumab is approved for alveolar soft part sarcoma, larotrectinib for NTRK fusion-positive STS, and afamitresgene autoleucel remains under evaluation in children with synovial sarcoma. Encouraging early results have been reported with tazemetostat and immune checkpoint inhibitors in epithelioid sarcoma and trastuzumab in desmoplastic small round cell tumor, respectively. Pediatric STS are rare and biologically heterogeneous. Genomic advances have refined risk stratification and uncovered therapeutic targets; further progress relies on international collaboration and trials.
Desmoplastic Small Round Cell Tumor Presenting as Bloody Ascites: A Case Report and Literature Review.
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive malignancy that predominantly affects young male patients. Its nonspecific clinical presentation and low diagnostic yield from ascitic fluid cytology pose a significant diagnostic challenge, particularly when presenting as ascites of unknown origin. A 30-year-old previously healthy young man presented with a 4-week history of progressive abdominal distention, intermittent fullness, unexplained weight gain, and bloody ascites. Cytology from paracentesis and tumor markers were nondiagnostic. Imaging revealed omental caking and multiple peritoneal nodules, suggestive of peritoneal carcinomatosis. Diagnostic laparoscopy identified extensive tumor seeding, and biopsy confirmed DSRCT based on histopathology, immunohistochemistry, and the presence of an EWS-WT1 fusion transcript on cytogenetic analysis, establishing the diagnosis of DSRCT. Chemotherapy was recommended; however, the patient's condition deteriorated rapidly, and he died within a few weeks before receiving treatment. DSRCT, although rare, is noted for aggressiveness and rapid progression and therefore should ideally be considered in the differential diagnosis of bloody ascites in young male patients, even when initial cytology is negative. Early application of diagnostic laparoscopy and biopsy may facilitate timely tissue diagnosis, particularly when imaging mimics peritoneal carcinomatosis.
Whole Abdominal Radiotherapy in Bone and Soft Tissue Sarcomas: Indications, Techniques, Clinical Outcomes, and Future Directions.
Managing bone and soft tissue sarcomas at high risk of intra-abdominal spread remains clinically challenging, and whole abdominal radiotherapy (WART) has become a pivotal locoregional component of multimodal therapy. For desmoplastic small round cell tumor (DSRCT)—the primary indication for WART—our practice prioritizes integrating WART with complete cytoreductive surgery and alkylator-based chemotherapy, with intensity-modulated radiotherapy (IMRT) or helical tomotherapy strongly recommended as preferred techniques due to their significant reduction in hematologic and gastrointestinal toxicity versus conventional 2D radiotherapy while preserving target coverage. For other subtypes (peritoneal rhabdomyosarcoma, Ewing sarcoma, myxoid liposarcoma), WART should be individualized: considered for confirmed peritoneal dissemination, residual disease post-chemotherapy, or incomplete resection, but avoided in extensive distant metastases. Future paradigms must optimize benefit-to-toxicity ratios, including routine proton/carbon ion therapy for pediatric/young adult patients to minimize late effects (e.g., growth abnormalities, infertility), exploration of WART combinations with targeted agents (e.g., IGF-1R inhibitors) or immunotherapies (e.g., PD-1/PD-L1 inhibitors) in prospective trials given promising preclinical/early clinical data, and use of molecular profiling and circulating tumor DNA (ctDNA) monitoring to guide patient selection and timely treatment adjustments. Long-term follow-up is essential for all patients to monitor late toxicities (e.g., small bowel obstruction, radiation nephritis) and secondary malignancies. In summary, WART is a cornerstone of curative-intent treatment for selected intra-abdominal sarcomas, but requires rigorous patient selection, advanced planning, and integration with systemic therapies, with continued international collaboration and trials critical to refining its role and improving outcomes for these rare, aggressive malignancies.
Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report With Atypical Morphologic Features and Literature Review.
Desmoplastic small round cell tumor (DSRCT), an aggressive malignancy classically seen in adolescents and young adults with a strong male predominance, is exceptionally rare in the ovary. We report a 33-year-old woman with ovarian DSRCT presenting as a pelvic mass and peritoneal metastases. Histopathology revealed small round cell nests within desmoplastic stroma, exhibiting atypical architectural patterns (single-file, follicular-like cystic, microcystic, and solid). Immunohistochemistry demonstrated coexpression of epithelial (pan-keratin) and mesenchymal markers (vimentin, desmin), while molecular analysis confirmed the EWSR1::WT1 gene fusion. Despite multimodal therapy, the patient died 24 months after surgery. A review of 23 ovarian DSRCTs proposed a classification: ovarian-dominant and extraovarian-dominant subtypes. Ovarian-dominant patients showed significantly prolonged median survival (24 vs 10 months, P = .026), underscoring the prognostic relevance of anatomical classification. Ovarian DSRCT poses diagnostic challenges due to histomorphological mimicry of common malignancies. Although this subclassification may aid prognostication, multicenter validation is warranted.
