Um tumor de células dendríticas é um tipo de tumor que se desenvolve a partir das células dendríticas, que são parte do sistema imunológico (o sistema de defesa do corpo). Geralmente, essa condição começa no sistema linfático e pode se espalhar para órgãos próximos ou para partes distantes do corpo (um processo chamado metástase). Os sintomas e a gravidade da doença dependem do subtipo (o tipo específico) e de onde o tumor está localizado.
Introdução
O que você precisa saber de cara
Um tumor de células dendríticas é um tipo de tumor que se desenvolve a partir das células dendríticas, que são parte do sistema imunológico (o sistema de defesa do corpo). Geralmente, essa condição começa no sistema linfático e pode se espalhar para órgãos próximos ou para partes distantes do corpo (um processo chamado metástase). Os sintomas e a gravidade da doença dependem do subtipo (o tipo específico) e de onde o tumor está localizado.
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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
+ 12 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
3 genes identificados com associação a esta condição.
Protein kinase involved in the transduction of mitogenic signals from the cell membrane to the nucleus (Probable). Phosphorylates MAP2K1, and thereby activates the MAP kinase signal transduction pathway (PubMed:21441910, PubMed:29433126). Phosphorylates PFKFB2 (PubMed:36402789). May play a role in the postsynaptic responses of hippocampal neurons (PubMed:1508179)
NucleusCytoplasmCell membrane
Dual specificity protein kinase which acts as an essential component of the MAP kinase signal transduction pathway. Binding of extracellular ligands such as growth factors, cytokines and hormones to their cell-surface receptors activates RAS and this initiates RAF1 activation. RAF1 then further activates the dual-specificity protein kinases MAP2K1/MEK1 and MAP2K2/MEK2. Both MAP2K1/MEK1 and MAP2K2/MEK2 function specifically in the MAPK/ERK cascade, and catalyze the concomitant phosphorylation of
Cytoplasm, cytoskeleton, microtubule organizing center, centrosomeCytoplasm, cytoskeleton, microtubule organizing center, spindle pole bodyCytoplasmNucleusMembrane
Cardiofaciocutaneous syndrome 3
A form of cardiofaciocutaneous syndrome, a multiple congenital anomaly disorder characterized by a distinctive facial appearance, heart defects and intellectual disability. Heart defects include pulmonic stenosis, atrial septal defects and hypertrophic cardiomyopathy. Some affected individuals present with ectodermal abnormalities such as sparse, friable hair, hyperkeratotic skin lesions and a generalized ichthyosis-like condition. Typical facial features are similar to Noonan syndrome. They include high forehead with bitemporal constriction, hypoplastic supraorbital ridges, downslanting palpebral fissures, a depressed nasal bridge, and posteriorly angulated ears with prominent helices. Distinctive features of CFC3 include macrostomia and horizontal shape of palpebral fissures.
Ras proteins bind GDP/GTP and possess intrinsic GTPase activity
Cell membraneGolgi apparatus membrane
Leukemia, juvenile myelomonocytic
An aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages.
Variantes genéticas (ClinVar)
593 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
51 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 dendríticas
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
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Outros ensaios clínicos
56 ensaios clínicos encontrados, 20 ativos.
Publicações mais relevantes
Common ancestral origin of indeterminate dendritic cell tumor and chronic myelomonocytic leukemia in clonal hematopoiesis.
Not available.
Effects of the Alkylating Agent Cyclophosphamide in Potentiating Anti-Tumor Immunity.
Cyclophosphamide (CPX) is an alkylating agent commonly used for various hematological and solid malignancies. In addition to its use as a cytotoxic agent to directly kill tumor cells, numerous immunomodulatory properties of CPX in the tumor microenvironment (TME) of several cancer types have also been documented. These properties include the selective depletion of immune-suppressive regulatory T cells (Tregs), triggering of immunogenic cell death (ICD) and enhanced antigen presentation, and release of type I interferons (IFNs). Moreover, preclinical models as well as human clinical trials have investigated the efficacy of the low-dose "metronomic" scheduling of CPX in combination with immunotherapies such as immune checkpoint inhibitors, dendritic cell tumor vaccines, and tumor antigen peptide vaccines. The metronomic dosing schedule involves administering a continuous (or frequent, such as daily) low dose of chemotherapy rather than using the canonical approach of administering the maximum tolerated dose. Despite the approval of immune checkpoint inhibitors for clinical usage against an increasing number of cancers, many malignancies simply do not respond to checkpoint inhibition, in part due to the heterogeneous intratumoral network of immune-suppressive cell populations. The immunomodulatory effects of cyclophosphamide have strong translational applicability and could serve to enhance and bolster anti-tumor immunity, potentially synergizing with immune checkpoint inhibitors and other existing immunotherapy agents.
