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Tumor de células pequenas redondas desmoplásicas
ORPHA:83469CID-10 · C48.2DOENÇA RARA
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lungInício adolescenteHerança Unknown
Também conhecida comoDSRCT

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.

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Introdução

O que você precisa saber de cara

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EM LINGUAGEM SIMPLES

O tumor de células pequenas redondas desmoplásicas (DSRCT) é um câncer raro e agressivo que se desenvolve principalmente no peritônio, a membrana que reveste a barriga. Os sintomas costumam surgir na adolescência ou na juventude, manifestando-se por dor abdominal, inchaço na barriga, perda involuntária de peso e fraqueza. Esta condição não possui um Protocolo Clínico e Diretrizes Terapêuticas (PCDT) oficial no SUS, exigindo acompanhamento em hospitais especializados em oncologia. Diversas pesquisas clínicas internacionais estão em andamento para avaliar novas formas de tratamento molecular e imunoterapia para a enfermidade.

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Informacoes curadas por IA — podem conter imprecisoes

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.

Pesquisas ativas
18 ensaios
54 total registrados no ClinicalTrials.gov
Publicações científicas
729 artigos
Último publicado: 2026 Apr 9

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
300
pacientes catalogados
Início
Adolescent
+ adult
🏥
SUS: Sem cobertura SUSScore: 5%
CID-10: C48.2
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
5 sintomas
🩸
Sangue
3 sintomas
📏
Crescimento
2 sintomas
🫁
Pulmão
1 sintomas

+ 7 sintomas em outras categorias

Características mais comuns

90%prev.
Morfologia anormal do peritônio
Muito frequente (99-80%)
90%prev.
Linfadenopatia
Muito frequente (99-80%)
90%prev.
Dor abdominal
Muito frequente (99-80%)
90%prev.
Distensão abdominal
Muito frequente (99-80%)
90%prev.
Sarcoma
Muito frequente (99-80%)
55%prev.
Íleo
Frequente (79-30%)
18sintomas
Muito frequente (5)
Frequente (5)
Ocasional (8)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 18 características clínicas mais associadas, ordenadas por frequência.

Morfologia anormal do peritônioAbnormality of the peritoneum
Muito frequente (99-80%)90%
LinfadenopatiaLymphadenopathy
Muito frequente (99-80%)90%
Dor abdominalAbdominal pain
Muito frequente (99-80%)90%
Distensão abdominalAbdominal distention
Muito frequente (99-80%)90%
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico729PubMed
Últimos 10 anos200publicações
Pico202444 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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.

EWSR1RNA-binding protein EWSPart of a fusion gene inAltamente restrito
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
278.5 TPM
Cérebro - Hemisfério cerebelar
273.8 TPM
Testículo
272.3 TPM
Baço
257.8 TPM
Cervix Endocervix
248.9 TPM
OUTRAS DOENÇAS (6)
Ewing sarcomaextraskeletal Ewing sarcomahistiocytoma, Angiomatoid fibrousextraskeletal myxoid chondrosarcoma
HGNC:3508UniProt:Q01844
WT1Wilms tumor proteinPart of a fusion gene inAltamente restrito
VIAS BIOLÓGICAS (3)
Nephron developmentNegative Regulation of CDH1 Gene TranscriptionTranscriptional regulation of testis differentiation
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Tecido-específico)
Útero
109.7 TPM
Fallopian Tube
75.2 TPM
Ovário
65.2 TPM
Testículo
40.5 TPM
Adipose Visceral Omentum
30.0 TPM
OUTRAS DOENÇAS (12)
Wilms tumor 1nephrotic syndrome, type 4Meacham syndromemalignant mesothelioma
HGNC:12796UniProt:P19544

Variantes genéticas (ClinVar)

1.677 variantes patogênicas registradas no ClinVar.

🧬 WT1: NM_024426.6(WT1):c.266C>A (p.Pro89His) ()
🧬 WT1: NM_024426.6(WT1):c.766G>A (p.Gly256Ser) ()
🧬 WT1: NM_024426.6(WT1):c.1017-14C>G ()
🧬 WT1: NM_024426.6(WT1):c.451T>C (p.Trp151Arg) ()
🧬 WT1: NM_024426.6(WT1):c.446C>T (p.Pro149Leu) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 2 variantes classificadas pelo ClinVar.

2
Patogênica (100.0%)
VARIANTES MAIS SIGNIFICATIVAS
LOC110806263: NM_198253.3(TERT):c.-124C>T [Conflicting classifications of pathogenicity]
TP53: NM_000546.6(TP53):c.818G>A (p.Arg273His) [Pathogenic]
NPIPB2: NM_001192.3(TNFRSF17):c.524C>T (p.Thr175Met) [other]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 32
2Fase 230
1Fase 115
·Pré-clínico7
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 54 ensaios
Carregando informações de tratamento...