Fusion-positive soft tissue tumors: A selective review.
Even for experts, the diagnosis of soft tissue tumors remains challenging, owing to their rarity, striking diversity, and sometimes subtle morphological differences. Over the past decade, however, significant advances have been made in our collective understanding of the molecular genetic pathogenesis of these rare lesions, especially with the discovery that many are characterized by the presence of characteristic gene fusions, which can be exploited for diagnostic purposes. This review article focuses on four fusion-driven soft tissue tumors which illustrate different aspects of our evolving understanding of these tumors: (1) NUTM1-rearranged sarcoma, a prototypical example of a novel, clinically significant entity defined almost entirely by molecular genetics; (2) SRF-rearranged myoid neoplasm, an entity whose recognition greatly clarifies our understanding of pediatric "leiomyosarcomas;" (3) superficial FET-ETS neurocristic tumor, a very recently described, clinically benign entity sharing identical fusion events with often-lethal Ewing sarcoma; and (4) an evolving family of glomoid/myoid neoplasms harboring EWSR1::WT1 fusions, but clearly differing from desmoplastic small round cell tumor. These examples illustrate the complexity of fusion-driven soft tissue tumors and the importance of integrating molecular genetic testing with other clinicopathological data, rather than viewing it in isolation.
Publicações recentes
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📚 EuropePMC475 artigos no totalmostrando 197
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Current opinion in pediatricsManagement and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center.
BMC cancerUnresectable Desmoplastic Small Round Cell Tumor: A Case Report and Review of the Literature.
Case reports in oncologySmall Desmoplastic Round Cell Tumor of the Kidney following Hodgkin Lymphoma: Case Report and Literature Review.
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Virchows Archiv : an international journal of pathologyA Case of Desmoplastic Small Round Cell Tumor: Highlighting Diagnostic Challenges and Underscoring the Need for Standardized Treatment Protocols.
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Asian journal of surgeryIntra-abdominal omental mass as a desmoplastic round cell tumor: A rare case report.
Clinical case reportsDesmoplastic small round cell tumor of the abdomen: A case report.
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Asian journal of surgeryDesmoplastic small round cell tumor with ovarian involvement on [18F]-FDG PET/CT.
Revista espanola de medicina nuclear e imagen molecularPalliative Radiation Therapy in the Treatment of Desmoplastic Small Round Cell Tumors.
Cureus[Desmoplastic round cell tumor].
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Diagnostic pathologyProgression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment - Experience from a Major Pediatric Cancer Center in Europe.
Klinische PadiatrieMetastatic Pulmonary Desmoplastic Small Round Cell Tumor on FDG PET/CT.
Clinical nuclear medicineGenomic Breakpoint Characterization and Transcriptome Analysis of Metastatic, Recurrent Desmoplastic Small Round Cell Tumor.
SarcomaDesmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings.
Quantitative imaging in medicine and surgeryCXCR4-Directed Imaging and Endoradiotherapy in Desmoplastic Small Round Cell Tumors.
Journal of nuclear medicine : official publication, Society of Nuclear MedicineA Review of the Use of Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Malignancy in Pediatric Patients.
CancersSTK11 Adnexal Tumor in an Adolescent Female: Diagnostic Pitfalls of a Recently Described Entity.
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International journal of surgical pathologyDesmoplastic Small Round Cell Tumor Involving Serous Fluid: Cytologic Features and Diagnostic Pitfalls: A Series of 8 Cases.
American journal of clinical pathologyDissociation protocols used for sarcoma tissues bias the transcriptome observed in single-cell and single-nucleus RNA sequencing.
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Pediatric blood & cancerIntra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review.
Current oncology (Toronto, Ont.)Effectiveness of irinotecan plus trabectedin on a desmoplastic small round cell tumor patient-derived xenograft.
Disease models & mechanismsIdentification of immunotherapy and radioimmunotherapy targets on desmoplastic small round cell tumors.
