Síndrome de Gerstmann é um distúrbio neurológico raro caracterizado por lesões no giro angular do hemisfério cerebral dominante. O giro angular situa-se no lobo parietal, próximo ao lobo temporal. Nomeado em homenagem a Josef Gerstmann, pode eventualmente mudar de nome para Síndrome Angular por recomendação da comunidade científica.
Introdução
O que você precisa saber de cara
Neoplasia rara associada a infecção viral, manifestando-se com anemia, derrames (pleural, pericárdico), distensão abdominal, obstrução intestinal, linfadenopatia e alterações hematológicas. Pode envolver carcinoma de mama, linfoma de células B e anormalidades na medula óssea e peritônio.
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Entender a doença
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Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 33 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 91 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.
Transcription factor that binds DNA in a non-specific manner, yet also specifically recognizes the core sequence 5'-CAC[GA]TG-3' (PubMed:24940000, PubMed:25956029). Activates the transcription of growth-related genes (PubMed:24940000, PubMed:25956029). Binds to the VEGFA promoter, promoting VEGFA production and subsequent sprouting angiogenesis (PubMed:24940000, PubMed:25956029). Regulator of somatic reprogramming, controls self-renewal of embryonic stem cells (By similarity). Functions with TAF
Nucleus, nucleoplasmNucleus, nucleolusNucleusCytoplasmChromosome
Inhibits the activity of dimeric NF-kappa-B/REL complexes by trapping REL (RELA/p65 and NFKB1/p50) dimers in the cytoplasm by masking their nuclear localization signals (PubMed:1493333, PubMed:36651806, PubMed:7479976). On cellular stimulation by immune and pro-inflammatory responses, becomes phosphorylated promoting ubiquitination and degradation, enabling the dimeric RELA to translocate to the nucleus and activate transcription (PubMed:7479976, PubMed:7628694, PubMed:7796813, PubMed:7878466)
CytoplasmNucleus
Ectodermal dysplasia and immunodeficiency 2
A form of ectoderma dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. This form of ectodermal dysplasia is associated with decreased production of pro-inflammatory cytokines and certain interferons, rendering patients susceptible to infection. EDAID2 inheritance is autosomal dominant.
Involved in pinching off the separated nuclei at the cleavage furrow and in cytokinesis (PubMed:20107318). Required for mitotic integrity and maintenance of chromosomal stability. Protects cells against mitotic errors, centrosomal amplification, micronucleus formation and aneuploidy. Plays a key role of midbody function involving abscission of the daughter cells during cytokinesis and appropriate chromosomal and nuclear segregation into the daughter cells (PubMed:22988245, PubMed:23713010)
CytoplasmMidbody
Lymphoma, Hodgkin, classic
A malignant disease characterized by progressive enlargement of the lymph nodes, spleen and general lymphoid tissue, and the presence of large, usually multinucleate, cells (Reed-Sternberg cells). Reed-Sternberg cells compose only 1-2% of the total tumor cell mass. The remainder is composed of a variety of reactive, mixed inflammatory cells consisting of lymphocytes, plasma cells, neutrophils, eosinophils and histiocytes.
Variantes genéticas (ClinVar)
131 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
33 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 raro induzido por vírus
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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Molecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.
Extranodal NK/T-cell lymphoma (ENKTL) is a rare, aggressive Epstein-Barr virus (EBV)-associated malignancy. The 5th edition of the WHO classification refined their nomenclature by removing "nasal type" and introducing related entities to reflect updated biological insights. This review synthesizes recent advances in epidemiology and molecular pathogenesis in ENKTL, highlighting recurrent driver gene alterations, particularly frequent mutations in X-linked genes such as DDX3X and MSN, alongside TP53 mutations and PD-L1 structural variations that facilitate immune evasion. We also discuss novel mouse models that replicate the ENKTL niche, revealing how Trp53 loss and the EBV oncoprotein LMP1 synergistically remodel the tumor microenvironment to promote lymphomagenesis. Therapeutically, while L-asparaginase-based regimens including SMILE and P-GemOx remain the standard of care, emerging immune checkpoint inhibitors have demonstrated encouraging efficacy, particularly in combination with chemotherapy. This review provides an integrated perspective on the evolving landscape of diagnosis, disease biology, and translational treatment strategies for NK/T-cell lymphoproliferative disorders.
Classic hodgkin lymphoma associated with Epstein-Barr virus reactivation during vedolizumab therapy for ulcerative colitis.
Epstein-Barr virus (EBV)-associated lymphoproliferative disorders have been reported in patients receiving immunosuppressive therapy for inflammatory bowel disease; however, the onset of classic Hodgkin lymphoma (cHL) during vedolizumab therapy is extremely rare. A 73 year-old woman with ulcerative colitis (UC) was steroid-dependent, showing disease exacerbation when prednisolone was tapered or discontinued. Azathioprine was administered eight years prior but was discontinued after a short period due to liver dysfunction. Vedolizumab was initiated 31 months before presentation. Despite initial improvements in UC with vedolizumab, hematochezia subsequently persisted. Other biologics or immunomodulators that could cause systemic immunosuppression were avoided because she was receiving treatment for breast cancer. Colonoscopy showed erosions in the transverse colon and an ulcerative lesion in the sigmoid colon. A biopsy revealed high-grade dysplasia in the transverse colon and inflammatory granulation tissue without malignancy in the sigmoid lesion; therefore, surgery was indicated. Laparoscopic total proctocolectomy was performed, and the surgical specimen contained Reed-Sternberg cells positive for CD20, CD30, PD-L1, and EBV-encoded RNA, establishing EBV-associated cHL. Although the development of cHL is likely multifactorial, clinicians should be aware that EBV-associated intestinal cHL may develop in patients with UC receiving vedolizumab when tumor-like ulcers or mural thickening are encountered.