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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Selecione um estado ou use sua localização para ver resultados.

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

Onde estão os ensaios

Com ensaio aberto em 5 países. O ponto verde marca onde há vaga agora.

🟢 Recrutando agora

12 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

54 ensaios clínicos encontrados, 18 ativos.

Distribuição por fase
NCT07695311 · EWSR1 Immunotherapy in Ewing Sarcoma and DSRCTEm breve
PHASE1🇺🇸 Estados Unidos
NCT04483778 · B7H3 CAR T Cell Immunotherapy for Recurrent/Refractory Solid…Ativo
PHASE1🇺🇸 Estados Unidos
NCT04022213 · A Study of the Drug I131-Omburtamab in People With Desmoplas…Ativo
PHASE2🇺🇸 Estados Unidos
NCT03618381 · EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory So…Ativo
PHASE1🇺🇸 Estados Unidos
NCT03478462 · Dose Escalation Study of CLR 131 in Pediatric Relapsed/Refra…Ativo
PHASE1🇦🇺 Austrália · 🇨🇦 Canadá · 🇺🇸 Estados Unidos
NCT01189643 · Irinotecan, Temozolomide and Bevacizumab in Combination With…Ativo
EARLY_PHASE1🇺🇸 Estados Unidos
NCT04145349 · CAMPFIRE: A Study of Ramucirumab (LY3009806) in Children and…Concluído
PHASE1, PHASE2🇦🇺 Austrália · 🇩🇪 Alemanha · 🇮🇹 Itália +4
NCT04095221 · A Study of the Drugs Prexasertib, Irinotecan, and Temozolomi…Concluído
PHASE1, PHASE2🇺🇸 Estados Unidos
NCT03275818 · Trial of Nab-paclitaxel in Patients With Desmoid Tumors and …Concluído
PHASE2🇪🇸 Espanha
NCT02982941 · Enoblituzumab (MGA271) in Children With B7-H3-expressing Sol…Concluído
PHASE1🇺🇸 Estados Unidos
NCT01567046 · Studying Genes in Tissue Samples From Younger and Adolescent…Concluído
🇺🇸 Estados Unidos
NCT01946529 · Therapeutic Trial for Patients With Ewing Sarcoma Family of …Concluído
PHASE2🇺🇸 Estados Unidos
NCT01532687 · Gemcitabine With or Without Pazopanib in Treating Patients W…Concluído
PHASE2🇺🇸 Estados Unidos
NCT01430637 · Studying Genes in Samples From Younger Patients With Desmopl…Concluído
NCT01277744 · Pilot Study of Hyperthermic Peritoneal Perfusion (HIPEC) for…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00526149 · BI 2536 in Treating Patients With Recurrent or Metastatic So…Concluído
PHASE2🇧🇪 Bélgica
NCT00474994 · Sunitinib in Treating Patients With Metastatic, Locally Adva…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00436657 · Continuous Hyperthermic Peritoneal Perfusion (CHPP) With Cis…Concluído
PHASE1🇺🇸 Estados Unidos
NCT00445965 · Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients Wi…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00562380 · AMG-479 in Treating Patients With Advanced Solid Tumors or N…Concluído
PHASE1🇺🇸 Estados Unidos
NCT00093821 · Tanespimycin in Treating Young Patients With Recurrent or Re…Concluído
PHASE1🇺🇸 Estados Unidos
NCT00055952 · Exatecan Mesylate in Treating Patients With Ewing's Sarcoma,…Concluído
PHASE2🇨🇦 Canadá · 🇺🇸 Estados Unidos
NCT00043979 · Stem Cell Transplantation in Patients With High-Risk and Rec…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00030667 · Imatinib Mesylate in Treating Patients With Relapsed or Refr…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00024258 · Arsenic Trioxide in Treating Patients With Advanced Neurobla…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00919269 · Collecting and Storing Tissue, Blood, and Bone Marrow Sample…Concluído
🇦🇺 Austrália · 🇨🇦 Canadá · 🇳🇱 Países Baixos +4
NCT00025441 · Combination Chemotherapy in Treating Children With Metastati…Concluído
PHASE2🇫🇷 França · 🇮🇪 Irlanda · 🇬🇧 Reino Unido
NCT00002898 · Surgery Followed by Chemotherapy in Treating Young Patients …Concluído
PHASE3🇬🇧 Reino Unido
NCT00002515 · Combination Chemotherapy Followed by Bone Marrow Transplanta…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00662233 · Combination Chemotherapy in Treating Patients With SarcomaConcluído
EARLY_PHASE1🇺🇸 Estados Unidos
NCT00002466 · Combination Chemotherapy and Radiation Therapy in Treating P…Concluído
PHASE2🇺🇸 Estados Unidos
NCT05266196 · A Rollover Protocol to Allow for Continued Access to the LSD…UNKNOWN
PHASE1, PHASE2🇺🇸 Estados Unidos
NCT03600649 · Clinical Trial of SP-2577 (Seclidemstat) in Patients With Re…UNKNOWN
PHASE1🇺🇸 Estados Unidos
NCT02982486 · A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sa…UNKNOWN
PHASE2
NCT02834169 · French National Registry of Rare Peritoneal Surface Malignan…UNKNOWN
🇫🇷 França
NCT04901806 · Study of PBI-200 in Subjects With NTRK-Fusion-Positive Solid…Encerrado
PHASE1🇦🇺 Austrália · 🇩🇰 Dinamarca · 🇫🇷 França +8
NCT04530487 · Donor Stem Cell Transplant After Chemotherapy for the Treatm…Encerrado
PHASE2🇺🇸 Estados Unidos
NCT01189253 · Doxorubicin Hydrochloride or Trabectedin in Treating Patient…Encerrado
PHASE2, PHASE3🇦🇹 Áustria · 🇧🇪 Bélgica · 🇩🇰 Dinamarca +10
NCT00089245 · Radiolabeled MAB Therapy in Patients With Refractory, Recurr…Encerrado
PHASE1🇺🇸 Estados Unidos
NCT00003926 · Amifostine to Protect From Side Effects of PSCT in Treating …Encerrado
PHASE1🇺🇸 Estados Unidos
NCT01125449 · Study of High Dose Intravenous (IV) Ascorbic Acid in Measura…Cancelado
PHASE2🇺🇸 Estados Unidos
NCT00334984 · Glutamine in Preventing Oral Mucositis in Patients Receiving…Cancelado
NA
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
353 papers (10 anos)
#1

