É uma doença rara de causa desconhecida, caracterizada por um inchaço dos espaços internos dos gânglios linfáticos e pelo acúmulo de certas células (chamadas histiócitos) nesses espaços. Esses histiócitos geralmente contêm linfócitos (um tipo de célula de defesa) em seu interior. As pessoas com essa condição podem apresentar inchaço dos gânglios linfáticos no pescoço (ínguas), febre, aumento do número de glóbulos brancos no sangue e aumento de um tipo de proteína no sangue (gama-globulina). A doença pode afetar outras partes do corpo além dos gânglios linfáticos, como a pele, os ossos e as vias respiratórias. Geralmente, a condição desaparece por conta própria.
Introdução
O que você precisa saber de cara
É uma doença rara de causa desconhecida, caracterizada por um inchaço dos espaços internos dos gânglios linfáticos e pelo acúmulo de certas células (chamadas histiócitos) nesses espaços. Esses histiócitos geralmente contêm linfócitos (um tipo de célula de defesa) em seu interior. As pessoas com essa condição podem apresentar inchaço dos gânglios linfáticos no pescoço (ínguas), febre, aumento do número de glóbulos brancos no sangue e aumento de um tipo de proteína no sangue (gama-globulina). A doença pode afetar outras partes do corpo além dos gânglios linfáticos, como a pele, os ossos e as vias respiratórias. Geralmente, a condição desaparece por conta própria.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 32 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 92 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
1 gene identificado com associação a esta condição.
Uniporter that mediates the facilitative transport of nucleoside across lysosomal and mitochondrial membranes (PubMed:15701636, PubMed:19164483, PubMed:20595384, PubMed:28729424). Functions as a non-electrogenic Na(+)-independent transporter (PubMed:15701636, PubMed:19164483, PubMed:28729424). Substrate transport is pH-dependent and enhanced under acidic condition, probably reflecting the location of the transporter in acidic intracellular compartments (PubMed:15701636, PubMed:19164483, PubMed:2
Lysosome membraneLate endosome membraneMitochondrion membraneCell membrane
Histiocytosis-lymphadenopathy plus syndrome
A syndrome characterized by the combination of features from 2 or more of four histiocytic disorders, originally thought to be distinct: Faisalabad histiocytosis (FHC), sinus histiocytosis with massive lymphadenopathy (SHML), H syndrome, and pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome (PHID). FHC features include joint deformities, sensorineural hearing loss, and subsequent development of generalized lymphadenopathy and swellings in the eyelids that contain histiocytes. SHML causes lymph node enlargement in children frequently accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. H syndrome is characterized by cutaneous hyperpigmentation and hypertrichosis, hepatosplenomegaly, heart anomalies, and hypogonadism; hearing loss is found in about half of patients. PHID is characterized by predominantly antibody-negative insulin-dependent diabetes mellitus associated with pigmented hypertrichosis and variable occurrence of other features of H syndrome.
Medicamentos e terapias
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: CRL4(CRBN) E3 ubiquitin ligase modulator
Variantes genéticas (ClinVar)
79 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
3 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 — Histiocitose sinusal com linfadenopatia maciça
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
Mostrando amostra de 200 publicações de um total de 1.054
Isolated Intracranial Rosai-Dorfman Disease Mimicking Sellar Pituitary Adenoma: a case report and literature review.
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is an extremely rare non-Langerhans cell histiocytosis. The diagnostic histiocytes in RDD are characterized by the immunophenotypic markers S100 (+), CD68 (+), and CD1a (-) (Abla O, Blood 131: 2877, 2018),(Emile JF, Blood 127: 2672, 2016) , and exhibit phagocytosis with varying frequency. To our knowledge, this is the first reported case of isolated Intracranial Rosai-Dorfman Disease Mimicking Sellar Pituitary Adenoma, with peripheral facial paralysis as the initial presentation. By integrating imaging findings, histopathological features, and treatment response, we explore the diagnostic challenges and management strategies, aiming to enhance clinicians' awareness of RDD involving the pituitary. We report the case of a 50-year-old Han Chinese female who presented with left-side peripheral facial paralysis and tender enlargement of the left cervical lymph nodes. Pituitary MRI revealed an intrasellar mass with homogeneous enhancement, initially diagnosed as a pituitary adenoma. The patient underwent transnasal transsphenoidal endoscopic resection of the pituitary adenoma. But histopathology and immunohistochemistry (S100+, CD68+, CD1a-) confirmed the diagnosis of RDD. Postoperatively, the facial paralysis improved, Unfortunately, 6 months follow-up MRI showed tumor recurrence. The clinical manifestations of intrasellar RDD are insidious and lack specificity, making it easy to confuse with common pituitary space-occupying lesions. Currently, surgical resection remains the gold standard for definitive diagnosis and is the primary therapeutic intervention, long-term follow-up is crucial for monitoring recurrence.