Human and viral whole genome sequencing identify HPV and APOBEC as oncogenic drivers in sinonasal squamous cell carcinoma.
Sinonasal squamous cell carcinoma (SNSCC) is an aggressive head and neck cancer of the sinonasal cavity which has not benefitted from therapeutic advances over decades1. Though historically attributed to inhaled carcinogens such as hardwood dust and tobacco smoking2, SNSCC is incidentally associated with human papillomavirus (HPV)3,4. Importantly, HPV is the primary oncogenic driver of >80% of anatomically adjacent oropharyngeal cancers5. While viral status drives clinical staging and treatment guidelines in these malignancies6,7, the potentially oncogenic consequences and prognostic value of host-virus interactions in SNSCC remain incompletely defined. Here, through paired host and viral whole-genome sequencing (WGS), we map the genomic footprint of HPV in SNSCC. Strikingly, lesser studied strains such as HPV45, 51, and 39 constitute driver infections in this rare but clinically credentialed cancer, where extrachromosomal DNA (ecDNA)-associated viral integration and APOBEC mutagenesis are shown to underpin somatic tumor evolution.
BK polyomavirus-associated progressive multifocal leukoencephalopathy following mogamulizumab therapy for erythrodermic mycosis fungoides.
BK polyomavirus (BKPyV) is a ubiquitous human pathogen that typically causes nephropathy and hemorrhagic cystitis in immunocompromised patients. Although BKPyV shares close genetic and structural similarity with JC polyomavirus (JCPyV), which is responsible for progressive multifocal leukoencephalopathy (PML), its neurotropic potential remains poorly characterized. Rare reports have suggested possible central nervous system (CNS) involvement under conditions of severe immune suppression. Here, we describe the first documented case of BKPyV-associated PML in a patient with erythrodermic mycosis fungoides treated with Mogamulizumab, a CCR4-targeting monoclonal antibody that profoundly alters immune surveillance. We describe a patient with erythrodermic mycosis fungoides and long-standing immunological frailty, who developed neurological symptoms during Mogamulizumab therapy. Brain MRI showed multifocal white matter lesions compatible with PML. BKPyV DNA was detected in plasma, urine, and cerebrospinal fluid (CSF), while JCPyV DNA was absent. Serological testing showed high anti-BKPyV and anti-JCPyV IgG levels in plasma, indicating prior exposure to both viruses, while antibodies were undetectable in CSF, consistent with lack of intrathecal synthesis. This compartmental dissociation between plasma and CSF, together with the detection of BKPyV DNA and the absence of JCPyV DNA in CSF, supports BKPyV as the etiological neurotropic agent responsible for leukoencephalopathy. Sequencing of the VP1 and NCCR regions revealed compartment-specific nucleotide and amino acid variants, including non-conservative substitutions in the CSF isolate, suggesting intra-host viral heterogeneity. Compartment-specific sequence variability of viral protein 1 (VP1) and structural rearrangements of the non-coding control region (NCCR), particularly the loss of the Q and R block in CSF-derived isolates, underscore intra-host heterogeneity of BKV and may contribute to its adaptation and neurotropic potential. This is the first documented case of BKPyV-associated PML in a Mogamulizumab-treated patient. These findings highlight intra-host heterogeneity at the protein level, possibly reflecting compartment-specific viral evolution, and underscore the need for vigilant BKPyV and JCPyV monitoring during Mogamulizumab treatment.
DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.
A variety of malignancies can be found in the sinonasal tract including the nasal cavity, paranasal sinuses, and skull base. Many of these are attributed to viruses, for example, carcinomas with the presence of transcriptionally active high-risk human papillomavirus, as well as Epstein-Barr virus associated malignant lymphoepithelial carcinoma and haematolymphoid neoplasias. Olfactory neuroblastoma (ONB) is a rare malignant tumor of the olfactory fossa with an unknown etiology. We present the first comprehensive virus study of ONB. By targeted next-generation sequencing for 41 DNA viruses, we investigated the presence of papillomaviruses, herpesviruses, polyomaviruses, and parvoviruses, among others, in 12 ONBs. In addition, herpesvirus presence was evaluated by qPCR. Low-risk HPV6 was detected in one sample. Human endogenous retroviruses were positive in all samples, supporting successful library preparation and sequencing, considered as an internal quality control. No other virus findings were observed. The present broad-scale virus study did not reveal abundant presence of viral DNA in ONB suggesting a limited viral role in its etiology. Nevertheless, our cohort exhibited a finding of low-risk HPV, which has been associated with increased risk of cancer progression of inverted sinonasal papilloma in the same anatomic site advocating for further research. Trial Registration: §31/07.03.2019.
Publicações recentes
Urothelial Carcinoma of the Bladder Following BK Virus Infection in a Pediatric Kidney Transplant Recipient.
[Clinical Characteristics and Prognostic Analysis of Patients with EBV-Positive Diffuse Large B-Cell Lymphoma].
