Tumores raros que afetam os tecidos que formam o sangue e os do sistema de defesa do corpo (sistema linfático). As células que dão origem a esses tumores são os histiócitos e as células acessórias, que são tipos de células de defesa do corpo. Podem acontecer em pessoas de qualquer idade e sua ocorrência não varia muito de uma região geográfica para outra. Essa categoria inclui o sarcoma histiocítico, histiocitose de células de Langerhans, sarcoma de células de Langerhans, sarcoma/tumor de células dendríticas interdigitantes, sarcoma/tumor de células dendríticas foliculares, e sarcoma de células dendríticas, sem outra especificação. (OMS, 2001)
Introdução
O que você precisa saber de cara
Tumores raros que afetam os tecidos que formam o sangue e os do sistema de defesa do corpo (sistema linfático). As células que dão origem a esses tumores são os histiócitos e as células acessórias, que são tipos de células de defesa do corpo. Podem acontecer em pessoas de qualquer idade e sua ocorrência não varia muito de uma região geográfica para outra. Essa categoria inclui o sarcoma histiocítico, histiocitose de células de Langerhans, sarcoma de células de Langerhans, sarcoma/tumor de células dendríticas interdigitantes, sarcoma/tumor de células dendríticas foliculares, e sarcoma de células dendríticas, sem outra especificação. (OMS, 2001)
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
+ 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 histiocíticas e dendríticas
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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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
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Myeloid sarcoma (MS) is a tumorous extramedullary proliferation of blast or blast equivalent cells (e.g., promonocytes or promyelocytes). The most frequent cutaneous presentation is often referred to as leukemia cutis (LC). These lesions, especially without the clinical context of a known bone marrow disease, pose a differential diagnostic challenge. In this retrospective multicenter clinico-pathological study on 154 patients with MS or LC, 169 samples were analyzed by morphology, immunohistochemistry, and fluorescent in situ hybridization, and a subset by additional sequencing [TP53]. The majority of cases were lysozyme positive (diffuse in 91% and focal in 5%), 51% showed diffuse and 6% focal expression of CD56, and IRF8 was strongly positive in 31% of the lesions. Lack of myeloperoxidase (MPO), CD117, and CD34 expression was observed in 27%, 39%, and 58%, respectively. PU.1 was positive in almost all instances (95%), but BRAF V600E was consistently negative. CD123 was diffusely (13%) or focally (25%) positive, which, in addition to frequent CD4 (73%) and CD56 expression, pointed to a phenotypic overlap with blastic plasmacytoid dendritic cell neoplasms. Survival analysis revealed that MS occurring at sanctuary sites (CNS, orbit, ovary, and testis) was characterized by excellent survival. Similarly to histiocytoses, there was a prognostic difference between isolated and multisystemic involvement by MS. Patients who underwent allogeneic hematopoietic stem cell transplantation showed significantly improved survival. In conclusion, this multicenter study suggests that most MS are of myelomonocytic/monoblastic origin, a high proportion of them are NPM1 mutated, and may lack expression of MPO and CD34. NPM1 mutation-specific antibodies should be integrated into the diagnostic panels for MS or LC, while IRF8 and PU.1 are not recommended as they cannot distinguish MS from histiocytic neoplasms.
Primary pulmonary histiocytic sarcoma with CNS metastasis: a case report and molecular profiling insights.
Histiocytic sarcoma (HS), reclassified in the WHO fifth edition as a Histiocytic/dendritic cell neoplasms, represents a rare hematopoietic malignancy with extranodal predominance and aggressive clinical behavior. This study reports the case of a 53-year-old female diagnosed with primary pulmonary HS, who presented with a 60-mm mass in the right middle lobe and later developed fatal brain metastases. Using a combination of pathology, whole-exome sequencing, and fusion gene analysis, we identified key molecular drivers of tumor development and spread. Major findings include the concurrent activation of the RAS/MAPK and PI3K/mTOR pathway activation (118 combined gene variants), TP53 biallelic inactivation, HLA locus alterations, and persistent LOC285045 fusions. Drug sensitivity profiling suggested potential responses to sunitinib and MEK inhibitors. By comparing this case with nine other reported cases of lung HS, we found that lung HS has a significantly worse survival (p=0.03) than HS at other sites. A high cell growth rate (Ki-67 >30%) and large tumor size (>50 mm) were identified as critical indicators of poor prognosis.
Clinical and Pathological Characteristics of Skin Cancer Patients at Southern Medical University, Guangzhou, China.