Update on pediatric soft tissue sarcomas.

Current opinion in pediatrics2026 Feb 01

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.

#2

Desmoplastic Small Round Cell Tumor Presenting as Bloody Ascites: A Case Report and Literature Review.

Surgical laparoscopy, endoscopy &amp; percutaneous techniques2026 Mar 17

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.

#3

Whole Abdominal Radiotherapy in Bone and Soft Tissue Sarcomas: Indications, Techniques, Clinical Outcomes, and Future Directions.

Current treatment options in oncology2026 Mar 14

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.

#4

Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report With Atypical Morphologic Features and Literature Review.

International journal of surgical pathology2026 Mar 13

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.

#5

Fusion-positive soft tissue tumors: A selective review.

Seminars in diagnostic pathology2026 Mar

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

Ver todas no PubMed

📚 EuropePMC475 artigos no totalmostrando 197

2026

Desmoplastic Small Round Cell Tumor Presenting as Bloody Ascites: A Case Report and Literature Review.

Surgical laparoscopy, endoscopy &amp; percutaneous techniques
2026

Whole Abdominal Radiotherapy in Bone and Soft Tissue Sarcomas: Indications, Techniques, Clinical Outcomes, and Future Directions.

Current treatment options in oncology
2026

Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report With Atypical Morphologic Features and Literature Review.

International journal of surgical pathology
2026

Fusion-positive soft tissue tumors: A selective review.

Seminars in diagnostic pathology
2026

Desmoplastic Small Round Blue Cell Tumor in a Young Adult Man: A Rare Case of Abdominal Sarcoma.

The American journal of case reports
2026

Radiologically Occult Desmoplastic Small Round Cell Tumor in a 36-Year-Old Man: A Case Report.

Cureus
2026

Harnessing AACR Project GENIE to Define the Molecular Features of Desmoplastic Small Round Cell Tumor.

Current issues in molecular biology
2026

Desmoplastic Small Round Cell Tumor: Study of Cytomorphologic and Immunophenotypical Features in Seven Cases, One With Unusual Rhabdoid Morphology.

Diagnostic cytopathology
2026

Update on pediatric soft tissue sarcomas.

Current opinion in pediatrics
2025

Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center.

BMC cancer
2025

Unresectable Desmoplastic Small Round Cell Tumor: A Case Report and Review of the Literature.

Case reports in oncology
2025

Small Desmoplastic Round Cell Tumor of the Kidney following Hodgkin Lymphoma: Case Report and Literature Review.

Case reports in oncology
2025

Trabectedin-irinotecan as a potential therapeutic option in desmoplastic small round cell tumor: A small case series.

Tumori
2025

Chemokine receptor-4-targeted imaging with [68Ga]Ga-Pentixafor PET: unveiling its diagnostic and prognostic potential across cancers: a systematic review.

Nuclear medicine communications
2025

EWS::WT1 Isoform-Dependent Regulation of Neogenes in Desmoplastic Small Round Cell Tumors.

bioRxiv : the preprint server for biology
2025

Rationale and design of the PAMSARC (pasireotide as maintenance treatment with monthly deep intramuscular injection in SSTR2/3/5-expressing synovial sarcoma and desmoplastic small round cell tumor) multicenter phase 2 trial.