A 43-Year-Old Man With Isolated Cutaneous Lymphomatoid Granulomatosis Presenting as a Chronic Necrotizing Ulcer of the Upper Arm.
Molecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.
Recurrent anti-TIF1γ-positive dermatomyositis coexisting with postoperative parotid lymphoepithelial carcinoma: a case report with pathogenesis analysis.
📚 EuropePMCmostrando 200
DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.
Neuropathology : official journal of the Japanese Society of NeuropathologyPrimary diaphragmatic inflammatory pseudotumour-like follicular/fibroblastic dendritic cell sarcoma mimicking a hepatic tumour: a case report.
Journal of surgical case reportsUrothelial Carcinoma of the Bladder Following BK Virus Infection in a Pediatric Kidney Transplant Recipient.
Pediatric transplantationHPV42: From a Low-Risk Genotype to a Potentially Oncogenic Virus.
Clinical and experimental dermatology[Clinical Characteristics and Prognostic Analysis of Patients with EBV-Positive Diffuse Large B-Cell Lymphoma].
Zhongguo shi yan xue ye xue za zhiA 43-Year-Old Man With Isolated Cutaneous Lymphomatoid Granulomatosis Presenting as a Chronic Necrotizing Ulcer of the Upper Arm.
The American journal of case reportsStereotactic radiosurgery for central nervous system lymphoma: a systematic review and meta-analysis.
Acta neurologica BelgicaMolecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.
Proceedings of the Japan Academy. Series B, Physical and biological sciencesRecurrent anti-TIF1γ-positive dermatomyositis coexisting with postoperative parotid lymphoepithelial carcinoma: a case report with pathogenesis analysis.
Frontiers in immunologyClassic hodgkin lymphoma associated with Epstein-Barr virus reactivation during vedolizumab therapy for ulcerative colitis.
Clinical journal of gastroenterologySuccessful Management of Suspected Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in BRAF-Mutant Cholangiocarcinoma Following Treatment With Immune Checkpoint Inhibitors and BRAF/MEK Inhibitors: A Case Report.
Cancer reports (Hoboken, N.J.)Severe Esophageal Ulceration With Enfortumab Vedotin Plus Pembrolizumab Therapy in Urothelial Carcinoma: A Case Report.
Anticancer researchBispecific Antibodies in B-Cell Lymphomas: Mechanisms, Efficacy, Toxicity, and Management.
Medicina (Kaunas, Lithuania)IDSA 2025 Guidelines on the use of vaccines for the prevention of seasonal COVID-19 infections in immunocompromised patients.
Clinical infectious diseases : an official publication of the Infectious Diseases Society of AmericaSingle-fraction low-energy superficial x-ray radiation for conjunctiva Kaposi sarcoma: A case report.
Archivos de la Sociedad Espanola de OftalmologiaHuman and viral whole genome sequencing identify HPV and APOBEC as oncogenic drivers in sinonasal squamous cell carcinoma.
medRxiv : the preprint server for health sciencesHigh PVR protein expression marks clear cell renal cell carcinoma with metastatic spread.
Frontiers in immunologyRapidly progressing Kaposi sarcoma in a human immunodeficiency virus-negative patient.
Dermatology online journalBK polyomavirus-associated progressive multifocal leukoencephalopathy following mogamulizumab therapy for erythrodermic mycosis fungoides.
Frontiers in cellular and infection microbiologyLack of Rb and p53 Expression in a Case of CM2B4 Negative Merkel Cell Carcinoma In Situ Associated With Squamous Dysplasia.
Journal of cutaneous pathologyLong-term outcomes and patient profiles following intensity-modulated radio-chemotherapy for nasopharyngeal cancer in a nonendemic region.
Frontiers in oncologyEarly diagnosis of extranodal NK/T lymphoma presenting with oral ulcer and lip swelling by metagenomics next-generation sequencing: a case report.
BMC oral healthEpstein-Barr virus (EBV)-associated smooth muscle tumor of the central nervous system in an HIV patient: diagnostic and therapeutic challenges.
BMC infectious diseasesPrimary Orbital Extranodal Natural Killer/T-Cell Lymphoma in a Young African Male: A Case Report.
Case reports in oncologyHemophagocytic lymphohistiocytosis associated with extranodal NK/T cell lymphoma, nasal type or aggressive NK cell leukemia: a retrospective multicenter study of Jiangsu Cooperative Lymphoma Group (JCLG).
Annals of hematologyBevacizumab-Related Gastrointestinal Perforation in Hepatocellular Carcinoma Patient: A Case Report.
Case reports in oncologyCryosurgical cranioplasty using autologous bone for metastatic skull lesion from hepatocellular carcinoma: illustrative case.
Journal of neurosurgery. Case lessonsOrbital Epstein-Barr virus associated smooth muscle tumor in an immunocompromised patient with T-cell deficiency.
Orbit (Amsterdam, Netherlands)Fibrin-Associated Large B-Cell Lymphoma of Prosthetic Aortic Valve and Aortic Root Graft.
Hematology reportsDiagnostic challenges in Burkitt's lymphoma with skull base and paravertebral involvement, and hearing loss: a case report.
Journal of medical case reportsEmergence of the novel PA-D27G mutation conferring reduced baloxavir susceptibility in influenza A viruses circulating in China, 2018-2025.
Emerging microbes & infectionsSTATH Downregulation and Poor Prognosis in Head and Neck Squamous Cell Carcinoma: Transcriptomic Analysis Reveals Poor Impact on Oral Health.
