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1.
J Comput Assist Tomogr ; 44(3): 450-461, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31972751

RESUMO

OBJECTIVE: Rosai-Dorfman disease (RDD) is a rare and idiopathic nonneoplastic disease of histiocytes that is characterized by lymphadenopathy and extranodal disease. In this study, we documented anatomical preferences, imaging findings, comorbid diseases, and ethnic differences in 32 RDD patients. METHODS: We conducted a retrospective review of pathologically confirmed cases seen at our institution from 1998 to 2016. These cases were analyzed for (a) anatomical locations, (b) radiologic appearance, (c) comorbid diseases, and (d) differences between ethnic groups. RESULTS: We found 32 patients with RDD, 18 were women and 14 were men. There were 51 lesions in all patients, 23.5% of which were nodal, involving 11 lymph node regions, and 76.5% were extranodal. Cervical lymph nodes and maxillofacial area were the most common affected nodal and extranodal locations, respectively. Only 4 (12.5%) of 32 patients had pure nodal involvement, whereas 20 (62.5%) of 32 had pure extranodal disease and 8 (25%) of 32 had mixed nodal and extranodal disease.Anatomically, RDD affected multiple organs in our cohort, including the lymphatic system, maxillofacial area (glandular and nonglandular tissues), superficial soft tissue, central nervous system, breast, peritoneum, gastrointestinal tract, and lungs.Radiologically, RDD presentation was variable from an organ to another. However, most lesions were hypermetabolic on 18F-fluorodeoxyglucose positron-emission tomography/computed tomography and isointense on T1-weighted magnetic resonance imaging. Computed tomographic findings were extremely variable between organs.Comorbid diseases were found in 11 patients. Those patients had 17 comorbid diseases; the most common were autoimmune diseases, viral diseases, and cancer.The organ distribution of RDD was slightly different between ethnic groups. The most frequent disease location for African Americans was lymph nodes; for whites, central nervous system and nonglandular maxillofacial (27.3% each); for Asians, lymph nodes, subcutaneous tissue, and nonglandular maxillofacial (25% each); and for Hispanics, lymph nodes and glandular maxillofacial (33.3% each). CONCLUSIONS: Rosai-Dorfman disease represents a wide-spectrum disease not limited to lymph nodes. Radiologically, RDD has diverse imaging findings. However, most lesions were hypermetabolic on 18F-fluorodeoxyglucose positron-emission tomography/computed tomography and isointense on T1-weighted imaging. Patients with RDD have a high rate of comorbid diseases including autoimmune disease, viral infections, and cancer.


Assuntos
Histiocitose Sinusal , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Comorbidade , Feminino , Fluordesoxiglucose F18 , Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/epidemiologia , Histiocitose Sinusal/patologia , Humanos , Linfonodos/patologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Estudos Retrospectivos , Adulto Jovem
2.
World Neurosurg ; 133: 69-73, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31550540

RESUMO

BACKGROUND: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-Langerhans cell reactive histiocytic disorder that rarely occurs in the central nervous system (CNS). Extranodal RDD most frequently involves the skin, upper respiratory tract, soft tissue, gastrointestinal tracts, bones, breast, and CNS. CASE DESCRIPTION: RDD of the CNS infiltrates most commonly the dura of the sella, cavernous sinus, and the periclival regions. It is usually clinically and radiologically mistaken for meningioma because of its focal dural-based aspect. RDD is confirmed histologically by lymphoplasmacytic cells and histiocytes of varying size showing emperipolesis (lymphocytophagocytosis). To date, only 4 cases of RDD displaying spinal cord compression secondary to craniocervical junction involvement have been reported. CONCLUSION: We report the case of a patient diagnosed with RDD localized at the foramen magnum extending to the base of the odontoid process and involving the V4 segment vertebral artery.


Assuntos
Histiocitose Sinusal/cirurgia , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Compressão da Medula Espinal/cirurgia , Artéria Vertebral/cirurgia , Diagnóstico Diferencial , Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/patologia , Artéria Vertebral/diagnóstico por imagem , Artéria Vertebral/patologia
4.
Clin Nucl Med ; 44(11): 873-875, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31584488

RESUMO

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonneoplastic histiocytic disorder that often involves the lymph nodes and occasionally the central nervous system or skeleton. However, the presence of extranodal Rosai-Dorfman lesions without lymphadenopathy is extremely rare. Here we present a case of a pathological confirmed Rosai-Dorfman disease with pituitary and skeletal involvement without lymph nodes involvement.