EBV-positive inflammatory follicular dendritic cell sarcoma of fibroblastic reticular cell phenotype arising in intrapancreatic accessory spleen.
Epstein-Barr Virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (EBV-IFDCS) is a rare tumor of low malignant potential. Abundant lymphocytes and plasma cells often infiltrate into this tumor tissue with lymphoid follicles and sarcoid-like granulomas, so that the differential diagnosis of this tumor includes inflammatory lesions such as IgG4-related disease, morphologically. This tumor typically arises in the spleen or liver, and shows follicular dendritic cell (FDC)-like immunophenotype. Here we presented a case of EBV-IFDCS arising in intrapancreatic accessory spleen, which was preoperatively considered as a pancreatic tumor. Immunohistochemistry revealed its fibroblastic reticular cell-like phenotype, namely alpha SMA, l-caldesmon, tenascin C, and transglutaminase II positivity, while FDC marker expression such as CD21 and clusterin was focal. Positivity for EBV-encoded small RNA in situ hybridization (EBER ISH) distinguished it from inflammatory lesions or conventional FDC sarcoma. The differential diagnosis of EBV-IFDCS is broad, ranging from non-neoplastic lesions to sarcoma, particularly when it arises in unusual sites. Therefore, careful gross and microscopic examination and adequate immunohistochemistry application including EBER ISH are important to diagnose it properly.
Tumor Vaccines for Malignant Melanoma: Progress, Challenges, and Future Directions.
Malignant melanoma, characterized by its high metastatic potential and resistance to conventional therapies, presents a major challenge in oncology. This review explores the current status and advancements in tumor vaccines for melanoma, focusing on peptide, DNA/RNA, dendritic cell, tumor cell, and neoantigen-based vaccines. Despite promising results, significant challenges remain, including the immunosuppressive tumor microenvironment, patient heterogeneity, and the need for more effective antigen presentation. Recent strategies, such as combining vaccines with immune checkpoint inhibitors (ICIs), aim to counteract immune evasion and enhance T cell responses. Emerging approaches, including personalized neoantigen vaccines and the use of self-amplifying RNA platforms, hold promise for overcoming tumor heterogeneity and improving vaccine efficacy. Additionally, optimizing vaccine delivery systems through nanotechnology and genetic modifications is essential for increasing stability and scalability. This review highlights the potential of these innovative strategies to address current limitations, with a focus on how future research can refine and combine these approaches to improve melanoma treatment outcomes.
Stealth missiles with precision guidance: A novel multifunctional nano-drug delivery system based on biomimetic cell membrane coating technology.
Nanodrug delivery systems (NDDSs) have demonstrated broad application prospects in disease treatment, prevention, and diagnosis due to their nanoscale size advantages and high drug-loading capacity. However, their clinical translation still faces multiple challenges, including rapid clearance by the reticuloendothelial system (RES), nonspecific targeting, and insufficient efficiency in crossing biological barriers. Cell membrane-coated biomimetic delivery systems (CMC-BDS), which integrates natural cell membranes onto nanoparticle (NPs) surfaces, provides nanodrugs with a versatile "biomimetic cloak," representing a highly promising surface engineering strategy. This approach enables nanocarriers to inherit the intrinsic biological properties of different cell sources, endowing them with immune evasion, prolonged circulation, dynamic targeting, biocompatibility, and biodegradability, while supporting the integration of diverse biomedical functions. Furthermore, surface functionalization modifications can enhance their programmability, multifunctionality, and biointerface adaptability, thereby optimizing targeted delivery efficiency and extending in vivo circulation time. This review first outlines the development and key preparation steps of cell membrane coating technology. It then discusses the selection strategies for various cell membrane types-including leukocyte, erythrocyte, platelet, dendritic cell, tumor cell, and bacterial membranes-while comparing their respective advantages and limitations. Finally, the review highlights recent advances in applying cell membrane-coated nanoparticles (CMC-NPs) for treating tumors, ischemic stroke, and inflammatory diseases.