CureusDisproportionality analysis of drug-associated progressive multifocal leukoencephalopathy: roles of underlying diseases and immunomodulatory therapies in FAERS.
Frontiers in immunologyTrends in Incidence and Survival of Patients With Primary Effusion Lymphoma in the United States: A Population Based Cohort Study.
Hematological oncologyTonsillar T-cell lymphoma with hepatic and renal metastases in a fattening domestic pig (Sus scrofa domesticus).
BMC veterinary research[Progress in Immunotherapy for Pulmonary Lymphoepithelioma Carcinoma].
Zhongguo fei ai za zhi = Chinese journal of lung cancerOutcomes of patients with HTLV-1 adult T-cell leukemia/lymphoma (ATLL) after allogeneic SCT: results of the EBMT LWP.
Bone marrow transplantationYuxingcao formula suppresses acute erythroleukemia through inhibition of AKT1 and FLI1.
FitoterapiaUnexpectedly low prevalence of hepatitis delta virus infection in Southern Viet Nam.
Scientific reportsLymphoepithelial Carcinoma of the Salivary Gland: A Systematic Review and Single Institution Experience.
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck SurgerySinonasal Lymphoepithelial Carcinoma With Insulinoma-Associated Protein 1 (INSM1) Expression: A Potential Pitfall.
International journal of surgical pathologyEpstein-Barr virus+ inflammatory follicular dendritic cell sarcoma with clonal immunoglobulin heavy chain gene rearrangement: A case report and literature review.
Oncology lettersSynaptotagmin-7 drives stress-induced cardiomyocyte necroptosis via the p53-Bak-mPTP axis.
TheranosticsPrediction of immunotherapy efficacy for pulmonary lymphoepithelioma-like carcinoma using baseline routine blood tests and serum tumor markers: a multicenter retrospective study.
Therapeutic advances in medical oncologyInvestigating Renal Papillary Adenoma: Identifying the Associated Conditions Through the Vigilant Lens of the Pathologist!
Indian journal of surgical oncologyCytomegalovirus retinitis during upadacitinib monotherapy for rheumatoid arthritis: a case report.
Journal of rheumatic diseasesPrevalence and time trends of human papillomavirus in penile cancer over 26 years-A comprehensive study of 1040 penile cancer cases from Denmark.
International journal of cancerDismal Outcome of EBV-Positive Nodal T/NK-Cell Lymphoma: A Multicenter Retrospective Study.
Clinical lymphoma, myeloma & leukemiaGene therapy for Krabbe disease: evidence from mouse and canine models.
GeneClassic Hodgkin lymphoma in a patient with Behçet's disease: a case report and review of the literature.
Clinical rheumatologyGenomic surveillance of HBV and HDV reveals genotype-specific risk of liver disease in central Vietnam.
Scientific reportsHepatitis B-associated cirrhosis combined with multiple nodular fatty infiltrates of the liver: A rare benign disease misdiagnosed as hepatocellular carcinoma - a case report.
MedicineLong-term survival with PD-1/PD-L1 inhibitors plus platinum-based chemotherapy versus chemotherapy alone in locally advanced or metastatic pulmonary lymphoepithelioma-like carcinoma: updated follow-up analysis.
Lung cancer (Amsterdam, Netherlands)A real-world safety analysis of infection-related adverse events associated with belimumab, rituximab, and TNF inhibitors using the FAERS database.
Scientific reportsQuantitative spatial profiling of PD-1/PD-L1 and TIGIT/CD155 interaction indicates poor survival outcome and resistance to adjuvant chemotherapy in pancreatic adenosquamous carcinoma.
European journal of cancer (Oxford, England : 1990)Metastatic Epstein-Barr Virus-Associated Lymphoepithelioma-Like Carcinoma of Small Bowel Origin.
Case reports in oncologyMultiple brain abscesses caused by Nocardia asiatica co-infection with Torque teno virus in an "immunocompetent" patient: a rare case report and literature review.
Frontiers in medicineHepatic Epstein-Barr Virus-Associated Smooth Muscle Tumor in a Young Woman With Congenital HIV.
CureusAn Autopsy Case of CD4-Positive Lymphoproliferative Disorder at 38 Years Post-Transplantation Presenting With Cardiac Invasion and Cerebral Infarctions.
CureusIsolated palatine tonsillar metastasis from urinary bladder tumor sparing lymph nodes: a case report.
Journal of surgical case reportsManagement and natural course of CIN 1 and CIN 2 before and after implementation of the revised cervical cancer screening in Germany.
Archives of gynecology and obstetricsA case of KSHV/HHV8-positive large B-cell lymphoma in a background of HIV-negative KSHV/HHV8-positive Multicentric Castleman disease.
Journal of infection and chemotherapy : official journal of the Japan Society of ChemotherapyEpstein-Barr virus in thyroid disease: an integrated immunovirological perspective.
Frontiers in immunologyHuman immunodeficiency virus negative, immunocompetent primary effusion lymphoma with a complete response on R-miniCHOP.
CytoJournalChromatin remodeling enhances MAP3K8 expression in HAM: a key pathogenesis for therapeutic intervention.
Nature communicationsRecent advances in infections as risk factors for lymphoma: a review of the effects of viral, bacterial, and fungal infections in lymphoma.