Spine-limited Rosai-Dorfman disease in a patient with ankylosing spondylitis - A rare mimicker of spinal tumors.
Rosai-Dorfman disease (RDD) is an uncommon histiocytic proliferative disorder that typically involves the lymph nodes but can also present with extranodal involvement. Central nervous system involvement is rare, and the spine-restricted RDD is an extremely rare variant that poses significant diagnostic challenges, often resulting in delays in diagnosis and management. We report a case of a 32-year-old gentleman with a history of back pain for 1-year, initially being managed as ankylosing spondylitis, which subsequently developed spastic paraparesis and was ultimately diagnosed with spine-restricted RDD. Recognizing this rare entity and its potential associations with immune-mediated conditions, such as human leukocyte antigen B27-related spondyloarthropathies and possibly inflammatory bowel disease, is essential to avoid misdiagnosis and guide individualized, multidisciplinary treatment strategies.
Rosai-Dorfman disease mimicking testicular malignancy in a patient with marginal zone lymphoma of the lung.
Rosai-Dorfman-Destombes disease (RDD) is a rare, benign histiocytic disorder, most often involving lymph nodes but showing extranodal manifestations in nearly 50% of cases, particularly in the skin and upper respiratory tract. A hallmark histological feature is emperipolesis-large CD68+ and S100+ histiocytes containing intact inflammatory cells. We report a unique case of bilateral testicular RDD in a man with prior extranodal marginal zone lymphoma of the lungs. Three years after lymphoma remission, he presented with bilateral testicular enlargement. Ultrasound and positron emission tomography/CT showed diffuse hypoechoic parenchyma with metabolic activity suggestive of malignancy. Bilateral orchiectomy revealed tan-yellow, fleshy masses infiltrating the testicular parenchyma. Histology confirmed RDD with emperipolesis and S100+/CD68+/CD1a- phenotype. Testicular RDD is extremely uncommon, with only ~15 cases described to date, and its association with haematological malignancy remains poorly understood. Recognition of this entity is essential to avoid unnecessary oncological treatment.
Spinal Rosai-Dorfman disease: systematic review and meta-analysis of clinical presentation, diagnosis, and treatment.
Spinal Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can mimic neoplastic and infectious processes, complicating diagnosis and management. Its natural history and optimal treatment strategies remain poorly defined. To systematically review and perform an individual patient data (IPD) meta-analysis of clinical presentation, diagnostic features, management, and outcomes in adult patients with spinal RDD. A PRISMA-compliant systematic review was performed using PubMed, Scopus, and Cochrane Library databases. Eligible studies included case reports, case series, and observational studies reporting histologically confirmed spinal RDD in adults (≥18 years). Patient-level data were pooled and analyzed using a one-stage mixed-effects model to identify factors associated with full neurological recovery. Recurrence-free survival (RFS) was assessed using Kaplan-Meier methodology. Sixty-seven studies reporting 85 patients (mean age 42.6 years; 70.6 % male) were included. Thoracic (61.2 %) and cervical (43.5 %) lesions predominated, with extradural (40 %), intradural-extramedullary (24.7 %), and intramedullary (31.8 %) involvement. Surgical resection was performed in 83.5 % of cases; gross total resection (GTR) was achieved in 45.9 %. At a mean follow-up of 19.9 months, 40 % of patients achieved full neurological recovery and 7.1 % experienced recurrence, most within one month postoperatively. Intradural-extramedullary location (RR 4.20, p = 0.008) and GTR (RR 4.31, p = 0.002) independently predicted full recovery, while intramedullary involvement was associated with worse outcomes (RR 0.29, p = 0.019). Intradural-extramedullary location and GTR strongly predict favorable outcomes in spinal RDD, whereas intramedullary disease confers a poorer prognosis. The high early recurrence rate underscores the need for close postoperative surveillance and consideration of adjuvant therapy in high-risk cases. Standardized surgical protocols and prospective IPD studies are needed to refine management and improve long-term outcomes.
Rosai-Dorfman Disease Primarily Involved in the CNS.
Publicações recentes
Cutaneous Rosai-Dorfman Disease Misdiagnosed as Granulomatous Dermatitis: A Diagnostic Pitfall.
Hodgkinoid histiocytosis, a distinctive histiocytic/dendritic cell disorder clinically mimicking drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms.
Rosai-Dorfman Disease in Nasal Cavity and Paranasal Sinuses: Imaging Features in a Series of 23 Patients.
Multifocal Rosai Dorfman disease and simultaneous endometrioid ovarian cancer: a case report.