Cancer treatment and research communications
2025

Immunotherapy and Targeted Therapies in Sarcoma: Proposed Synergy with Combination Treatment.

Journal of immunotherapy and precision oncology
2026

Antitumor Activity of Trastuzumab Deruxtecan in Pediatric Solid Tumors with Variable HER2 Expression.

Molecular cancer therapeutics
2025

Near Complete Response With Trastuzumab Deruxtecan in a Teenager With Relapsing Metastatic Desmoplastic Small Round Cell Tumor.

Pediatric blood &amp; cancer
2025

Seclidemstat (SP-2577) Induces Transcriptomic Reprogramming and Cytotoxicity in Multiple Fusion-Positive Sarcomas.

Cancer research communications
2025

Desmoplastic small round cell tumor in children: Report of six cases.

Pathology, research and practice
2025

Desmoplastic Small Round Cell Tumors and the Role of Androgen Receptors.

Current treatment options in oncology
2025

An Autopsy Case of a Desmoplastic Small Round Cell Tumor with a Rapid Fatal Outcome due to Diffuse Cystic Liver Metastases.

Internal medicine (Tokyo, Japan)
2025

Ewing Sarcoma, Desmoplastic Small Round Cell Tumor, and Other Round Cell Sarcomas.

Hematology/oncology clinics of North America
2025

Whole Abdominal Radiotherapy Is Tolerable and Effective in Children and Young Adults With Intra-Abdominal Sarcoma: A Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) Study.

Pediatric blood &amp; cancer
2025

ACR-368, a CHK1/2 Kinase Inhibitor, in Patients With Relapsed or Refractory Desmoplastic Small Round Cell Tumor: Phase I/II Trial Results.

JCO oncology advances
2025

Strategies for the Treatment of Desmoplastic Small Round Cell Tumor: A Case Series.

Pediatric blood &amp; cancer
2025

Desmoplastic small round cell tumor: an update of current management practices.

Journal of the Egyptian National Cancer Institute
2025

Case Report: Differentiating hepatic desmoplastic small round cell tumor from hydatidosis in a school-aged boy: the role of contrast-enhanced and interventional ultrasound.

Frontiers in oncology
2025

Computed tomography manifestations and pathological features of intra-abdominal desmoplastic small round cell tumor.

Pakistan journal of medical sciences
2025

Multiple Desmoplastic Small Round Cell Tumor in the Intestine: A Case Report.

Surgical case reports
2025

Prognostic factors of desmoplastic small round cell tumor in children and adolescents: a population-based study.

Pediatric surgery international
2025

Durable response to treatment of an atypical desmoplastic small round cell tumor with enfortumab-vedotin.

Journal of cancer research and clinical oncology
2025

Glucose Transporter 1 (GLUT1) Positivity in Ganglion Cysts: A Diagnostic Caveat Due to Overlap With Perineuriomas and Other Fibromyxoid Lesions.

Cureus
2025

High expression level of ERBB2 and efficacy of trastuzumab deruxtecan in desmoplastic small round cell tumour: a monocentric case series report.

ESMO open
2025

Cinematic Rendering of Desmoplastic Small Round Cell Tumor.

Radiology. Imaging cancer
2025

Variant Pseudocystic Desmoplastic Small Round Cell Tumor With Heterologous Mullerian Cysts.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2024

Pelvic Desmoplastic Small Round Cell Tumor on 18F-PSMA PET/CT.

Clinical nuclear medicine
2024

Desmoplastic Small Round Cell Tumor: a study of CT, MRI, PET/CT multimodal imaging features and their correlations with pathology.

BMC medical imaging
2024

Role and Efficacy of 18F-FDG PET/CT in Following Up Desmoplastic Small Round Cell Tumor of the Abdomen: A Case Report.

Cureus
2025

Emerging round cell sarcomas in children.

Virchows Archiv : an international journal of pathology
2024

A Case of Desmoplastic Small Round Cell Tumor: Highlighting Diagnostic Challenges and Underscoring the Need for Standardized Treatment Protocols.

Cureus
2024

Desmoplastic Small Round Cell Tumor in a Young Adult: A Case Report Highlighting Diagnostic Challenges and Treatment Approaches.

Cureus
2025

Replication Stress Is an Actionable Genetic Vulnerability in Desmoplastic Small Round Cell Tumors.

Cancer research
2024

[Pediatric soft tissue tumors : Tumors of uncertain origin].

Pathologie (Heidelberg, Germany)
2024

Multiple Abdominal Desmoplastic Small Round-Cell Tumors Treated With Fan Beam Computed Tomography-Guided Adaptive Radiotherapy (FBCT-gART): A Case Report.

Cureus
2024

Adult prostate stromal sarcoma: Desmoplastic small round cell tumor.

Asian journal of surgery
2024

EWS-WT1 fusion isoforms establish oncogenic programs and therapeutic vulnerabilities in desmoplastic small round cell tumors.