Archives of microbiologyMetastasis of nasopharyngeal carcinoma to the spleen: a case report.
AME case reportsA Rare Diagnosis of HPV-Positive Rectal Squamous Cell Carcinoma in a 59-Year-Old Heterosexual Woman: Implications for Screening Guidelines.
The American journal of case reportsExtranodal Epstein-Barr Virus-Positive NK/T Non-Hodgkin Lymphoma With Cutaneous Involvement: A Case Report.
Pediatric dermatology[Mixed type II cryoglobulinemiaassociated glomerulonephritis revealing an extragastric marginal zone lymphoma in the absence of viral infection: A rare case report from Bourges Hospital].
Nephrologie & therapeutiqueAir leakage is a strong risk factor for pneumonia-related acute respiratory distress syndrome.
Journal of thoracic diseasePrimary Cutaneous B-Cell Lymphomas: An Updated Portrait of Classification, Biology, and Clinical Management.
European journal of haematologyEpstein-Barr Virus-Associated Early Gastric Carcinoma with Lymphoid Stroma Mimicking a Submucosal Tumor: A Typical Case Diagnosed by Endoscopic Resection and Treated by Local Resection with Sentinel Node Navigation.
Acta medica OkayamaEpstein-Barr Virus-Associated Smooth Muscle Tumors in inborn Errors of Immunity: A single-center Case Series and Literature Overview.
Journal of clinical immunologyA review of cutaneous manifestations of HTLV-associated adult T-cell leukemia/lymphoma.
Frontiers in medicineHistopathological variants of head and neck squamous cell carcinomas: A multicenter study in Latin America.
Annals of diagnostic pathologySpontaneous and transitory remission of follicular lymphoma in patient with SARS-COV-2.
Revista de la Facultad de Ciencias Medicas (Cordoba, Argentina)Oral Plasmablastic Lymphoma: Case Series and Literature Review.
Head & neckPrimary intracranial plasmablastic lymphoma with intradural extramedullary metastasis: a case report.
BMC neurologyThe pathogenic role and genomic characteristics of Epstein-Barr virus in vitreoretinal lymphoma.
Virologica SinicaThe global, regional, and national burden of cancer, 1990-2023, with forecasts to 2050: a systematic analysis for the Global Burden of Disease Study 2023.
Lancet (London, England)Polymorphism of BoLA-DRB3 in Semen and Its Influence on Progeny Derived from Semen with Resistance and Susceptibility to Bovine Leukemia Virus Proviral Load.
Pathogens (Basel, Switzerland)Novel Therapeutic Development for Nasopharyngeal Carcinoma.
Current oncology (Toronto, Ont.)Adult T-cell leukemia/lymphoma in French Guiana (1990-2019): Epidemiology, clinical features, and HTLV-1 genetic diversity in the two main ethnic populations.
International journal of cancerPediatric Liver Transplant Complications: EBV-Associated Tumors and Infection Management Strategies.
The American journal of case reportsEBV-associated smooth muscle tumour: a clinicopathological and genetic study of nine cases revealing heterogeneous immune statuses and novel pathogenic mutations.
HistopathologyAdverse effects of Janus kinase inhibitors with relevance for daily practice in dermatology.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGLymphangiogenesis driven by VEGF-C reshapes tumor immune landscape and enables tumor eradication by viral immunotherapy.
bioRxiv : the preprint server for biologyAnti-interferon-γ autoantibodies syndrome and opportunistic infections: systematic literature review.
Frontiers in immunologyCase report: Recipient-derived, late-onset post-transplant lymphoproliferative disorder involving a transplanted liver.
Transplant immunologyInborn Errors of Immunity in Pediatric Hematology and Oncology: Diagnostic Principles for Clinical Practice.
Journal of clinical medicineSquamous Cell Carcinoma In Situ (SCCIS) of the Nipple Following Breast Conserving Therapy: Case Report.
Hawai'i journal of health & social welfareHPV-associated squamous cell carcinoma in situ arising from urethral caruncle: A case of the unusual occurrence in the common urogenital lesion.
International journal of surgery case reports[Monomorphic epitheliotropic intestinal T-cell lymphoma: a clinicopathological and genetic mutation characteristics analysis of forty-two cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyMethotrexate-Associated Primary Cutaneous Epstein-Barr Virus-Positive Polymorphic B-Cell Lymphoproliferative Disorder With Metachronous T-Cell Clonally Related Angioimmunoblastic T-Cell Lymphoma: A Case Report and Review of the Literature.
The American Journal of dermatopathologyCD20-Negative Large B-Cell Lymphomas: The Diagnostic Challenge of Tumors with Downregulation of Mature B-Cell Marker Expression.
International journal of molecular sciencesCaspase cleavage of Kaposi sarcoma-associated herpesvirus proteins: a role for K5 in preventing caspase-mediated cell death during lytic replication.
Journal of virologyFollicular Helper T-Cell Lymphomas With Epstein-Barr Virus-Positive Neoplastic Cells: A Rare Scenario With Diagnostic Implications.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncLocalized Stage Plasmablastic Lymphoma with Ulcerative Colitis in Long-term Remission after Chemoradiotherapy.
Internal medicine (Tokyo, Japan)Coexistence of human pegivirus infection and poor hematopoietic reconstruction in a lymphoma patient after autologous hematopoietic stem cell transplantation: A case report and literature review.
MedicineCase Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge.