Assuntos
Osso e Ossos/patologia , Fluordesoxiglucose F18 , Histiocitose Sinusal/diagnóstico por imagem , Hipófise/patologia , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Osso e Ossos/diagnóstico por imagem , Criança , Histiocitose Sinusal/patologia , Humanos , Masculino , Hipófise/diagnóstico por imagem
5.
World Neurosurg ; 132: 309-313, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31525482

RESUMO

INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare disease that can be triggered by either viral or bacterial infection. Several parts of the body can be involved, from the central nervous system to the pelvic regions had been reported. At present, there is a serious lack of guidelines as to how to treat cases of RDD involving the spine. Current trends show that surgery remains the first method of choice to cure this disease, but in refractory or recurrent RDD, repeat surgery cannot guarantee total resection. Under such circumstances, adjuvant therapy can be very useful. Here, we share our experience of treating recurrent spinal RDD. CASE DESCRIPTION: Our patient was a 32-year-old male patient with hepatitis B virus infection. He suffered from lower back pain with lower limb weakness after being hit by a metal pipe. Laminectomy of T11-12 was performed to decompress the spinal cord. The final pathological results demonstrated chronic inflammation. After surgery, the lower limbs improved and the patient recovered quite well. However, he returned 3 years later with the same complaints and was diagnosed with RDD. Durectomy was performed and repaired with an artificial dura. Thalidomide therapy was initiated. Currently, 9 years later, the patient has not experienced recurrence or any form of neurological deficit. CONCLUSIONS: RDD is a rare disease and can be misdiagnosed easily. Although it may resolve spontaneously, recurrence is possible. Hence, extensive follow-up is necessary. Surgery remained the first choice of surgery, however, when encountered recurrent or nonresectable RDD, adjuvant therapy such as corticosteroids, thalidomide, and radiotherapy could help. In this article, we shared our experience using thalidomide in treating nonresectable RDD.


Assuntos
Histiocitose Sinusal/terapia , Imunossupressores/uso terapêutico , Doenças da Coluna Vertebral/terapia , Talidomida/uso terapêutico , Vértebras Torácicas/cirurgia , Adulto , Histiocitose Sinusal/complicações , Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/patologia , Humanos , Dor Lombar/etiologia , Extremidade Inferior , Masculino , Debilidade Muscular/etiologia , Recidiva , Doenças da Coluna Vertebral/complicações , Doenças da Coluna Vertebral/diagnóstico por imagem , Doenças da Coluna Vertebral/patologia
6.
J Pak Med Assoc ; 69(8): 1213-1215, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31431783

RESUMO

Sinus histiocytosis with massive lymphadenopathy also known as Rosai-Dorfman disease is a rare benign disease that typically manifests as lymphadenopathy with or without systemic manifestations whose etiology remains poorly understood. Most common clinical presentation is painless bilateral cervical lymphadenopathy. However, it also can present in various extranodal sites and can easily be missed because of its rarity if not considered in the differential diagnosis. It commonly occurs in children and young adults with a slightly male predominance. Clinically patients may be mistaken for lymphoma and other infectious disorders like tuberculosis especially in developing countries like in Pakistan where tuberculosis is more prevalent. Here we report a case of a 38-years-old gentleman with bilateral cervical lymphadenopathy and worsening ascites. His symptoms initially mimicking tuberculosis and lymphoma, was finally diagnosed as having Rosai-Dorfman's Disease on intra abdominal lymph node biopsy.


Assuntos
Histiocitose Sinusal/diagnóstico , Adulto , Biópsia , Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/patologia , Humanos , Linfonodos/patologia , Masculino , Tomografia Computadorizada por Raios X
7.
J Craniofac Surg ; 30(6): e489-e490, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30921073

RESUMO

Rosai-Dorfman disease is very an important histoproliferative disorders. It rarely involves cranial and facial bones. It can make intracranial and extracranial region extension. Therefore, it can mimic dermal and dural pathologies. Target sign is an important sign for diagnosis on computed tomography and magnetic resonance imaging.


Assuntos
Histiocitose Sinusal/diagnóstico por imagem , Administração Cutânea , Criança , Diagnóstico Diferencial , Humanos , Imagem por Ressonância Magnética , Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X
9.
Clin Nucl Med ; 44(6): 485-488, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30762829

RESUMO

Rosai-Dorfman disease is typically presented as lymphadenopathy, especially in the neck. Extranodal diseases also frequently occur but are generally concurrent with nodal disease. We report FDG PET/CT findings of solitary femoral Rosai-Dorfman disease without any lymphadenopathy in a pediatric patient.


Assuntos
Histiocitose Sinusal/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Criança , Fluordesoxiglucose F18 , Humanos , Masculino , Compostos Radiofarmacêuticos
10.
Radiologia ; 61(3): 215-224, 2019.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30686482

RESUMO

OBJECTIVE: To review the imaging findings for the different types of pulmonary histiocytosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans cell histiocytosis in Rosai-Dorfman disease and Erdheim-Chester disease. We also review the etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis. CONCLUSION: Langerhans cell histiocytosis, Rosai-Dorfman disease, and Erdheim-Chester disease are idiopathic diseases in which the proliferation and infiltration of histiocytes is the histologic finding that confirms the diagnosis. Langerhans cell histiocytosis manifests as nodules and cysts that spare the costophrenic angles; it typically appears in smokers. Although it is uncommon in nonsmokers, Langerhans cell histiocytosis should also be considered in nonsmokers treated with chemotherapy and radiotherapy in whom cavitated nodules appear and should be included in the differential diagnosis together with metastatic disease and opportunistic infections. Rosai-Dorfman disease and Erdheim-Chester disease present with less specific thoracic findings such as adenopathies, interstitial thickening, and pleural effusion. In Erdheim-Chester disease, the characteristic extrathoracic manifestations are usually key for the diagnosis.