The incidence of skin cancer is increasing in China, yet large-scale clinical and pathological data from China remain scarce. A retrospective analysis was conducted of 648 histopathologically confirmed skin cancer cases diagnosed at the Dermatology Hospital, Southern Medical University from January 2020 to July 2023. Patient demographics, tumour distribution, histological subtypes, and treatment approaches were evaluated. Squamous cell carcinoma (31.9%) and basal cell carcinoma (31.2%) were the most prevalent, followed by cutaneous lymphomas (13.9%), dendritic cell and histiocytic neoplasms (13.3%), and melanoma (9.7%). Over half of the patients (53.5%) were aged ≥ 60 years. basal cell carcinoma and squamous cell carcinoma primarily involved the head and neck, while acral sites were dominant in melanoma (79.4%). Most cases of both basal cell carcinoma and squamous cell carcinoma were managed surgically, with surgical treatment performed in 95.5% of basal cell carcinomas and 94.2% of squamous cell carcinomas. Skin cancers in China predominantly affect older adults and commonly involve sun-exposed areas. Basal cell carcinoma is typically managed surgically due to its low-risk nature, while squamous cell carcinoma demonstrates clinical heterogeneity, warranting varied treatment strategies. The higher proportion of cutaneous lymphomas among younger patients highlights the need for age-specific diagnostic and therapeutic considerations.
Histiocytic Sarcoma Treated With Autologous Stem Cell Transplantation: A Case Report and Literature Review of the Role of Autologous and Allogenic Stem Cell Transplantation.
BACKGROUND Histiocytic sarcoma is a rare and aggressive hematopoietic malignancy, characterized by tumor cells exhibiting features of histiocytes or dendritic cells. Extranodal involvement, particularly in the gastrointestinal tract, skin, and soft tissues, is common and often associated with aggressive clinical behavior and poor outcomes. Due to its rarity, no standardized treatment has been established. Current therapeutic approaches are generally adapted from aggressive non-Hodgkin lymphoma protocols, typically involving multi-agent chemotherapy. However, therapeutic responses are often suboptimal, with frequent relapses. In selected cases, hematopoietic stem cell transplantation has been employed following intensive chemotherapy for disease control. CASE REPORT A 44-year-old woman presented with pancytopenia and was initially diagnosed with immune thrombocytopenia. After splenectomy for refractory disease, histopathlogic examination of spleen confirmed histiocytic sarcoma. She received 6 cycles of chemotherapy with cyclophosphamide, doxorubicin (hydroxydaunorubicin), vincristine (Oncovin), etoposide, and prednisone. Despite an initial response, the disease progressed. She underwent high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation, but ultimately died due to disease relapse. CONCLUSIONS This case demonstrates the highly aggressive course and poor prognosis of histiocytic sarcoma, even after intensive chemotherapy and autologous stem cell transplanatation. Given the highly aggressive nature of histiocytic sarcoma and its generally poor prognosis, allogeneic stem cell transplantation may offer a more effective strategy for achieving long-term remission, particularly in patients with relapsed or refractory disease. Large-scale studies are needed to establish treatment guidelines for histiocytic sarcoma.
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Liver involvement by histiocytic and dendritic cell neoplasms signals high-risk disease, often necessitating closer monitoring and aggressive management. Severe cases may progress to liver failure, requiring transplantation. Liver involvement occurs in about one-third of patients with systemic juvenile xanthogranuloma (JXG) and 20% to 60% of pediatric patients with Langerhans cell histiocytosis (LCH), particularly in multiorgan disease. Tyrosine kinase inhibitors show promise in LCH treatment, but optimal timing for treatment cessation remains uncertain. We present 2 pediatric cases, 1 with LCH, and the other with disseminated JXG, along with a literature review emphasizing liver histopathology and transplant considerations. These cases highlight distinct histological patterns. In LCH, progressive bile duct destruction led to ductopenic cholestatic cirrhosis and secondary sclerosing cholangitis. In contrast, in the case of JXG, bile ducts remained intact despite being surrounded by histiocytes. In both, disease localization to larger, segmental portal tracts may reduce liver biopsy sensitivity. In LCH, BRAF inhibitor therapy triggered a granulomatous reaction that could mimic disease recurrence in the liver graft. Other histiocytoses typically spare the bile ducts and do not cause biliary cirrhosis. Recognizing these distinct infiltration patterns can aid diagnosis and management.
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Pathologie (Heidelberg, Germany)An Analysis of the Pathologic Features of Blastic Plasmacytoid Dendritic Cell Neoplasm Based on a Comprehensive Literature Database of Cases.