Rosai-Dorfman disease with thyroid involvement: a case report and literature review.
📚 EuropePMC1.583 artigos no totalmostrando 198
Extranodal Rosai-Dorfman Disease Presenting as a Pancreatic Mass Associated With Superior Mesenteric Artery Thrombosis.
ACG case reports journalCiliochoroidal and Conjunctival Rosai-Dorfman Disease.
OphthalmologyFacial Cutaneous Rosai-Dorfman Disease Misdiagnosed as Sporotrichosis: A Case Report and Literature Review.
Clinical, cosmetic and investigational dermatologyAn intriguing case of central airway obstruction: a diagnostic challenge.
Breathe (Sheffield, England)Cutaneous-to-Bronchial Rosai-Dorfman Disease without Lymphadenopathy: A Rare Case Report and Literature Review.
Clinical, cosmetic and investigational dermatologyExtranodal Rosai-Dorfman Disease of the Male Breast: A Case Report and Literature Review.
The American Journal of dermatopathology[Rosai-Dorfman disease of the breast: a clinicopathological and molecular genetic analysis of four cases].
Zhonghua bing li xue za zhi = Chinese journal of pathologyRosai-Dorfman Disease Primarily Involved in the CNS.
NeurologyCutaneous Rosai-Dorfman disease: a case report of disease limited only to skin.
Dermatology reportsFine-Needle Aspiration as a Key Diagnostic Tool in Pediatric Rosai-Dorfman Disease: A Case Report of a 12-Year-Old Male.
International medical case reports journalIsolated Intracranial Rosai-Dorfman Disease Mimicking Sellar Pituitary Adenoma: a case report and literature review.
BMC neurologyOrigin, diagnosis and treatment of periocular Histiocytic disorders- state-of-the-art review.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle OphthalmologieA Rare Lymphoproliferative Disorder in a Teenager with Spontaneous Resolution on Positron Emission Tomography Scan.
Euroasian journal of hepato-gastroenterologyCD71 expression in Rosai-Dorfman disease: a useful adjunct marker in the differential diagnosis of histiocytic proliferations.
HistopathologyOphthalmic Rosai-Dorfman Disease and the Revised Classification of Histiocytoses: A Case Series and Review of the Literature.
Ophthalmic plastic and reconstructive surgeryAnti-SAE Antibody-Positive Dermatomyositis Presenting With Widespread Erythema Complicated by Rosai-Dorfman Disease.
The Journal of dermatologySpine-limited Rosai-Dorfman disease in a patient with ankylosing spondylitis - A rare mimicker of spinal tumors.
Surgical neurology internationalIsolated Cervical Lymphadenopathy in Pediatric Rosai-Dorfman Disease: A Case Report and Literature Review.
CureusAn unusual cause of angioedema: Rosai-Dorfman disease.
Postepy dermatologii i alergologiiAnesthesia management for surgical excision in patients with Rosai-Dorfman disease involving multiple organs throughout the body: a case report and literature review.
BMC anesthesiologyHistiocytic Sarcoma Arising From Pediatric Rosai Dorfman Disease: Two Novel Cases and Literature Review.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyThe Value of High-Frequency Ultrasound in the Evaluation of Cutaneous Rosai-Dorfman Disease: A Case Series and Literature Review.
Diagnostics (Basel, Switzerland)Destombes - The Forgotten Pathologist: Rosai-Dorfman Disease, From Initial Recognition to the Latest and Greatest.
Archives of pathology & laboratory medicineClinicopathologic features of localized ALK-positive histiocytosis: A study of three cases and review of literature.
Indian journal of pathology & microbiologyEducational Case: Rosai-Dorfman disease mimicking metastatic carcinoma.
Academic pathologyRosai-Dorfman disease mimicking testicular malignancy in a patient with marginal zone lymphoma of the lung.
BMJ case reportsThe use of MEK inhibitors in pediatric patients presenting with respiratory distress and laryngeal Rosai-Dorfman Disease: Two case reports.
International journal of pediatric otorhinolaryngologyA Rare Case of Extranodal Rosai-Dorfman Disease Involving the Entire Colon.
CureusClinicopathological insights into ophthalmic Rosai-Dorfman disease: A 15-year retrospective analysis.
Indian journal of ophthalmologyCase Report: Improvement from no light perception after radiotherapy and surgical debulking for orbital Rosai-Dorfman disease.
Frontiers in ophthalmologyEn bloc resection for extra-nodal Rosai-Dorfman disease in the anterior mediastinum: a case report.
BMC surgeryExtranodal Rosai-Dorfman disease in a male involving the breast and subcutaneous soft tissue: An uncommon distribution.