Frontiers in oncologyImaging of pediatric abdominal soft tissue tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.
Pediatric blood & cancerDesmoplastic small round cell tumor: from state of the art to future clinical prospects.
Expert review of anticancer therapyLong-term survivors with desmoplastic small round cell tumor (DSRCT): Results from a retrospective single-institution case series analysis.
Cancer medicineExpanding the Spectrum of Tumors With EWSR::WT1 Gene Fusion by a Peritoneal Case That Is Not a Desmoplastic Small Round Cell Tumor.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncIncarcerated metastasis as the first manifestation of a rare sarcoma.
Rozhledy v chirurgii : mesicnik Ceskoslovenske chirurgicke spolecnostiMolecular profiling in desmoplastic small round cell tumours.
The international journal of biochemistry & cell biologyEstablishment of predictive nomogram and web-based survival risk calculator for desmoplastic small round cell tumor: A propensity score-adjusted, population-based study.
Biomolecules & biomedicineCytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignant tumors in children: Initial experience in a single institution.
Frontiers in surgeryTranscription regulators and ultra-rare and other rare translocation-related sarcomas treated with trabectedin: A proof of principle from a post-hoc analysis.
Frontiers in oncologyDesmoplastic small round cell tumor cancer stem cell-like cells resist chemotherapy but remain dependent on the EWSR1-WT1 oncoprotein.
Frontiers in cell and developmental biologyGiant desmoplastic small round cell tumor of the abdomen: A case report.
Frontiers in oncologyCategorizing Malignant Small Round Cell Tumors in Aspiration Cytology: An Institutional Experience.
Journal of microscopy and ultrastructureDesmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall.
CureusDesmoplastic small round cell tumor involving the uterine cervix: The first reported case in the literature, and brief review of gynecologic presentations.
Gynecologic oncology reportsPediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG).
Cancer management and researchDesmoplastic Small Round Cell Tumor of the Head and Neck: A Clinicopathological, Immunohistochemical and Molecular Analysis of Three Cases with Literature Review.
International journal of surgical pathologyAnesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.
Paediatric anaesthesiaMalignant rhabdoid tumor: Cyto-histologic correlation and immunohistochemical characterization of a rare pediatric malignancy and its differential diagnoses.
Annals of diagnostic pathologyLiver metastasis in soft tissue sarcoma at initial presentation.
Journal of surgical oncologyBeyond Wilms tumor: imaging findings and outcomes of rare renal tumors in children.
Pediatric radiologyChanging incidence and survival of desmoplastic small round cell tumor in the USA.
Proceedings (Baylor University. Medical Center)Extracellular Vesicles: A New Source of Biomarkers in Pediatric Solid Tumors? A Systematic Review.
Frontiers in oncologyLurbinectedin Inhibits the EWS-WT1 Transcription Factor in Desmoplastic Small Round Cell Tumor.
Molecular cancer therapeuticsThe androgen receptor is a therapeutic target in desmoplastic small round cell sarcoma.
Nature communicationsDesmoplastic Small Round Cell Tumor of the Uterus: A Report of Molecularly Confirmed Case with EWSR1-WT1 Fusion.
Diagnostics (Basel, Switzerland)Neuroendocrine and Rare Tumor Advances: A New and Promising TRAIL Emerges.
Clinical cancer research : an official journal of the American Association for Cancer ResearchSelective Immunoreactivity for WT1 Carboxy-Terminus Distinguishes Desmoplastic Small Round Cell Tumor From its Histologic Mimics.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyTrabectedin-irinotecan, a potentially promising combination in relapsed desmoplastic small round cell tumor: report of two cases.
Journal of chemotherapy (Florence, Italy)Highlighting the Diversity of Desmoplastic Small Round Cell Tumor: A Case Series.
International journal of surgical pathologySalt-Inducible Kinase 1 is a potential therapeutic target in Desmoplastic Small Round Cell Tumor.
OncogenesisThe added value of CEUS and ultrasound-guided biopsy in diagnosing an aggressive desmoplastic small round cell tumour of peritoneum in a young male. A case report.
Medical ultrasonographyThe Small Round Cell Sarcomas Complexities and Desmoplastic Presentation.
Acta cytologicaPrimary Pulmonary Round Cell Sarcomas: Multiple Potential Pitfalls for the Pathologist.
International journal of surgical pathologyMulti-site desmoplastic small round cell tumors are genetically related and immune-cold.