Archives of pathology & laboratory medicineFollicular Dendritic Cell Sarcoma of Uterine Corpus: Report of 2 Cases.
International journal of gynecological pathology : official journal of the International Society of Gynecological PathologistsHepatic epithelioid hemangioendothelioma after thirteen years' follow-up: A case report and review of literature.
World journal of clinical cases[Reactive lymphadenopathies].
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HistopathologyThe 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms.
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BloodCyclin D1 Expression and Molecular Genetic Findings in Periocular Histiocytoses and Neoplasms of Macrophage-Dendritic Cell Lineage.
American journal of ophthalmologySpindle cell tumor with histiocytic and myogenic marker expression in the lymph node of a human T-cell leukemia virus type 1 carrier.
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Veterinary and comparative oncologyFollicular dendritic cell sarcoma.
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Journal of comparative pathologyPseudomalignancies in Children: Histological Clues, and Pitfalls to Be Avoided.
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Radiologic clinics of North AmericaFollicular dendritic cell sarcoma of the cervical lymph node diagnosed on fine needle aspiration cytology.
Cytopathology : official journal of the British Society for Clinical CytologyIndeterminate Cell Histiocytosis of the Spine: A Case Report.
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Applied immunohistochemistry & molecular morphology : AIMMCase Report: Eighteen Month Relapse- Free Survival Following Radical Multidisciplinary Oncological Treatment in a 68-Year-Old Male Patient With Histiocytic Sarcoma.
Frontiers in oncologyAn advanced case of gastric histiocytic sarcoma treated with chemotherapy and gastrectomy: a case report and review of literature.
Clinical journal of gastroenterologyDistinct immune signatures in chronic lymphocytic leukemia and Richter syndrome.
Blood cancer journalA Pedigree Analysis and Clonal Correlations of the Coexistence of B-Cell Lymphoma and Histiocytic/Dendritic Cell Tumor.
International journal of surgical pathologyHistiocytic and Dendritic Cell Sarcomas of Hematopoietic Origin Share Targetable Genomic Alterations Distinct from Follicular Dendritic Cell Sarcoma.
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Indian journal of pathology & microbiologyExpression of CD1 molecules and colony-stimulating factor 1 receptor in indeterminate cell histiocytosis.
The Journal of dermatologyFrom the archives of MD Anderson Cancer Center: A case of concurrent follicular lymphoma and Langerhans cell sarcoma with a review of the literature.
Annals of diagnostic pathologyHistiocytosis and Neoplasms of Macrophage-Dendritic Cell Lineages: Multimodality Imaging with Emphasis on PET/CT.
Radiographics : a review publication of the Radiological Society of North America, IncIonized Calcium Binding Adaptor Molecule 1 (IBA1).
American journal of clinical pathologyCytologic and histological features of rare nonepithelial and nonlymphoid tumors of the thyroid.
Cancer cytopathologyNodal histiocytic sarcoma with prominent eosinophilic infiltration: expression of eotaxin-2 on tumor cells.
Diagnostic pathologyAtypical cutaneous histiocytic eruption in a patient with chronic myelomonocytic leukemia: A case report.
Journal of cutaneous pathology[Histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Comparison of recent WHO classification published 2017 and classification of Histiocyte Society published 2016].
Vnitrni lekarstviIdentification of hub genes associated with the pathogenesis of diffuse large B-cell lymphoma subtype one characterized by host response via integrated bioinformatic analyses.
PeerJHistiocytic and dendritic cell neoplasms: Reappraisal of a Japanese series based on t(14;18) and neoplastic PD-L1 expression.
Pathology internationalMyeloid, mast cell, histiocytic and dendritic cell neoplasms and proliferations involving the spleen.
Seminars in diagnostic pathologyExtranodal Follicular Dendritic Cell Sarcoma-A Review: "What the Mind Does Not Know the Eye Does Not See".
Advances in anatomic pathologyThe mutational landscape of histiocytic sarcoma associated with lymphoid malignancy.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncSolitary congenital Langerhans cell histiocytoma: A pattern of benign, spontaneous regression in patients with single lesion disease.
Pediatric dermatologyImmunophenotyping of Nonneoplastic and Neoplastic Histiocytes in Cats and Characterization of a Novel Cell Line Derived From Feline Progressive Histiocytosis.
Veterinary pathologyDescriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience.