Radiology case reportsPostoperative Radiotherapy in Spinal Rosai-Dorfman Disease: A Case Report and Literature Review.
Case reports in oncologyFDG PET/CT in a Case of Primary Multifocal Intraparenchymal Rosai-Dorfman Disease of the Brain.
Clinical nuclear medicinePrednisone and Vincristine for the Treatment of Pediatric Rosai-Dorfman Disease: A Case Report.
Case reports in medicineExtranodal Rosai-Dorfman Disease With Increased IgG4-Positive Plasma Cells Involving the Breast: A Case Report With Review of the Literature.
The American Journal of dermatopathologyEmperipolesis, not hemophagocytosis: chronic myelomonocytic leukemia with neoplasia-associated Rosai-Dorfman disease.
International journal of hematologyRosai-Dorfman Disease Involving Thymus and Aorta Revealed by 18 F-FDG PET/CT.
Clinical nuclear medicinePrimary Rosai-Dorfman disease of lung and mediastinum: A clinicopathological and immunohistochemical study of five cases.
Pathology, research and practiceSpinal Rosai-Dorfman disease: systematic review and meta-analysis of clinical presentation, diagnosis, and treatment.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaRosai Dorfman Disease: A Myriad of Presentations-Multisystem Involvement Including Bone Marrow.
European journal of case reports in internal medicineA Red Lobulated Facial Mass: A Clinicopathological Challenge.
International journal of dermatologyRare Rosai-Dorfman disease presenting with basal ganglia and bilateral cerebral peduncles-diagnosis and challenges.
Quantitative imaging in medicine and surgeryRare Subcutaneous Manifestation of Rosai-Dorfman Disease on FDG PET/CT in an Adolescent.
Clinical nuclear medicineIntracranial manifestations of adult Rosai-Dorfman disease: a systematic review and IPD meta-analysis of 327 cases.
Acta neurochirurgicaAdvances in the diagnosis and management of pediatric Langerhans cell histiocytosis and Rosai-Dorfman disease: therapies, biomarkers, and response assessment.
Hematology. American Society of Hematology. Education ProgramExtensive Intra-abdominal Rosai-Dorfman Disease in an Elderly Patient.
Radiology. Imaging cancerRosai-Dorfman Disease, Presenting as a Mass in the Trachea: A Case Report.
Respirology case reportsCharacteristics of histiocytic neoplasms presenting as breast masses.
British journal of haematologyVEXAS syndrome with eosinophilia and pathologically mimicking histiocytosis: a case report.
Modern rheumatology case reportsPeriorbital Rosai-Dorfman disease: first UK case series.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologieExtranodal Rosai-Dorfman disease with ocular involvement in 5 cases with a literature review.
International journal of ophthalmologyGeneralized Eruptive Histiocytosis: A Case Report in an Elderly Person With a Positive Rheumatoid Factor.
CureusIgG4 plasma cells in hematopathology: beyond IgG4-related disease.
Journal of hematopathologyReal-world outcomes in adult histiocytosis: the uncharted territory of orphan diseases.
Leukemia & lymphomaCorrigendum to "A rare presentation of Rosai-Dorfman disease as a single dural-based lumbar intradural lesion: A case report" [Heliyon Volume 10, Issue 7, APRIL 15 2024, Article e28542].
HeliyonRosai-Dorfman disease: an infrequent entity with heterogeneous presentation. Case series of six patients.
Anais brasileiros de dermatologiaRosai-Dorfman disease presenting without systemic symptoms in childhood.
BMJ case reportsSpectrum of Cytological Findings in Idiopathic Granulomatous Mastitis With Clinical and Radiological Correlation: A Case Series From a North Indian Tertiary Center.
Diagnostic cytopathologyHistiocyte Society blueprint for non-Langerhans cell histiocytosis research: unraveling complex diseases through collaboration.
HaematologicaRosai-Dorfman disease mimicking lung cancer: a case report.
Frontiers in oncologyIntraoperative Frozen Section Diagnosis of Brain Rosai-Dorfman Disease Clinically and Radiologically Mimicking Meningioma.
In vivo (Athens, Greece)Rosai-Dorfman Disease of the Breast: Radiologic-Pathologic Correlation.
Journal of breast imagingSinus and palatal Rosai-Dorfman Disease: Case report and review of the literature.
American journal of otolaryngologySurgical Treatment for a 10-Year-Old Children With Recurrent Wheezing Due to Tracheal Rosai-Dorfman Disease.
Pediatric pulmonologySpontaneous Regression of Rosai-Dorfman Disease Presenting as a Thigh Mass With Vascular Involvement: A Case Report.