NPJ precision oncologyIntracranial desmoplastic small round cell tumor after childhood acute myeloid leukemia treated with metronomic oral cyclophosphamide.
Pediatric blood & cancerNext generation sequencing in a cohort of patients with rare sarcoma histotypes: A single institution experience.
Pathology, research and practiceOmental desmoplastic small round cell tumor with metastasis.
Indian journal of pathology & microbiologyPhase II Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor.
Clinical cancer research : an official journal of the American Association for Cancer ResearchPrimary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review.
Diagnostic pathologyAssociation between biologically effective dose and local control after stereotactic body radiotherapy for metastatic sarcoma.
Journal of radiosurgery and SBRTDesmoplastic Small Round Cell Tumor in a Pregnant Woman: A Case Report and Literature Review.
The Yale journal of biology and medicineLobular Carcinoma of the Breast Metastatic to the Ovary: A Clinicopathologic Study of 38 Cases.
The American journal of surgical pathologyEWSR1-WT1 Target Genes and Therapeutic Options Identified in a Novel DSRCT In Vitro Model.
CancersImmunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor.
Frontiers in oncologyBiodistribution and Radiation Dosimetry of Intraperitoneally Administered 124I-Omburtamab in Patients with Desmoplastic Small Round Cell Tumors.
Journal of nuclear medicine : official publication, Society of Nuclear MedicineNovel patient-derived models of desmoplastic small round cell tumor confirm a targetable dependency on ERBB signaling.
Disease models & mechanismsThe contribution of Juan Rosai to the pathology of soft tissue tumors.
PathologicaA rare cause of bilateral pleural effusion - desmoplastic small round cell tumor.
Monaldi archives for chest disease = Archivio Monaldi per le malattie del toracePeritoneal disease: key imaging findings that help in the differential diagnosis.
The British journal of radiologyCase Report: Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Application in Intraperitoneally Disseminated Inflammatory Myofibroblastic Tumor and in the Youngest Patient in the World: New Indication and Modification of Technique.
Frontiers in surgeryCommon Pitfalls in Ewing Sarcoma and Desmoplastic Small Round Cell Tumor Diagnosis Seen in a Study of 115 Cases.
Medical sciences (Basel, Switzerland)An unusual testicular mass: a case of desmoplastic small round cell tumour.
PathologyPrimary Desmoplastic Small Round Cell Tumor of the Femur: Case Report and Review of a Rare Intraosseous Malignancy.
International journal of surgical pathologyDesmoplastic small round cell tumour presenting as cervical lymph nodes metastases with solid pattern morphology and novel EWSR1-WT1 fusion transcript.
PathologyA Classic Presentation of Desmoplastic Small Round Cell Tumor.
CureusIntraabdominal and ganglionic desmoplastic small round cell tumor: a case series.
Journal of medical case reportsIntra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives.
Frontiers in oncologyInvestigation of spleen CXCR4 expression by [68Ga]Pentixafor PET in a cohort of 145 solid cancer patients.
EJNMMI researchMesenchymal/non-epithelial mimickers of neuroendocrine neoplasms with a focus on fusion gene-associated and SWI/SNF-deficient tumors.
Virchows Archiv : an international journal of pathologyUncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations. Spanish Group for Sarcoma research (GEIS -GROUP). Part II.
Cancer treatment reviewsDesmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review.
ORL; journal for oto-rhino-laryngology and its related specialtiesDesmoplastic Small Round Cell Tumors With EWS-WT1 Transcript Expression: Should We Consider Children and Adult Patients Differently?
Journal of pediatric hematology/oncologyDesmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review.
Anticancer researchA Review of Effusion Cytomorphology of Small Round Cell Tumors.
Acta cytologicaClinicopathological features of desmoplastic small round cell tumors: clinical series and literature review.
World journal of surgical oncologyEWSR1-WT1 gene fusions in neoplasms other than desmoplastic small round cell tumor: a report of three unusual tumors involving the female genital tract and review of the literature.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncDesmoplastic Small Round Cell Tumor of the Kidney With Mainly Pulmonary Symptoms by 18F-FDG PET/CT.
UrologyGenomic and transcriptomic characterization of desmoplastic small round cell tumors.
Genes, chromosomes & cancerYttrium-90 radioembolization in desmoplastic small round cell tumor with recurrent hepatic metastasis following hyperthermic intraperitoneal chemotherapy.