Nature communications
2025

Surgical management of rare tumors (Part 1).

Pediatric blood &amp; cancer
2024

Immunohistochemical Evaluation of Schlafen 11 (SLFN11) Expression in Cancer in the Search of Biomarker-Informed Treatment Targets: A Study of 127 Entities Represented by 6658 Tumors.

The American journal of surgical pathology
2024

Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics.

Frontiers in cell and developmental biology
2024

HER2 Antibody-Drug Conjugates Are Active against Desmoplastic Small Round Cell Tumor.

Clinical cancer research : an official journal of the American Association for Cancer Research
2024

Primary desmoplastic small round cell tumour of the prostate.

Journal of clinical pathology
2024

ISLET-1 expression in soft tissue neoplasms reveals high sensitivity but moderate specificity for desmoplastic small round cell tumors and potential utility as a diagnostic biomarker.

Pathology, research and practice
2024

Desmoplastic Small Round Cell Tumor as a Rare Cause of an Incarcerated Epigastric Linea Alba Hernia: A Case Report and Literature Review.

Cureus
2024

Undifferentiated Round Cell Sarcoma With CRTC1::SS18 Fusion: Expanding Clinicopathologic Features of a Rare Translocation Sarcoma With Prominent Desmoplastic Stroma.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2024

Desmoplastic small round cell tumor of the kidney: a case report and discussion.

Frontiers in oncology
2024

Seclidemstat blocks the transcriptional function of multiple FET-fusion oncoproteins.

bioRxiv : the preprint server for biology
2024

Single-cell multiomics profiling reveals heterogeneous transcriptional programs and microenvironment in DSRCTs.

Cell reports. Medicine
2024

Imaging Diagnosis of Desmoplastic Small Round Cell Tumor: A Report of Two Cases.

Cureus
2024

Imaging of desmoplastic small round-cell tumor on 68Ga-FAPI PET/CT.

Revista espanola de medicina nuclear e imagen molecular
2024

Multimodality imaging of ureteral desmoplastic small round cell tumor: a case description and literature analysis of 18F-fluoro-2-deoxy-d-glucose positron emission tomography-computed tomography findings.

Quantitative imaging in medicine and surgery
2024

Comprehensive Transcriptomic Analysis of EWSR1::WT1 Targets Identifies CDK4/6 Inhibitors as an Effective Therapy for Desmoplastic Small Round Cell Tumors.

Cancer research
2024

Abdominopelvic desmoplastic small round cell tumor with metastasis: A case report and literature review.

Medicine
2024

Enzalutamide induces cytotoxicity in desmoplastic small round cell tumor independent of the androgen receptor.

Communications biology
2024

Desmoplastic Small Round Cell Tumor Presenting as an Intra/Extracranial Mass.

Cureus
2024

Desmoplastic small round cell tumor of bone revealed by 18F-FDG PET/CT: a case report with literature review.

Skeletal radiology
2024

Case report: An unusual presentation of intra-abdominal desmoplastic small round cell tumor.

Frontiers in oncology
2024

Multifocal Desmoplastic Small Round Cell Tumor: A Case Report of a Rare Neoplasm.

Cureus
2024

Treatment of Abdominal Desmoplastic Small Round Cell Tumor Induces Acute Myeloid Leukemia-M5: A Case Report and Literature Review.

OncoTargets and therapy
2024

Effective Treatment of Anlotinib Combined With Chemotherapy in Children With Desmoplastic Small Round Cell Tumor: A Case Series in a Single-center and Literature Review.

Journal of pediatric hematology/oncology
2024

Technical Considerations in Surgical Resection of Desmoplastic Small Round Cell Tumors.

Journal of pediatric surgery
2024

Desmoplastic small round-cell tumor.

Asian journal of surgery
2024

Successful surgical treatment of desmoplastic small round cell tumor in a postmenopausal woman: A rare case report.

International journal of surgery case reports
2024

Transcriptomic analysis identifies B-lymphocyte kinase as a therapeutic target for desmoplastic small round cell tumor cancer stem cell-like cells.

Oncogenesis
2024

EWSR1::WT1 Fusions in Neoplasms Other Than Conventional Desmoplastic Small Round Cell Tumor: Three Tumors Occurring Outside the Female Genital Tract.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2024

Increased Prevalence of Clostridioides difficile Infection Among Pediatric Oncology Patients: Risk Factors for Infection and Complications.

The Pediatric infectious disease journal
2024

A case report of chest wall desmoplastic small round cell tumor in children.

International journal of surgery case reports
2024

CXCR4-directed PET/CT with [68 Ga]Ga-pentixafor in solid tumors-a comprehensive analysis of imaging findings and comparison with histopathology.

European journal of nuclear medicine and molecular imaging
2024

Primary desmoplastic small round-cell tumor with intraspinal extension mimicking neurinoma: Imaging diagnostic clues.