Publicações recentes
Common ancestral origin of indeterminate dendritic cell tumor and chronic myelomonocytic leukemia in clonal hematopoiesis.
EBV-positive inflammatory follicular dendritic cell sarcoma of fibroblastic reticular cell phenotype arising in intrapancreatic accessory spleen.
Tumor Vaccines for Malignant Melanoma: Progress, Challenges, and Future Directions.
Effects of the Alkylating Agent Cyclophosphamide in Potentiating Anti-Tumor Immunity.
Stealth missiles with precision guidance: A novel multifunctional nano-drug delivery system based on biomimetic cell membrane coating technology.
📚 EuropePMC173 artigos no totalmostrando 86
Common ancestral origin of indeterminate dendritic cell tumor and chronic myelomonocytic leukemia in clonal hematopoiesis.
HaematologicaEBV-positive inflammatory follicular dendritic cell sarcoma of fibroblastic reticular cell phenotype arising in intrapancreatic accessory spleen.
Journal of clinical and experimental hematopathology : JCEHTumor Vaccines for Malignant Melanoma: Progress, Challenges, and Future Directions.
Oncology researchEffects of the Alkylating Agent Cyclophosphamide in Potentiating Anti-Tumor Immunity.
International journal of molecular sciencesStealth missiles with precision guidance: A novel multifunctional nano-drug delivery system based on biomimetic cell membrane coating technology.
Materials today. BioA Rare Case of Follicular Dendritic Cell Sarcoma of Tonsil-Clinical Report.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India[Cell therapy with tumor infiltrating lymphocytes; a therapeutic alternative for malignant melanoma and soon also other types of cancer].
LakartidningenRadiotherapy for cytokeratin-positive interstitial reticulum cell (CIRC) tumor on epicardium: a case report.
International cancer conference journalWidespread Cutaneous Indeterminate Dendritic Cell Tumor (IDCT) With ETV3::NOAC2 Rearrangement Successfully Treated With PUVA Therapy: A Case Report.
Case reports in hematologyAn in vitro investigation into the cytotoxic impact of antigen-presenting dendritic cell-tumor infiltrating lymphocytes on ovarian cancer cells.
BMC cancerBlastic plasmacytoid dendritic cell neoplasm: Two case reports.
World journal of clinical oncologyA rare case of an EBV-positive inflammatory follicular dendritic cell tumor of the spleen.
Journal of surgical case reportsImmunotherapeutic allogeneic dendritic cell and autologous tumor cell fusion vaccine alone or combined with radiotherapy in canine oral malignant melanoma is safe and potentially effective.
Frontiers in veterinary scienceA sarcomatoid malignancy originating in the right cervical lymph nodes with atypical pathological characteristics: a case report of an incidental finding.
AME case reportsMalignant Dendritic Cell Sarcomas in the Skin: 2 Cases of Rare Sarcoma Subtypes With Literature Review.
The American Journal of dermatopathologyOverall clinical course of indeterminate dendritic cell tumor patients without skin lesions: A rare case report.
World journal of clinical casesA Rare Case of Follicular Dendritic Cell Tumor of the Abdominal Cavity Visualized by 18 F-AlF-NOTA-Octreotide PET/CT.
Clinical nuclear medicine[Plasmacytoid dendritic cell tumor of the testis in a child: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathology[Blastic plasmacytoid dendritic cell tumor treated with DVT regimen: a case report and literature review].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiGlobal trends in tumor microenvironment-related research on tumor vaccine: a review and bibliometric analysis.
Frontiers in immunologyRecurrent Chemotherapy Treated Indeterminate Dendritic Cell Tumor: Case Report and Literature Review.
Clinical, cosmetic and investigational dermatologyInflammatory Pseudotumor of the Liver.
Surgical pathology clinicsPrimary cutaneous interdigitating dendritic cell sarcoma (IDCS): Report of a new case and literature review.
Pathology, research and practiceMagnetic nanoparticles enhance the cellular immune response of dendritic cell tumor vaccines by realizing the cytoplasmic delivery of tumor antigens.
Bioengineering & translational medicineBlastic plasmacytoid dendritic cell tumor in children.