Yonsei medical journalSpectrum of histiocytic neoplasms associated with diverse haematological malignancies bearing the same oncogenic mutation.
The journal of pathology. Clinical researchHistological and Immunohistochemical Features of Normal Histiocytes and Langerhans Cells, and Histiocytic Sarcomas in Four-Toed Hedgehogs (Atelerix albiventris).
Journal of comparative pathology[Erdheim-Chester disease-A histiocytic multisystem disease with unusual renal involvement].
Der InternistIndeterminate dendritic cell tumor in the pancreas.
Journal of surgical case reports[Peripheral T-cell lymphoma with follicular helper of T cell phenotype of Waldeyer's ring: a clinicopathological and genetic study of eight cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyFine-Needle Cytological Characteristics of Carcinoma Breast with Medullary or Medullary-like Features Masquerading as Dendritic Reticulum Cell Sarcoma: An Attempt to Explore the Reasons for Erroneous Cytologic Interpretation.
Journal of cytologyHistiocytic and dendritic cell neoplasms of the mediastinum.
Mediastinum (Hong Kong, China)A Remarkable Response of Granulomatous Hypophysitis to Infliximab in a Patient With a Background of Crohn's Disease-A Case Report.
Frontiers in endocrinologySuperficial dermal melanocytosis of the elderly: An exceptional occurrence?
Journal of cutaneous pathologyLangerhans cell histiocytosis of an intra-mammary lymph node in an 18-year-old woman.
PathologicaHematolymphoid Neoplasms Rarely Mimic Undifferentiated Pleomorphic Sarcoma of Soft Tissue.
Archives of pathology & laboratory medicineCould cathepsin-k be a driver of the myofibroblastic differentiation observed in dermatofibroma, atypical fibroxanthoma and pleomorphic dermal sarcoma?
Acta histochemicaConcurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma in a patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma.
Journal of cutaneous pathologyUpdate on the classification of T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms.
Expert review of hematology[Clinicopathological characteristics and differential diagnosis of interdigitating dendritic cell sarcoma].
Zhonghua zhong liu za zhi [Chinese journal of oncology]Angiofibroma of the Eyelid: A Rare Clinical and Histologic Variant.
Ophthalmic plastic and reconstructive surgeryNon-infectious granulomatous dermatoses.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGGenome-wide DNA copy number analysis and targeted transcriptional analysis of canine histiocytic malignancies identifies diagnostic signatures and highlights disruption of spindle assembly complex.
Chromosome research : an international journal on the molecular, supramolecular and evolutionary aspects of chromosome biologySOX-10 staining in dermal scars.
Journal of cutaneous pathologyClinical significance of the two-base insertion mutation in the TP53 gene in canine histiocytic sarcoma.
Research in veterinary scienceAmerican Registry of Pathology Expert Opinions: Immunohistochemical evaluation of classic Hodgkin lymphoma.
Annals of diagnostic pathologyThe Immunology of Macrophage Activation Syndrome.
Frontiers in immunologyClinical efficacy of bortezomib and lenalidomide in blastic plasmacytoid dendritic cell neoplasm.
Annals of hematologyCutaneous adult xanthogranuloma with a small portion of BRAFV 600E mutated Langerhans cell histiocytosis populations: A case report and the review of published work.
The Journal of dermatologyLangerhans Cell Histiocytosis of the Skull in a Burr Hole Site Covered with Hydroxyapatite Material.
World neurosurgeryHistiocytic Sarcoma: Review, Discussion of Transformation From B-Cell Lymphoma, and Differential Diagnosis.
Archives of pathology & laboratory medicineHistiocytic Sarcoma Associated With Follicular Lymphoma: Evidence for Dramatic Response With Rituximab and Bendamustine Alone and a Review of the Literature.
Clinical lymphoma, myeloma & leukemiaKRAS mutation in secondary malignant histiocytosis arising from low grade follicular lymphoma.
Diagnostic pathologyIndeterminate dendritic cell neoplasm accompanied by eosinophilic pneumonia successfully treated by systemic steroid therapy: Report of the first case with muscular and parotid involvement and review of published work.
The Journal of dermatologyEpithelioid Fibrous Histiocytoma: A Concise Review.
The American Journal of dermatopathologyA Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a+) Mimicking Glioblastoma Multiforme in a Young Adult.
World neurosurgerySynchronous case of follicular lymphoma and Langerhans cell sarcoma in the same lymph node.
Pathology internationalLangerhans Cell Histiocytoma: A Benign Histiocytic Neoplasm of Diverse Lines of Terminal Differentiation.