Asian journal of surgery
2023

Intra-abdominal omental mass as a desmoplastic round cell tumor: A rare case report.

Clinical case reports
2023

Desmoplastic small round cell tumor of the abdomen: A case report.

Medicine
2023

A Practical Approach to Small Round Cell Tumors Involving the Gastrointestinal Tract and Abdomen.

Surgical pathology clinics
2024

Intracranial desmoplastic small round-cell tumor.

Asian journal of surgery
2024

Desmoplastic small round cell tumor with ovarian involvement on [18F]-FDG PET/CT.

Revista espanola de medicina nuclear e imagen molecular
2023

Palliative Radiation Therapy in the Treatment of Desmoplastic Small Round Cell Tumors.

Cureus
2023

[Desmoplastic round cell tumor].

Medicina
2023

Desmoplastic small round cell tumor of the liver: diagnosing a rare case on liver biopsy.

Diagnostic pathology
2023

Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment - Experience from a Major Pediatric Cancer Center in Europe.

Klinische Padiatrie
2023

Metastatic Pulmonary Desmoplastic Small Round Cell Tumor on FDG PET/CT.

Clinical nuclear medicine
2023

Genomic Breakpoint Characterization and Transcriptome Analysis of Metastatic, Recurrent Desmoplastic Small Round Cell Tumor.

Sarcoma
2023

Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings.

Quantitative imaging in medicine and surgery
2023

CXCR4-Directed Imaging and Endoradiotherapy in Desmoplastic Small Round Cell Tumors.

Journal of nuclear medicine : official publication, Society of Nuclear Medicine
2023

A Review of the Use of Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Malignancy in Pediatric Patients.

Cancers
2023

STK11 Adnexal Tumor in an Adolescent Female: Diagnostic Pitfalls of a Recently Described Entity.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2024

Diagnostic Utility of GATA3 and ISL1 in Differentiating Neuroblastoma From Other Pediatric Malignant Small Round Blue Cell Tumors.

International journal of surgical pathology
2023

Desmoplastic Small Round Cell Tumor Involving Serous Fluid: Cytologic Features and Diagnostic Pitfalls: A Series of 8 Cases.

American journal of clinical pathology
2023

Dissociation protocols used for sarcoma tissues bias the transcriptome observed in single-cell and single-nucleus RNA sequencing.

BMC cancer
2023

Intra-abdominal desmoplastic small round cell tumor: The European pediatric Soft tissue sarcoma Study Group (EpSSG) experience.

Pediatric blood &amp; cancer
2023

Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review.

Current oncology (Toronto, Ont.)
2023

Effectiveness of irinotecan plus trabectedin on a desmoplastic small round cell tumor patient-derived xenograft.

Disease models &amp; mechanisms
2023

Identification of immunotherapy and radioimmunotherapy targets on desmoplastic small round cell tumors.

Frontiers in oncology
2023

Imaging of pediatric abdominal soft tissue tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Pediatric blood &amp; cancer
2023

Desmoplastic small round cell tumor: from state of the art to future clinical prospects.

Expert review of anticancer therapy
2023

Long-term survivors with desmoplastic small round cell tumor (DSRCT): Results from a retrospective single-institution case series analysis.

Cancer medicine
2023

Expanding 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, Inc
2023

Incarcerated metastasis as the first manifestation of a rare sarcoma.

Rozhledy v chirurgii : mesicnik Ceskoslovenske chirurgicke spolecnosti
2023

Molecular profiling in desmoplastic small round cell tumours.

The international journal of biochemistry &amp; cell biology
2023

Establishment of predictive nomogram and web-based survival risk calculator for desmoplastic small round cell tumor: A propensity score-adjusted, population-based study.

Biomolecules &amp; biomedicine
2022

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal malignant tumors in children: Initial experience in a single institution.

Frontiers in surgery
2022

Transcription regulators and ultra-rare and other rare translocation-related sarcomas treated with trabectedin: A proof of principle from a post-hoc analysis.

Frontiers in oncology
2022

Desmoplastic small round cell tumor cancer stem cell-like cells resist chemotherapy but remain dependent on the EWSR1-WT1 oncoprotein.

Frontiers in cell and developmental biology
2022

Giant desmoplastic small round cell tumor of the abdomen: A case report.

Frontiers in oncology
2024

Categorizing Malignant Small Round Cell Tumors in Aspiration Cytology: An Institutional Experience.

Journal of microscopy and ultrastructure
2022

Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall.

Cureus
2022

Desmoplastic small round cell tumor involving the uterine cervix: The first reported case in the literature, and brief review of gynecologic presentations.

Gynecologic oncology reports
2022

Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG).

Cancer management and research
2023

Desmoplastic 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 pathology
2023

Anesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.

Paediatric anaesthesia
2022

Malignant rhabdoid tumor: Cyto-histologic correlation and immunohistochemical characterization of a rare pediatric malignancy and its differential diagnoses.