Minerva pediatrics[Clinical study of mesenchymal stem cells from third-party donors in the treatment of refractory late onset hemorrhagic cystitis after allogeneic hematopoietic stem cell transplanation].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiBlastic Indeterminate Dendritic Cell Tumor Associated With Chronic Myelomonocytic Leukemia.
The American Journal of dermatopathologyDevelopment of a Dendritic Cell/Tumor Cell Fusion Cell Membrane Nano-Vaccine for the Treatment of Ovarian Cancer.
Frontiers in immunologyTypical leonine facies in a patient with indeterminate dendritic cell tumor.
International journal of dermatologyFollicular dendritic cell sarcoma.
PathologicaHepatic inflammatory pseudotumor-like follicular dendritic cell tumor with hepatic lymphoma history: A case report and literature review.
MedicineA new PD-1-specific nanobody enhances the antitumor activity of T-cells in synergy with dendritic cell vaccine.
Cancer lettersInflammatory pseudotumor-like follicular dendritic cell tumor of the spleen: a case report and approach to differential diagnosis.
Radiology case reportsFollicular dendritic cell tumor in the context of a Castleman disease in a patient with Diffuse Large B Cell Lymphoma: Diagnosis and follow-up with 18F-FDG PET/CT.
Revista espanola de medicina nuclear e imagen molecularHepatic inflammatory pseudotumor-like follicular dendritic cell tumor: a case report.
Journal of medical case reportsDisseminated Indeterminate Dendritic Cell Tumor: A Rare Presentation.
Indian dermatology online journalA Pedigree Analysis and Clonal Correlations of the Coexistence of B-Cell Lymphoma and Histiocytic/Dendritic Cell Tumor.
International journal of surgical pathologyInterdigitating Dendritic Cell Tumor of Submandibular Lymph Node: Case Report and Literature Review.
Iranian journal of pathologyDendritic Cell Tumor Vaccination via Fc Gamma Receptor Targeting: Lessons Learned from Pre-Clinical and Translational Studies.
VaccinesIndeterminate Dendritic Cell Tumor With Persistent Complete Metabolic Response to BRAF/MEK Inhibition.
HemaSphereImmune Checkpoint Blockade Enhances Immune Activity of Therapeutic Lung Cancer Vaccine.
Vaccines[Dendritic cell tumor vaccine assisted by Tiaohengfang polysaccharides enhances immune function of S180 tumor-bearing mice and inhibits tumor growth].
Xi bao yu fen zi mian yi xue za zhi = Chinese journal of cellular and molecular immunology[Clinicopathological characteristics of indeterminate dendritic cell tumor of four cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyDescriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience.
Yonsei medical journalIndeterminate dendritic cell tumor in the pancreas.
Journal of surgical case reportsIndeterminate Dendritic Cell Tumor as Cutaneous Involvement of Chronic Myelomonocytic Leukemia Successfully Treated With Phototherapy.
The American Journal of dermatopathologyDendritic Cell-Based Therapy Using LY6E Peptide with a Putative Role Against Colorectal Cancer.
ImmunoTargets and therapySpinal Cord Compression Caused by Fibroblastic Reticular Cell Tumor (FRCT) Originating from Thoracic Spine.
World neurosurgeryRelative hematopoietic stem cell transplantation for the treatment of blastic plasmacytoid dendritic cell neoplasm: a case report and literature review.
International journal of clinical and experimental pathologyPancreatic Inflammatory Pseudotumor-Like Follicular Dendritic Cell Tumor.
Case reports in pathologyImaging findings of inflammatory pseudotumor-like follicular dendritic cell tumors of the liver: Two case reports and literature review.
World journal of gastroenterologyPost-scabietic nodules: Mimicker of infantile indeterminate cell histiocytosis and potential diagnostic pitfall.
Journal of cutaneous pathologyHistiocytic and Dendritic Cell Neoplasms.
Surgical pathology clinicsParaneoplastic disorders associated with miscellaneous neoplasms with focus on selected soft tissue and Undifferentiated/ rhabdoid malignancies.
Seminars in diagnostic pathologyHost Immunity Following Near-Infrared Photoimmunotherapy Is Enhanced with PD-1 Checkpoint Blockade to Eradicate Established Antigenic Tumors.
Cancer immunology researchA Case of Indeterminate Dendritic Cell Tumor: A Rare Neoplasm with Langerhans Cell Lineage.