Radiology case reportsDesmoplastic Small Round Cell Tumor with "Pure" Spindle Cell Morphology and Novel EWS-WT1 Fusion Transcript: Expanding the Morphological and Molecular Spectrum of This Rare Entity.
Diagnostics (Basel, Switzerland)Influence of UGT1A1 *6/*28 Polymorphisms on Irinotecan-Related Toxicity and Survival in Pediatric Patients with Relapsed/Refractory Solid Tumors Treated with the VIT Regimen.
Pharmacogenomics and personalized medicineComprehensive Molecular Profiling of Desmoplastic Small Round Cell Tumor.
Molecular cancer research : MCRNuclear expression of DDIT3 distinguishes high-grade myxoid liposarcoma from other round cell sarcomas.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncDesmoplastic Small Round Cell Tumor With Ascending Intraspinal Metastasis at Recurrence: Case Report and Review of the Literature.
Journal of pediatric hematology/oncologyTreatment Concepts and Challenges in Nonrhabdomyosarcoma Soft Tissue Sarcomas.
Surgical oncology clinics of North AmericaThe Role and Clinical Effectiveness of Multiline Chemotherapy in Advanced Desmoplastic Small Round Cell Tumor.
Clinical Medicine Insights. OncologyMultiagent chemotherapy including IrIVA regimen and maintenance therapy in the treatment of desmoplastic small round cell tumor.
TumoriDesmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy.
CancersHyperthermic intraperitoneal chemotherapy (HIPEC) as another treatment modality for desmoplastic round cell tumour patients: first paediatric experience from UK.
BMJ case reportsDesmoplastic small round cell tumor of the ovary: A rare but poor prognostic disease in a young woman!
Indian journal of pathology & microbiologyFISH for EWSR1 in Ewing's sarcoma family of tumors: Experience from a tertiary care cancer center.
Indian journal of pathology & microbiologyDesmoplastic myxoid tumor, SMARCB1-mutant: a new variant of SMARCB1-deficient tumor of the central nervous system preferentially arising in the pineal region.
Virchows Archiv : an international journal of pathologyDesmoplastic Small Round Cell Tumors: A review with focus on clinical management and therapeutic options.
Cancer treatment reviewsRecurrent infantile inflammatory myofibroblastic tumor of mesentery--Case report and review of imaging findings.
Radiology case reportsNKX3.1 a useful marker for mesenchymal chondrosarcoma: An immunohistochemical study.
Annals of diagnostic pathologyThe addition of autologous stem cell transplantation to neoadjuvant chemotherapy, radiation, and HIPEC for patients with unresectable desmoplastic small round cell tumor: a single center case series.
International journal of surgery. OncologyEmerging Kinase Therapeutic Targets in Pancreatic Ductal Adenocarcinoma and Pancreatic Cancer Desmoplasia.
International journal of molecular sciencesTherapeutic Potential of NTRK3 Inhibition in Desmoplastic Small Round Cell Tumor.
Clinical cancer research : an official journal of the American Association for Cancer ResearchINSM1 Expression in Angiosarcoma.
American journal of clinical pathologyClinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients.
SarcomaMediastinal desmoplastic small round cell tumor.
MedicineB7H3-Directed Intraperitoneal Radioimmunotherapy With Radioiodinated Omburtamab for Desmoplastic Small Round Cell Tumor and Other Peritoneal Tumors: Results of a Phase I Study.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyChemotherapy combined with apatinib for the treatment of desmoplastic small round cell tumors: A case report.
Journal of cancer research and therapeuticsDesmoplastic Small Round Cell Tumor: A Rare Location in the Parotid Gland.
CureusThe spectrum of rare central nervous system (CNS) tumors with EWSR1-non-ETS fusions: experience from three pediatric institutions with review of the literature.
Brain pathology (Zurich, Switzerland)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.
- Update on pediatric soft tissue sarcomas.
- Desmoplastic Small Round Cell Tumor Presenting as Bloody Ascites: A Case Report and Literature Review.
- Whole Abdominal Radiotherapy in Bone and Soft Tissue Sarcomas: Indications, Techniques, Clinical Outcomes, and Future Directions.
- Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report With Atypical Morphologic Features and Literature Review.
- Fusion-positive soft tissue tumors: A selective review.
- Multi-layered molecular profiling informs the diagnosis and targeted therapy of desmoplastic small round cell tumor.
- 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:83469(Orphanet)
- MONDO:0019373(MONDO)
- GARD:6265(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q5264831(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