Annals of diagnostic pathology
2022

Liver metastasis in soft tissue sarcoma at initial presentation.

Journal of surgical oncology
2022

Beyond Wilms tumor: imaging findings and outcomes of rare renal tumors in children.

Pediatric radiology
2022

Changing incidence and survival of desmoplastic small round cell tumor in the USA.

Proceedings (Baylor University. Medical Center)
2022

Extracellular Vesicles: A New Source of Biomarkers in Pediatric Solid Tumors? A Systematic Review.

Frontiers in oncology
2022

Lurbinectedin Inhibits the EWS-WT1 Transcription Factor in Desmoplastic Small Round Cell Tumor.

Molecular cancer therapeutics
2022

The androgen receptor is a therapeutic target in desmoplastic small round cell sarcoma.

Nature communications
2022

Desmoplastic Small Round Cell Tumor of the Uterus: A Report of Molecularly Confirmed Case with EWSR1-WT1 Fusion.

Diagnostics (Basel, Switzerland)
2022

Neuroendocrine and Rare Tumor Advances: A New and Promising TRAIL Emerges.

Clinical cancer research : an official journal of the American Association for Cancer Research
2022

Selective 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 Society
2023

Trabectedin-irinotecan, a potentially promising combination in relapsed desmoplastic small round cell tumor: report of two cases.

Journal of chemotherapy (Florence, Italy)
2023

Highlighting the Diversity of Desmoplastic Small Round Cell Tumor: A Case Series.

International journal of surgical pathology
2022

Salt-Inducible Kinase 1 is a potential therapeutic target in Desmoplastic Small Round Cell Tumor.

Oncogenesis
2022

The 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 ultrasonography
2022

The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation.

Acta cytologica
2022

Primary Pulmonary Round Cell Sarcomas: Multiple Potential Pitfalls for the Pathologist.

International journal of surgical pathology
2022

Multi-site desmoplastic small round cell tumors are genetically related and immune-cold.

NPJ precision oncology
2022

Intracranial desmoplastic small round cell tumor after childhood acute myeloid leukemia treated with metronomic oral cyclophosphamide.

Pediatric blood &amp; cancer
2022

Next generation sequencing in a cohort of patients with rare sarcoma histotypes: A single institution experience.

Pathology, research and practice
2022

Omental desmoplastic small round cell tumor with metastasis.

Indian journal of pathology &amp; microbiology
2022

Phase 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 Research
2022

Primary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review.

Diagnostic pathology
2022

Association between biologically effective dose and local control after stereotactic body radiotherapy for metastatic sarcoma.

Journal of radiosurgery and SBRT
2021

Desmoplastic Small Round Cell Tumor in a Pregnant Woman: A Case Report and Literature Review.

The Yale journal of biology and medicine
2022

Lobular Carcinoma of the Breast Metastatic to the Ovary: A Clinicopathologic Study of 38 Cases.

The American journal of surgical pathology
2021

EWSR1-WT1 Target Genes and Therapeutic Options Identified in a Novel DSRCT In Vitro Model.

Cancers
2021

Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor.

Frontiers in oncology
2022

Biodistribution 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 Medicine
2022

Novel patient-derived models of desmoplastic small round cell tumor confirm a targetable dependency on ERBB signaling.

Disease models &amp; mechanisms
2021

The contribution of Juan Rosai to the pathology of soft tissue tumors.

Pathologica
2021

A rare cause of bilateral pleural effusion - desmoplastic small round cell tumor.

Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace
2022

Peritoneal disease: key imaging findings that help in the differential diagnosis.

The British journal of radiology
2021

Case 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 surgery
2021

Common Pitfalls in Ewing Sarcoma and Desmoplastic Small Round Cell Tumor Diagnosis Seen in a Study of 115 Cases.

Medical sciences (Basel, Switzerland)
2022

An unusual testicular mass: a case of desmoplastic small round cell tumour.

Pathology
2022

Primary Desmoplastic Small Round Cell Tumor of the Femur: Case Report and Review of a Rare Intraosseous Malignancy.

International journal of surgical pathology
2022

Desmoplastic small round cell tumour presenting as cervical lymph nodes metastases with solid pattern morphology and novel EWSR1-WT1 fusion transcript.

Pathology
2021

A Classic Presentation of Desmoplastic Small Round Cell Tumor.

Cureus
2021

Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series.

Journal of medical case reports
2021

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives.

Frontiers in oncology
2021

Investigation of spleen CXCR4 expression by [68Ga]Pentixafor PET in a cohort of 145 solid cancer patients.

EJNMMI research
2021

Mesenchymal/non-epithelial mimickers of neuroendocrine neoplasms with a focus on fusion gene-associated and SWI/SNF-deficient tumors.

Virchows Archiv : an international journal of pathology
2021

Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations. Spanish Group for Sarcoma research (GEIS -GROUP). Part II.