Journal of investigative medicine high impact case reportsRosai-Dorfman Disease: A Rare Disease with Uncommon 18F-FDG PET CT Findings.
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaCutaneous Rosai-Dorfman Disease After COVID-19 Vaccination: Rare Occurrence with Challenging Presentation.
Seminars in musculoskeletal radiologyIntrathoracic Rosai-Dorfman Disease in Elderly Patients: A Case Report.
Clinical case reportsA Case of Extra-nodal Rosai-Dorfman Disease.
CureusLangerhans cell histiocytosis with mesenteric involvement successfully treated with trametinib: a rare cause of small intestine obstruction.
International journal of hematologyCutaneous Rosai-Dorfman disease with near-complete response to the MEK inhibitor cobimetinib.
JAAD case reportsRosai-Dorfman disease mimicking IgG4-related disease.
Wiener medizinische Wochenschrift (1946)Radiation Therapy for Primary Cutaneous Rosai-Dorfman Disease of the Face: A Case Report Resistant to Multiple Lines of Treatment and a Literature Review.
CureusCutaneous Rosai-Dorfman Disease Mimicking Granulomatous Dermatoses: A Rare Case Report.
Pediatric dermatologyExtranodal Rosai-Dorfman Disease in a Pediatric Patient: A Case Report.
The American journal of case reportsUtility of cardiac magnetic resonance imaging in the evaluation of cardiovascular involvement in pediatric Rosai-Dorfman disease.
Pediatric radiologyExtranodal Rosai-Dorfman Disease of the Breast Presenting as Inflammatory Breast Cancer.
Balkan medical journalRosai-Dorfman Disease With Novel BRAF Fusion Involving the Central Nervous System.
International journal of surgical pathologyThymic Rosai-Dorfman Disease: A Rare Presentation With Unusual Histopathologic Features.
International journal of surgical pathologyThe relevance of clinical-pathological correlation in the diagnosis of cutaneous Rosai-Dorfman disease.
Anais brasileiros de dermatologiaUncovering hidden immune defects in childhood granulomatous disorders: a case report.
Frontiers in immunologyCNS Manifestations of Rosai-Dorfman Disease.
JPMA. The Journal of the Pakistan Medical AssociationSinonasal presentations of Rosai-Dorfman disease: a scoping review.
The Journal of laryngology and otologyIsolated osseous Rosai-Dorfman disease: a case report and review of literature.
Skeletal radiologyPrimary Cutaneous Rosai-Dorfman Disease: A Cyto-Histo Correlate.
Cytopathology : official journal of the British Society for Clinical CytologyA linear presentation of cutaneous Rosai Dorfman disease.
JAAD case reportsRosai-Dorfman Disease Case Report.
Case reports in dermatologyRosai-Dorfman Disease Presenting as a Bone-Destructive Mass in the Maxillary Sinus With Orbital Extension: A Diagnostic and Therapeutic Challenge.
CureusSurgical Treatment of Intracranial Rosai-Dorfman Disease.
The Journal of craniofacial surgeryA rare case of Rosai-Dorfman disease mimicking a malignant lymphoproliferative process and IgG4-related disease.
Contemporary oncology (Poznan, Poland)Rosai-Dorfman Disease: Meningioma Mimics.
Mayo Clinic proceedingsRosai-Dorfman disease presenting as a scleral nodule in a female with multisystem inflammatory syndrome post-COVID-19 infection.
Taiwan journal of ophthalmologyLeft shoulder pain from humeral Rosai-Dorfman disease.
JAAPA : official journal of the American Academy of Physician AssistantsCutaneous Rosai-Dorfman Disease: A Report of 2 Cases and a Review of Recent Literature (2018-2023).
Case reports in dermatologyAnaesthesia for proximal femoral nailing in a patient with "Rosai Dorfman disease" with prior laryngotracheal reconstruction - Not all rosy as it seems.
Trauma case reportsRosai-Dorfman Disease: The Role of Radiation Therapy.
Practical radiation oncologyRosai-Dorfman Disease Preceding Acute Myeloid Leukemia: An Extremely Rare Case and A Literature Review.
European journal of case reports in internal medicineSuccessful treatment of Rosai-Dorfman disease in the era of targeted therapy.
Internal medicine journalReal-world experience with targeted therapy in patients with histiocytic neoplasms in the Netherlands and in Belgium.
Blood neoplasiaTreatment of RAS-associated leukoproliferative disease (RALD) and Rosai-Dorfman disease in a patient with KRAS mutation.
Leukemia & lymphomaRosai-Dorfman disease of the breast: A six-case clinicopathologic study.