Frontiers in oncologyCase Report: Long-term response control in a patient with metastatic gastric squamous cell carcinoma treated with nivolumab and chemoradiotherapy.
Frontiers in immunologyClinical and functional characterization of a novel TNFRSF9 variant causing immune dysregulation with predisposition to EBV-driven lymphomagenesis.
Frontiers in immunologyPure red cell aplasia due to Parvovirus B19 infection and atezolizumab: case report and literature review.
Immunotherapy[Thymic Lymphoepithelial Carcinoma:Report of a Case].
Kyobu geka. The Japanese journal of thoracic surgeryEBV-positive inflammatory follicular dendritic cell sarcoma occurring in different organs: a case report and literature review.
Frontiers in oncologyInduction chemoradiotherapy achieves long-term recurrence-free survival in locally advanced pulmonary lymphoepithelioma-like carcinoma: a case report and literature review.
Frontiers in immunologyCase Report: Inherited chromosomally integrated HHV-6B in a pediatric medulloblastoma patient with encephalitis.
Frontiers in immunologyPrimary Immunodeficiencies and EBV-Associated Diseases.
Current topics in microbiology and immunologyMedicinal Products Based on Adeno-Associated Viral Vectors: A Regulatory Perspective on the Potential Risk of Insertion-Mediated Tumorigenesis.
Human gene therapyGenomic and Transcriptomic Landscape of Epstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma: A Multicenter Study.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncRare Peritoneal Involvement in Adult T-Cell Leukemia/Lymphoma: A Diagnostic Conundrum.
CureusEpstein-Barr virus-associated intrahepatic cholangiocarcinoma: a diverse morphological spectrum that correlates with patient prognosis.
PathologyBuschke-Löwenstein Tumor: An Unusual Therapeutic Approach.
GE Portuguese journal of gastroenterologyPET/CT Detection of Epstein-Barr Virus-Positive Lacrimal Sac Carcinoma in Unknown Primary Cervical Nodal Metastasis.
Clinical nuclear medicineAn adeno-associated virus gene therapy strategy for anti-obesity treatment by nanocarrier-based delivery systems.
Journal of nanobiotechnologyOther iatrogenic immunodeficiency-associated lymphoproliferative disorders after multidrug immunosuppressive therapy for anti-melanoma differentiation association gene 5 antibody-positive dermatomyositis: a case report.
Modern rheumatology case reportsLymphoepithelioma-Like Carcinoma of the Urinary Bladder: A Case Report.
CureusReticular and Myxoid Variant of EBV-positive Nasopharyngeal Carcinoma Mimicking Salivary Gland Tumors: A Case Series.
Head and neck pathologyThe Genetic Landscape of Acute Necrotizing Encephalopathy: Insights Into the Possible Pathogenesis.
Journal of clinical neurology (Seoul, Korea)Management of Patients With AIDS and Retroperitoneal Calcified Fibrosis During the Perioperative Period: A Case Report.
Journal of perianesthesia nursing : official journal of the American Society of PeriAnesthesia NursesSublingual Dermoid Cyst Mimicking Ranula on Imaging: Surgical Treatment and Outcome in a Human Immunodeficiency Virus (HIV)-Positive Patient.
CureusCrescentic glomerulonephritis associated with NK-large granular lymphocytic leukemia: A case report.
MedicineDifferentiation of Human Neurons and Neuron-Like Cells to Study VZV Neuronal Infection.
Methods in molecular biology (Clifton, N.J.)A case of acute myeloid leukemia with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis successfully treated by cord blood transplantation.
Journal of infection and chemotherapy : official journal of the Japan Society of ChemotherapyPolyomavirus Associated Trichodysplasia Spinulosa in a Pediatric Kidney Transplant Recipient.
Pediatric transplantationA Case of Life-Threatening Airway Obstruction Due to Epstein-Barr Virus-Associated Smooth Muscle Tumor With HIV Infection.
Open forum infectious diseasesBrentuximab vedotin monotherapy for CD30-positive adult T-cell leukemia/lymphoma with HTLV-1-infected Hodgkin and Reed-Sternberg-like cells.
Journal of clinical and experimental hematopathology : JCEHPlasmablastic Lymphoma-A Crosstalk Between Myeloma and Lymphoma.
Clinical lymphoma, myeloma & leukemiaEfficacy and Safety of Fuzheng Jiedu Xiaoji Formula Combined with Conventional Western Therapy in Advanced HBV-HCC: A Single-Center, Randomized Controlled Trial.
Chinese journal of integrative medicineAn HTLV-1 carrier with sporadic late-onset nemaline myopathy accompanied by skin lesions indicating indolent adult T-cell leukemia/lymphoma and Sjögren's syndrome: a case report and literature review.
Neuromuscular disorders : NMDSquamous cell carcinoma of unknown primary (SCCUP): a genomic landscape study.
ESMO openExtramedullary plasmacytoma in children is a genetically distinct localized neoplasia curable by surgical resection.
Blood advancesOvarian cancer risk and survival according to tumor sex hormone receptor expression: An ovarian Cancer association consortium and ovarian tumor tissue analysis consortium pooled analysis.
Gynecologic oncologyEfficacy and prognosis of allogeneic hematopoietic stem cell transplantation in aggressive NK-cell leukemia: a meta-analysis.
Annals of hematologyCase Report: Anti-NMDAR encephalitis associated with neurobrucellosis: causality or coexistence?