Annals of dermatologyPredictors of Response to Autologous Dendritic Cell Therapy in Glioblastoma Multiforme.
Frontiers in immunologyInterdigitating dendritic cell tumor: A rare case report with review of literature.
Journal of cancer research and therapeuticsIndeterminate Dendritic Cell Tumor: A Report of Two New Cases Lacking the ETV3-NCOA2 Translocation and a Literature Review.
The American Journal of dermatopathologyVaccine therapy in hematologic malignancies.
BloodRecurrent Indeterminate Dendritic Cell Tumor of the Skin.
Journal of pathology and translational medicineInflammatory pseudotumor-like follicular dendritic cell tumor of the spleen.
The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology[Clinicopathologic and molecular characteristics of malignant gastrointestinal neuroectodermal tumors].
Zhonghua bing li xue za zhi = Chinese journal of pathologyCellular Immunotherapy for Hematologic Malignancies: Beyond Bone Marrow Transplantation.
Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow TransplantationAggressive Indeterminate Dendritic Cell Tumor Mimicking Scalp Angiosarcoma.
Annals of dermatologyChronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor: Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia.
Journal of cutaneous pathologyAdvances in immunotherapy in multiple myeloma.
Current opinion in oncologyIndeterminate Dendritic Cell Tumor in Thoracic Spine: A Case Report.
World neurosurgerySplenic Epstein-Barr Virus-Associated Inflammatory Pseudotumor.
Archives of pathology & laboratory medicineHepatic inflammatory pseudotumour-like follicular dendritic cell tumor: A case report.
Molecular and clinical oncology[Indeterminate dendritic cell tumor:report of two cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyImmunomonitoring reveals interruption of anergy after vaccination in a case of type-2-papillary renal cell carcinoma.
ImmunotherapyParaneoplastic Pemphigus Associated with Follicular Dendritic Cell Tumor in the Mediastinum.
Case reports in dermatological medicineGene Expression Profile of Dendritic Cell-Tumor Cell Hybrids Determined by Microarrays and Its Implications for Cancer Immunotherapy.
Journal of immunology researchThe impact of dendritic cell-tumor fusion cells on cancer vaccines - past progress and future strategies.
ImmunotherapyEpstein-Barr virus-associated inflammatory pseudotumor presenting as a colonic mass.
Human pathologyPrimary Indeterminate Dendritic Cell Tumor of Skin Correlated to Mosquito Bite.
MedicineA case of indeterminate dendritic cell tumor presenting with leonine facies.
Journal of cutaneous pathologyIndeterminate Dendritic Cell Tumor: A Case Report of a Rare Langerhans Cell Lineage Disease.
Journal of pathology and translational medicineResection is an effective treatment for recurrent follicular dendritic cell sarcoma from retroperitoneum: unusual presentation of a rare tumor.
International journal of clinical and experimental medicineFollicular dendritic cell sarcoma with extensive lymph node involvement: A case report.
Oncology lettersFollicular Dendritic Cell Sarcoma Mediastinum - a case report.
Indian journal of surgical oncologyClinicopathological features of angiomatoid fibrous histiocytoma: a series of 21 cases with variant morphology.
International journal of clinical and experimental pathologyInflammatory Pseudotumor-like Follicular Dendritic Cell Tumor of the Spleen: Case Report and Review of the Literature.
Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in MedicineMultimodality imaging of Epstein-Barr virus-associated inflammatory pseudotumor-like follicular dendritic cell tumor of the spleen: case report and literature review.
Clinical imagingInterdigitating dendritic cell sarcoma.
Journal of the National Comprehensive Cancer Network : JNCCNAssociações
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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.
- Common ancestral origin of indeterminate dendritic cell tumor and chronic myelomonocytic leukemia in clonal hematopoiesis.
- Effects of the Alkylating Agent Cyclophosphamide in Potentiating Anti-Tumor Immunity.
- EBV-positive inflammatory follicular dendritic cell sarcoma of fibroblastic reticular cell phenotype arising in intrapancreatic accessory spleen.
- Tumor Vaccines for Malignant Melanoma: Progress, Challenges, and Future Directions.
- Stealth missiles with precision guidance: A novel multifunctional nano-drug delivery system based on biomimetic cell membrane coating technology.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98289(Orphanet)
- MONDO:0020082(MONDO)
- GARD:8317(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q56014397(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