Annals of diagnostic pathologyExtensive Bone Marrow Involvement by BRAF V660E-Mutated Bi-Phenotypic Erdheim-Chester Neoplasm/Rosai-Dorfman Disease (Mixed Histiocytic Neoplasm) with Atypical Histological Features and Fulminant Hemophagocytosis.
Case reports in oncologyIntranodal Rosai-Dorfman Disease and its Extranodal Recurrence: A Rare Case Review.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaRevisiting the molecular landscape of Rosai-Dorfman disease: insights from whole exome sequencing of Saudi patients.
Frontiers in oncologyA Case of Facial Rosai-Dorfman Disease With External Ear, Laryngeal, and Eye Involvement.
International journal of dermatologyA Case of Rosai-Dorfman Disease Complicated by Bone Metastasis From Prostate Cancer.
CureusDiagnostic Challenges in Intracranial Rosai-Dorfman Disease: Differentiating It From Meningiomas Using Imaging.
CureusMixed histiocytosis of Langerhans cell with Rosai-Dorfman disease.
JAAD case reportsMerkel cell polyomavirus DNA sequences in tissue from a patient with Histiocytosis.
Journal of clinical and experimental hematopathology : JCEHEfficacy of Radiotherapy in Patients with Relapsing Primary Rosai-Dorfman Disease of the Nasal Cavity.
Medicina (Kaunas, Lithuania)Primary Cutaneous Rosai-Dorfman Disease in a Treated Case of Breast Carcinoma-Cytological Diagnostic Enigma.
Cytopathology : official journal of the British Society for Clinical Cytology[Cutaneous Rosai-Dorfman disease: one case with auricle, scalp mass and secretory otitis media as clinical manifestations].
Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgeryMultimodality Imaging Assessment of Rosai-Dorfman Disease: A Case Report.
Ultraschall in der Medizin (Stuttgart, Germany : 1980)Case Report: Radiological characteristics and pathological correlation analysis of extranodal Rosai-Dorfman disease of liver.
Frontiers in oncologyRosai-Dorfman Disease in a 4-Month-Old Female Presenting With Cervical Lymphadenopathy.
Clinical case reportsRenal mass secondary to Rosai-Dorfman disease: A case report.
Actas urologicas espanolasNasopharyngeal Representation of Rosai-Dorfman Disease; a Case Report.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaRosai-Dorfman disease of the ascending aorta and pulmonary artery mimicking intramural hematoma and mass.
European heart journalHistiocytoses and reactive proliferations of histiocytes: current state of the art and evolving concepts-a report from the joint CSHP-EA4HP-SH workshop 2024, Hefei, China.
Virchows Archiv : an international journal of pathologyNeuropsychological Aspects of a Patient With Intracranial Rosai-Dorfman Disease: Case Report.
Clinical case reportsRosai-Dorfman Disease of the Pericardium Accompanied by Monoclonal Gammopathy: A Case Report with CT and PET Imaging Findings.
Journal of the Korean Society of RadiologyOrbital and ocular adnexal histiocytic tumors; a multidisciplinary literature review.
Orbit (Amsterdam, Netherlands)Rosai-Dorfman Disease Mimicking Metastasis to the Spleen From Breast Cancer.
Clinical nuclear medicineMonocytic meningitis complicating histiocytosis and response to MEK-inhibitor: a case series.
Annals of hematologyCase Report: Coexistence of Rosai-Dorfman disease and B-cell acute lymphoblastic leukemia in an adolescent.
Frontiers in pediatricsCase report: Pulmonary non-Langerhans cell histiocytosis in a dog with acute interstitial granulomatous pneumonia.
Frontiers in veterinary scienceRespiratory tract involvement in Rosai-Dorfman disease - A report of two distinct cases.
HeliyonCutaneous Rosai-Dorfman disease with spontaneous partial involution.
JAAD case reportsRosai-Dorfman disease disguised as BRAF V600E-positive histiocytosis - a diagnostic pitfall.
Blood researchA case report of breast Rosai-Dorfman disease and a literature review.
Frontiers in oncologyAtypical Pelvic Tumors in Children.
CancersTeaching NeuroImage: Isolated Rosai-Dorfman Disease Resembling a Staghorn in the Spine.
NeurologyMusculoskeletal Manifestations of Rare Lymphoproliferative Neoplasms: A Retrospective Case Report of Four Cases.
Journal of orthopaedic case reportsUnveiling the enigma: Navigating extranodal Rosai-Dorfman disease in the maxilla - A teen's journey.
Journal of oral and maxillofacial pathology : JOMFPRosaï-Dorfman-Destombes Disease: A Clinical Case Report.
CureusA Rare Instance of Rosai-dorfman Disease With Mimicking Meningioma.
The Journal of craniofacial surgeryHypofractionated Radiation Therapy for Subcutaneous Rosai-Dorfman Disease: A Case Report.