Frontiers in immunologyMerkel Cell Polyomavirus (MCPyV) and Its Possible Role in Head and Neck Cancers.
BiomedicinesA Critical Role for Epstein-Barr Virus in Primary Effusion Lymphoma.
Current topics in microbiology and immunologyFDA Approval Summary: Afamitresgene Autoleucel for Adults with HLA-Restricted, MAGE-A4-Positive Unresectable or Metastatic Synovial Sarcoma after Prior Chemotherapy.
Clinical cancer research : an official journal of the American Association for Cancer ResearchThe relationship between multiple plasma biomarker levels and renal disease activity in Fabry disease.
BMC nephrologyInflammatory myopathy and myocarditis are relevant complications of natural feline immunodeficiency virus infection.
Veterinary pathologyHPV42: A Common Low-Risk HPV Type Associated With Distinctive Cervicovaginal and Cutaneous Neoplasia.
The American journal of surgical pathologyExpression variation of Akt and cytokines in lamivudine-resistant HBV rare mutation that prematurely truncates the overlapping surface protein.
Human immunologyPrimary Extranodal Natural Killer/T-cell Lymphoma of the Bladder on 18 F-FDG PET/CT.
Clinical nuclear medicineEpstein-Barr virus-positive small cell neuroendocrine carcinoma of the nasopharynx: A clinicopathologic study of 7 cases and literature review.
Human pathologyPresentation of extranodal NK/T-cell lymphoma as a pituitary mass: A case report and review of the literature.
Surgical neurology internationalDistinct characteristics and social determinants in adult T-cell leukaemia/lymphoma patients at a tertiary cancer centre in Canada.
British journal of haematologyVirus-induced RGMa expression drives neurodegeneration in HTLV-1-associated myelopathy.
JCI insightIntegration of anti-PD-1 antibody into chemotherapeutic regimens improved the outcome of aggressive NK cell leukemia: a single-center retrospective real-world analysis.
Frontiers in immunologyDiagnosis and treatment of pulmonary lymphoepithelioma-like carcinoma: A case report.
World journal of clinical oncologyDisseminated talaromycosis in HIV-negative patients with lung cancer: a rare case report and literature review.
BMC infectious diseasesKaposi sarcoma herpesvirus (KSHV) inflammatory cytokine syndrome (KICS): a case study.
BMC infectious diseasesPrimary Gastrointestinal EBV-Associated Classical Hodgkin Lymphoma in Crohn Disease on Anti-TNF-α Therapy: A Rare Association.
JGH open : an open access journal of gastroenterology and hepatologyConcomitant Splenic Tuberculosis and Epstein-Barr Virus-Related T-Cell Leukemia/Lymphoma in a 28-Year-Old Pregnant Woman in South Sudan.
The American journal of tropical medicine and hygieneUnderstanding Merkel Cell Carcinoma: Pathogenic Signaling, Extracellular Matrix Dynamics, and Novel Treatment Approaches.
CancersFibrin-associated large B-cell lymphoma arising in a cystic lymphangiomatous lesion of the adrenal gland: A case report and overview of the entity.
Pathology, research and practiceSustained circulation of enterovirus D68 in Europe in 2023 and the continued evolution of enterovirus D68 B3-lineages associated with distinct amino acid substitutions in VP1 protein.
Journal of clinical virology : the official publication of the Pan American Society for Clinical VirologyPlasmablastic lymphoma with cutaneous manifestations in an immunocompetent man: A rare case report with literature review.
Journal of cancer research and therapeuticsComprehensive review of morphological diagnosis of adult T-cell leukemia-lymphoma.
Expert review of hematologyFrequent association of malignant effusions in plasmablastic lymphoma: a single-institutional experience of nine cases in Taiwan.
Blood researchOral Neisseria gonorrhoeae Promotes KSHV Lytic Replication.
Journal of medical virologyBurkitt lymphoma in patients with chronic lymphocytic leukemia and other indolent B-cell lymphoid malignancies.
Leukemia & lymphomaPosttransplant EBV-Positive Smooth Muscle Tumors in Children, Adolescents, and Young Adults: A Multi-Institution Experience.
Pediatric blood & cancerHIV-negative primary effusion lymphoma: a series of seven cases.
HistopathologyKaposi's Sarcoma, Epidemiology and Clinical Characteristics in a Tertiary Hospital: A Cross-Sectional Study.
Dermatology (Basel, Switzerland)Large B-cell lymphoma associated with fluid overload: two new case reports with molecular insights.
Virchows Archiv : an international journal of pathologyNSD3::FGFR1 : A Novel Gene Fusion First to Be Described in Merkel Cell Carcinoma.
The American Journal of dermatopathologyPrimary effusion lymphoma: therapeutic strategies targeting viral and cellular mechanisms.
Expert review of anticancer therapyEBV-associated leiomyosarcoma in an immunocompromised child: A unique intracranial case with genomic study.
International journal of surgery case reportsClinicopathological analysis of lymphoepithelioma-like intrahepatic cholangiocarcinoma.
Pathology, research and practiceJC polyomavirus-associated nephropathy in a kidney allograft: A case report and literature review.
Transplant immunologyDermatomyositis as the First Manifestation of Nasopharyngeal Carcinoma-A Rare Case Report.
Medicina (Kaunas, Lithuania)Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil.