Cancer treatment reviews
2022

Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review.

ORL; journal for oto-rhino-laryngology and its related specialties
2022

Desmoplastic Small Round Cell Tumors With EWS-WT1 Transcript Expression: Should We Consider Children and Adult Patients Differently?

Journal of pediatric hematology/oncology
2021

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review.

Anticancer research
2022

A Review of Effusion Cytomorphology of Small Round Cell Tumors.

Acta cytologica
2021

Clinicopathological features of desmoplastic small round cell tumors: clinical series and literature review.

World journal of surgical oncology
2021

EWSR1-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, Inc
2021

Desmoplastic Small Round Cell Tumor of the Kidney With Mainly Pulmonary Symptoms by 18F-FDG PET/CT.

Urology
2021

Genomic and transcriptomic characterization of desmoplastic small round cell tumors.

Genes, chromosomes &amp; cancer
2021

Yttrium-90 radioembolization in desmoplastic small round cell tumor with recurrent hepatic metastasis following hyperthermic intraperitoneal chemotherapy.

Radiology case reports
2021

Desmoplastic 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)
2021

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 medicine
2021

Comprehensive Molecular Profiling of Desmoplastic Small Round Cell Tumor.

Molecular cancer research : MCR
2021

Nuclear 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, Inc
2022

Desmoplastic Small Round Cell Tumor With Ascending Intraspinal Metastasis at Recurrence: Case Report and Review of the Literature.

Journal of pediatric hematology/oncology
2021

Treatment Concepts and Challenges in Nonrhabdomyosarcoma Soft Tissue Sarcomas.

Surgical oncology clinics of North America
2021

The Role and Clinical Effectiveness of Multiline Chemotherapy in Advanced Desmoplastic Small Round Cell Tumor.

Clinical Medicine Insights. Oncology
2022

Multiagent chemotherapy including IrIVA regimen and maintenance therapy in the treatment of desmoplastic small round cell tumor.

Tumori
2021

Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy.

Cancers
2021

Hyperthermic intraperitoneal chemotherapy (HIPEC) as another treatment modality for desmoplastic round cell tumour patients: first paediatric experience from UK.

BMJ case reports
2021

Desmoplastic small round cell tumor of the ovary: A rare but poor prognostic disease in a young woman!

Indian journal of pathology &amp; microbiology
2021

FISH for EWSR1 in Ewing's sarcoma family of tumors: Experience from a tertiary care cancer center.

Indian journal of pathology &amp; microbiology
2021

Desmoplastic 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 pathology
2021

Desmoplastic Small Round Cell Tumors: A review with focus on clinical management and therapeutic options.

Cancer treatment reviews
2021

Recurrent infantile inflammatory myofibroblastic tumor of mesentery--Case report and review of imaging findings.

Radiology case reports
2021

NKX3.1 a useful marker for mesenchymal chondrosarcoma: An immunohistochemical study.

Annals of diagnostic pathology
2020

The 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. Oncology
2020

Emerging Kinase Therapeutic Targets in Pancreatic Ductal Adenocarcinoma and Pancreatic Cancer Desmoplasia.

International journal of molecular sciences
2021

Therapeutic Potential of NTRK3 Inhibition in Desmoplastic Small Round Cell Tumor.

Clinical cancer research : an official journal of the American Association for Cancer Research
2021

INSM1 Expression in Angiosarcoma.

American journal of clinical pathology
2020

Clinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients.

Sarcoma
2020

Mediastinal desmoplastic small round cell tumor.

Medicine
2020

B7H3-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 Oncology
2020

Chemotherapy combined with apatinib for the treatment of desmoplastic small round cell tumors: A case report.

Journal of cancer research and therapeutics
2020

Desmoplastic Small Round Cell Tumor: A Rare Location in the Parotid Gland.

Cureus
2021

The 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)
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Associações

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Doença com base genética

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Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

O tumor de células pequenas redondas desmoplásicas é descrito na literatura médica como um tipo agressivo de sarcoma de partes moles. As possibilidades de resposta terapêutica dependem do estadiamento e da extensão das lesões no peritônio e em órgãos como o fígado.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

7 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. ORPHA:83469
    Orphanet
  2. GARD:6265
    GARD (NIH)
  3. 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.

Citar este verbete

Raras. (s.d.). Tumor de células pequenas redondas desmoplásicas. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/tumor-de-celulas-pequenas-redondas-desmoplasicas

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Tumor de células pequenas redondas desmoplásicas
Compêndio · Raras BR

Tumor de células pequenas redondas desmoplásicas

ORPHA:83469 · MONDO:0019373
Prevalência
<1 / 1 000 000
Casos
300 casos conhecidos
Herança
Not applicable
CID-10
C48.2 · Neoplasia maligna do peritônio
Ensaios
18 ativos
Início
Adolescent, Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0281508
EuropePMC
Wikidata
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Rara