The American Journal of dermatopathologyBiallelic inactivation of the retinoblastoma gene results in transformation of chronic myelomonocytic leukemia to a blastic plasmacytoid dendritic cell neoplasm: shared clonal origins of two aggressive neoplasms.
Blood cancer journalMany faces of the same myeloid neoplasm: a case of leukaemia cutis with mixed histiocytic and Langerhans cell differentiation.
Journal of clinical pathologyPigmented Epithelioid Melanocytoma (Animal Types of Melanoma) on the Nose.
Case reports in oncology[Histiocytic and dendritic cell neoplasms: Review of the literature].
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de CitologiaEstablishment and characterization of a cell line from a feline histiocytic sarcoma.
Veterinary immunology and immunopathologyEpstein-Barr virus (EBV)-associated lymphoid proliferations, a 2018 update.
Human pathologyIndeterminate Dendritic Cell Tumor: A Report of Two New Cases Lacking the ETV3-NCOA2 Translocation and a Literature Review.
The American Journal of dermatopathologyPD-1 Expression in Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and Large B-cell Richter Transformation (DLBCL-RT): A Characteristic Feature of DLBCL-RT and Potential Surrogate Marker for Clonal Relatedness.
The American journal of surgical pathologyExpression of the transcription factor ZBTB46 distinguishes human histiocytic disorders of classical dendritic cell origin.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncThe t(14;18) translocation is absent from endothelial and follicular dendritic cells of follicular lymphoma (FL) and shows heterogeneous presence in preserved FL mantle zones.
Diagnostic pathologyInterdigitating dendritic cell sarcoma: Clinicopathologic study of 8 cases with review of the literature.
Annals of diagnostic pathologyApplicability of 2008 World Health Organization classification system of hematolymphoid neoplasms: Learning experiences.
Indian journal of pathology & microbiologyMolecular characterization of the histiocytoses: Neoplasia of dendritic cells and macrophages.
Seminars in cell & developmental biologyA novel canine histiocytic sarcoma cell line: initial characterization and utilization for drug screening studies.
BMC cancerE-Selectin Ligands in the Human Mononuclear Phagocyte System: Implications for Infection, Inflammation, and Immunotherapy.
Frontiers in immunologyHistiocytosis - cutaneous manifestations of hematopoietic neoplasm and non-neoplastic histiocytic proliferations.
Journal of the European Academy of Dermatology and Venereology : JEADVExpression of enhancer of zeste homolog 2 (EZH2) protein in histiocytic and dendritic cell neoplasms with evidence for p-ERK1/2-related, but not MYC- or p-STAT3-related cell signaling.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncPrognostic factors for histiocytic and dendritic cell neoplasms.
OncotargetLymph Node Haematopoietic, Histiocytic, Dendritic Proliferations and Other Lymphoid Organs.
Monographs in clinical cytologyAssociações
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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.
- Frequent NPM1 mutation, monoblastic/monocytic origin and prognostic significance of organ and system involvement in myeloid sarcoma: a multicenter study.
- Primary pulmonary histiocytic sarcoma with CNS metastasis: a case report and molecular profiling insights.
- Clinical and Pathological Characteristics of Skin Cancer Patients at Southern Medical University, Guangzhou, China.
- Histiocytic Sarcoma Treated With Autologous Stem Cell Transplantation: A Case Report and Literature Review of the Role of Autologous and Allogenic Stem Cell Transplantation.
- Bile Duct Targeting or Preservation: Contrasting Liver Histology in Langerhans Cell Histiocytosis and Disseminated Juvenile Xanthogranuloma.Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society· 2026· PMID 41170802mais citado
- Nodal histiocytic sarcoma mimicking metastatic carcinoma, a challenging diagnosis - Case report of an extremely rare hematolymphoid neoplasm with recent updates.
- Plasmacytoid dendritic cell proliferations, malignant histiocytic (macrophage/dendritic cell) neoplasms and follicular dendritic cell neoplasms: report from the 2024 Joint CSHP/EA4HP/SH workshop.
- Fifth edition WHO classification: precursor lymphoid neoplasms, acute leukaemias of mixed or ambiguous lineage, myeloid/lymphoid neoplasms, and histiocytic and dendritic cell neoplasms, including strategies for application in resource-limited settings.
- Molecular Pathogenesis of the Histiocytic and Dendritic Cell Neoplasms.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98287(Orphanet)
- MONDO:0006247(MONDO)
- GARD:19441(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q18556903(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