Frontiers in medicineConcurrent hemophagocytic lymphohistiocytosis and thrombotic microangiopathy in a patient with Epstein-Barr virus infection and gastric adenocarcinoma: the puzzle started with microangiopathic hemolytic anemia.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisEpstein-Barr Virus-Associated Pneumonia in a Healthy Adult Presenting With Pulmonary Nodules and Deep Vein Thrombosis: A Case Report and Literature Review.
Journal of medical virologyPilocytic astrocytoma in a child with spinal muscular atrophy treated with onasemnogene abeparvovec.
Molecular therapy : the journal of the American Society of Gene TherapyMerkel cell carcinoma in Taiwan: a subset is chronic arsenicism-related, and the Merkel cell polyomavirus-negative cases are pathologically distinct from virus-related cases with a poorer outcome.
PathologyAn Unusual Case of Extracavitary/Solid Variant Primary Effusion Lymphoma With Associated Hemophagocytic Lymphohistiocytosis.
Journal of medical casesAge-related immunosenescence and Epstein-Barr virus-positive mucocutaneous ulcer presenting as oral cancer: a case report.
Frontiers in medicinePenile Cancer: Innovations in Ultrastructural and Vibrational Markers.
ACS omegaSurvival and prognostic factors for primary lung extranodal NK/T-cell lymphoma: a retrospective study of data from China and the SEER database.
Frontiers in oncologyEfficacy and safety of immunotherapy in locally advanced or metastatic pulmonary lymphoepithelioma-like carcinoma: a multicenter retrospective study.
Therapeutic advances in medical oncologyClinicopathologic Features and Viral Status of Low-risk HPV6 and HPV11-Associated Squamous Cell Carcinoma of the Uterine Cervix and Vulva.
The American journal of surgical pathologyEpstein-Barr virus-associated smooth muscle tumor with multiple lesions in a patient with AIDS.
AIDS (London, England)[Urinary Salmonella Enteritidis Carriage in a Patient Living with HIV].
Mikrobiyoloji bulteniAngioimmunoblastic T-cell lymphoma: Characterization of clonal T and B cells and a patient-derived xenograft study of coexisting T- and B-cell proliferation.
EJHaemEpstein-Barr virus status drives morphological and molecular intra-tumour heterogeneity in gastric cancer: insights from a case report and literature review.
Virchows Archiv : an international journal of pathologyEssential Thrombocythemia: A Review.
JAMANasopharyngeal carcinoma with unusual metastatic spread to the spine and meninges: a case report with literature review.
Journal of surgical case reportsA case report: spleen Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma.
Journal of gastrointestinal oncologyMmuPV1 infection of Tmc6/Ever1 or Tmc8/Ever2 deficient FVB mice as a model of βHPV in typical epidermodysplasia verruciformis.
PLoS pathogensA Rare Case of Severe Facial Disfiguration Due to Extranodal NK/T-Cell Lymphoma.
Journal of asthma and allergy[Clinicopathological Analysis of 14 Cases of Primary Pulmonary Lymphoepithelial Carcinoma].
Zhongguo fei ai za zhi = Chinese journal of lung cancerFrom Fibrosis to Granuloma: Drug Induced Systemic Sarcoidosis-Like Reaction After Rituximab in a Patient with Primary Sjögren's Syndrome.
European journal of case reports in internal medicineMultifocal Head and Neck Tumor in a Patient With HIV: A Rare Case.
CureusMumps Myelitis-A Rare Cause of Myelitis.
The American journal of tropical medicine and hygieneRare Cutaneous Primary Presentation of Extracavitary Primary Effusion Lymphoma.
The American Journal of dermatopathologyA Unique Case of Metastatic Oral Kaposi Sarcoma in an HIV-Negative Patient.
Ear, nose, & throat journalEpstein-Barr Virus-Associated Smooth Muscle Tumor in the Liver Post Kidney Transplant: A Case Report.
CureusDEK::AFF2 fusion-associated middle ear non-keratinizing squamous cell carcinoma: A case report.
World journal of clinical oncologyAssociações
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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.
- Molecular pathogenesis and evolving treatment landscape of extranodal NK/T-cell lymphoma.Proceedings of the Japan Academy. Series B, Physical and biological sciences· 2026· PMID 41813156mais citado
- Classic hodgkin lymphoma associated with Epstein-Barr virus reactivation during vedolizumab therapy for ulcerative colitis.
- Human and viral whole genome sequencing identify HPV and APOBEC as oncogenic drivers in sinonasal squamous cell carcinoma.
- BK polyomavirus-associated progressive multifocal leukoencephalopathy following mogamulizumab therapy for erythrodermic mycosis fungoides.
- DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.Neuropathology : official journal of the Japanese Society of Neuropathology· 2026· PMID 41874350mais citado
- Urothelial Carcinoma of the Bladder Following BK Virus Infection in a Pediatric Kidney Transplant Recipient.
- [Clinical Characteristics and Prognostic Analysis of Patients with EBV-Positive Diffuse Large B-Cell Lymphoma].
- A 43-Year-Old Man With Isolated Cutaneous Lymphomatoid Granulomatosis Presenting as a Chronic Necrotizing Ulcer of the Upper Arm.
- Recurrent anti-TIF1γ-positive dermatomyositis coexisting with postoperative parotid lymphoepithelial carcinoma: a case report with pathogenesis analysis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:289635(Orphanet)
- MONDO:0017341(MONDO)
- GARD:21145(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q604261(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