Advances in radiation oncologyRefractory Pericardial Effusion in a Patient with Rosai-Dorfman Disease.
The Journal of the Association of Physicians of IndiaUnraveling Histiocytic Disorders: Two Cases From Resource-Limited Settings.
CureusAsymptomatic pink nodules in an African-American woman.
JAAD case reportsPleural Rosai-Dorfman disease complicated with renal clear cell carcinoma: a case report and literature review.
Frontiers in oncologyRare histopathological diagnosis of malakoplakia and Rosai‑Dorfman disease in the same uterus mimicking malignancy: A case report.
Experimental and therapeutic medicineClinical Spectrum and Outcome of Kidney Involvement in Non-Langerhans Histiocytosis.
Kidney international reportsPrimary intraosseous Rosai-Dorfman disease: Clinicopathological features and an assessment of a possible relationship with IgG4-related disease.
Annals of diagnostic pathologyCavoatrial Bypass for Cardiac Complications From Rosai-Dorfman Disease.
Annals of thoracic surgery short reportsRosai-Dorfman Disease Presenting With FDG-Avid Breast Masses and Axillary Lymph Nodes on PET-CT in a Patient With Recent Diagnosis of Endometrial Carcinoma: A Diagnostic Dilemma.
European journal of breast healthAchievement of Clinical Remission in Life-Threatening Rosai-Dorfman Disease With Cladribine.
CureusRosai-Dorfman disease: A rare presentation as an isolated axillary lymphadenopathy, a case report and literature review.
International journal of surgery case reportsCommentary on: "Cutaneous Rosai-Dorfman Disease: A Systematic Review and Reappraisal of Its Treatment and Prognosis".
Archives of dermatological researchApproach to the Patient: From Endocrinopathy to the Diagnosis of a Histiocytic Disorder.
The Journal of clinical endocrinology and metabolismIsolated Osseous Rosai Dorfman Disease of Foot Bones. Report of a Patient Misdiagnosed and Treated as Chronic Osteomyelitis.
International journal of surgical pathologyCase report: Pediatric intraventricular Rosai-Dorfman disease: clinical insights and surgical strategies in a decade-long observational study and literature review.
Frontiers in oncologyExtensive Multiorgan and Neurological Involvement on F-18 FDG PET-CT in a Case of Rosai-Dorfman Disease.
World journal of nuclear medicineBRAF V600E gene mutation is present in primary intraosseous Rosai-Dorfman disease.
Human pathologyRare histiocytic neoplasm: A case report.
Surgical neurology internationalExtranodal Rosai-Dorfman disease masquerading as intrathoracic invasive malignancy with ankylosing spondylitis: a case report.
Journal of cardiothoracic surgeryWarning from heart: An unusual case report of isolated Rosai-Dorfman disease in an Asian.
British journal of haematologyA Rare Case of Rosai Dorfman Destombes Disease Presenting With Lupus Nephritis and Central Nervous System Small Vessel Vasculitis.
CureusBRAF V600E mutation and high expression of PD-L1 in Rosai-Dorfman disease: case report and review of the literature.
Journal of hematopathologyExtranodal Rosai-Dorfman disease in the breast: a literature review from 1969 to 2023.
Cirugia y cirujanosRosai-Dorfman Disease of the Lacrimal Sac.
Ophthalmic plastic and reconstructive surgeryIRF8 Demonstrates Positivity in a Significant Subset of Histiocytic and Dendritic Cell Neoplasms.
The American journal of surgical pathologyRadiology-pathology correlation: Rosai-Dorfman disease.
The neuroradiology journalDestombes-Rosai-Dorfman disease: A case series of nodular and extra nodular involvement.
International journal of surgery case reportsArginine Vasopressin Deficiency Heralding Rosai-Dorfman Disease With Neurological Manifestations.
JCEM case reportsCutaneous involvement in Rosai-Dorfman disease: clinical and dermoscopic features.
International journal of dermatologyFacial cutaneous Rosai-Dorfman disease treated with pulsed dye laser: a case report and literature review.
Anais brasileiros de dermatologiaA Rare Case of Extra-Nodal Rosai-Dorfman Disease of the Cheek.
Journal of Nippon Medical School = Nippon Ika Daigaku zasshi[A case of orbital extranodal Rosai-Dorfman disease with increased IgG4 positive plasma cells].
[Zhonghua yan ke za zhi] Chinese journal of ophthalmologyUncommon Fibroinflammatory Sinonasal Tract Lesions: Granulomatosis with Polyangiitis, Eosinophilic Angiocentric Fibrosis, and Rosai-Dorfman Disease.
Surgical pathology clinics[Multifocal primary Rosai-Dorfman disease of trachea: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologySLC29A3 Pathogenic Variants Resulting in Dural Based Fibroinflammatory Mass Lesions and H Syndrome Treated With Cobimetinib: A Case Report.
Neurology. GeneticsOptic neuritis in Rosai Dorfman disease.
Eye (London, England)A rare case of nodular plaque variant of cutaneous-only Rosai-Dorfman disease: A case report.
SAGE open medical case reportsRosai-Dorfman sphenoorbital histiocytosis with intraparenchymal invasion: Do we have to consider this skull base pathology as a malignant disease?
Surgical neurology internationalClinicopathological characteristics of extranodal Rosai-Dorfman disease: A retrospective case series of 25 patients.
Annals of diagnostic pathologyOrbital masses as a rare presentation of Rosai-Dorfman disease: Clinicopathologic characterization of five cases.
Annals of diagnostic pathologyRare presentation and unconventional treatment of Rosai-Dorfman disease.
BMJ case reportsHistiocytic neoplasms: a brief review and differential diagnosis.
Journal of clinical and experimental hematopathology : JCEHRosai-Dorfman-Destombes disease in adults: a single center experience.
Annals of hematologyExtranodal Rosai-Dorfman Disease Presenting as a Rapidly Enlarging Limbal Mass.
OphthalmologyOne case of Rosai-Dorfman disease misdiagnosed as facial inflammation.
Hua xi kou qiang yi xue za zhi = Huaxi kouqiang yixue zazhi = West China journal of stomatologyRosai-Dorfman disease presenting as a perirectal mass: A case report and review of diagnostic challenges.
Radiology case reportsCommon progenitor origin for Rosai-Dorfman disease and clear cell sarcoma.
The Journal of pathologyGastrointestinal Manifestations of Rosai-Dorfman Disease.
Digestive diseases and sciencesA maxillary location of extra-nodal Rosai Dorfman disease: a case report.
The Pan African medical journalRosai-Dorfman Disease Mimicking Metastatic Disease.
Radiology. Imaging cancerMultimodality Imaging of a Rare Extranodal Rosai-Dorfman Disease Involving Great Vessels.
Circulation. Cardiovascular imagingRosai-Dorfman Disease in a Middle-Aged Man With Recurrent Pleural Effusions and Lymphadenopathy.
CureusCutaneous Rosai-Dorfman disease with MAP2K1 mutation, initially mimicking an infection with parasitized histiocytes.
Journal of cutaneous pathology[Infraglottic portion Rosai-Dorfman disease: a case report].
Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgeryMultifocal Intracranial Rosai-Dorfman Disease Mimicking Immunoglobulin G4-Related Pachymeningitis.
NeurologyThe Lone Lump: Cutaneous Rosai-Dorfman Disease as an Isolated Upper Arm Lesion.
CureusAn Unusual Cause of Lymphadenopathy: Rosai Dorfman Disease in a 7-Year-Old Female Zambian Child: Case Report and Literature Review.
Clinical medicine insights. Case reportsEmperipolesis in pleural fluid mesothelial cells. A phenomenon not associated with Rosai-Dorfman disease, report of a case.
Ultrastructural pathologyManagement of intracranial Rosai-Dorfman disease: An institutional experience.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaCutaneous Rosai-Dorfman disease arising in a patient with psoriatic arthritis.
Italian journal of dermatology and venereologyAssociações
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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.
- Isolated Intracranial Rosai-Dorfman Disease Mimicking Sellar Pituitary Adenoma: a case report and literature review.
- Spine-limited Rosai-Dorfman disease in a patient with ankylosing spondylitis - A rare mimicker of spinal tumors.
- Rosai-Dorfman disease mimicking testicular malignancy in a patient with marginal zone lymphoma of the lung.
- Spinal Rosai-Dorfman disease: systematic review and meta-analysis of clinical presentation, diagnosis, and treatment.Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia· 2026· PMID 41385968mais citado
- Rosai-Dorfman Disease Primarily Involved in the CNS.
- Cutaneous Rosai-Dorfman Disease Misdiagnosed as Granulomatous Dermatitis: A Diagnostic Pitfall.
- Hodgkinoid histiocytosis, a distinctive histiocytic/dendritic cell disorder clinically mimicking drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms.
- Rosai-Dorfman Disease in Nasal Cavity and Paranasal Sinuses: Imaging Features in a Series of 23 Patients.
- Multifocal Rosai Dorfman disease and simultaneous endometrioid ovarian cancer: a case report.
- Rosai-Dorfman disease with thyroid involvement: a case report and literature review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:158014(Orphanet)
- MONDO:0006412(MONDO)
- GARD:7588(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q1567631(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