Assuntos
Doença de Erdheim-Chester/diagnóstico por imagem , Histiocitose de Células de Langerhans/diagnóstico por imagem , Histiocitose Sinusal/diagnóstico por imagem , Fumar/efeitos adversos , Adulto , Doença de Erdheim-Chester/etiologia , Doença de Erdheim-Chester/patologia , Doença de Erdheim-Chester/terapia , Feminino , Histiocitose de Células de Langerhans/etiologia , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/terapia , Histiocitose Sinusal/etiologia , Histiocitose Sinusal/patologia , Histiocitose Sinusal/terapia , Humanos , Pneumopatias , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Adulto Jovem
11.
Asian Cardiovasc Thorac Ann ; 27(2): 132-134, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30509136

RESUMO

Rosai-Dorfman disease or sinus histiocytosis is a nonmalignant disease chiefly presenting as a painless cervical lymphadenopathy, although extranodal involvement is also frequent. Involvement of the tracheobronchial tree is extremely rare, and there are no clear guidelines on management. Rosai-Dorfman disease usually presents as a tracheal tumor, although it may also present with extraluminal compression. Only 12 cases have been reported in the English language literature. Histopathology is required to confirm the diagnosis. We describe what we think is India's first reported case of histologically proven tracheal Rosai-Dorfman disease in a 40-year-old otherwise healthy lady.


Assuntos
Histiócitos/patologia , Histiocitose Sinusal/patologia , Doenças da Traqueia/patologia , Adulto , Biomarcadores/análise , Biópsia , Broncoscopia , Feminino , Histiócitos/química , Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/cirurgia , Humanos , Imuno-Histoquímica , Proteínas S100/análise , Doenças da Traqueia/diagnóstico por imagem , Doenças da Traqueia/cirurgia , Resultado do Tratamento
12.
Int J Neurosci ; 129(4): 393-396, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30296195

RESUMO

The association between Rosai-Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.


Assuntos
Encéfalo/patologia , Neoplasias da Mama/complicações , Histiocitose Sinusal/etiologia , Histiocitose Sinusal/patologia , Encéfalo/diagnóstico por imagem , Feminino , Histiocitose Sinusal/diagnóstico por imagem , Humanos , Pessoa de Meia-Idade
13.
J Hand Surg Am ; 44(1): 70.e1-70.e5, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29678430

RESUMO

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare entity in which bone involvement occurs in less than 5% to 10% of patients and skeletal cases are usually multifocal. Extranodal solitary lesions of bone without nodal involvement or additional clinical manifestations are exceedingly rare. We present the clinical course and management of a 34-year-old African American woman who presented with the disease as a solitary lesion of the distal radius.


Assuntos
Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/cirurgia , Rádio (Anatomia)/diagnóstico por imagem , Rádio (Anatomia)/cirurgia , Adulto , Feminino , Humanos , Imagem por Ressonância Magnética , Radiografia
16.
Arch. Soc. Esp. Oftalmol ; 93(10): 515-518, oct. 2018. ilus
Artigo em Espanhol | IBECS | ID: ibc-175129

RESUMO

Objetivo: Reportar un caso de tumoración orbitaria y proptosis, diagnosticado como enfermedad de Rosai-Dorfman (ERD). Método: Reporte de caso clínico basado en revisión de la historia clínica, imágenes radiológicas y resultados de patología. Resultados: Varón de 42 años con tumoración orbitaria y proptosis del ojo derecho. Se obtuvo una biopsia quirúrgica y el diagnostico de ERD se realizó mediante microscopia e inmunohistoquímica. El manejo definitivo incluyó citorreducción tumoral abierta, con buena respuesta. Discusión: La ERD es una rara condición proliferativa, benigna de origen desconocido. Raramente afecta las cavidades craneanas, siendo la presentación orbitaria muy inusual. La confirmación diagnóstica es clave para tomar decisiones quirúrgicas


Objective: To report a case of orbital mass and proptosis, diagnosed as Rosai-Dorfman disease (RDD). Methods: Clinical case report based on the review of clinical charts, radiological images, and histopathology. Results: A 42-year-old male with orbital mass and proptosis of the right eye. A surgical biopsy was performed, and the diagnosis of RDD was established using microscopy and immunohistochemistry. Definitive management included open tumour cytoreduction, with good response. Discussion: The RDD is a rare, benign, proliferative condition of unknown origin. It rarely affects the cranial cavities, with the orbital presentation being very unusual. Diagnostic confirmation is essential for the best surgical management


Assuntos
Humanos , Masculino , Adulto , Histiocitose Sinusal/diagnóstico por imagem , Histiocitose Sinusal/cirurgia , Aparelho Lacrimal/diagnóstico por imagem , Procedimentos Cirúrgicos Oftalmológicos , Histiocitose Sinusal/patologia , Fundo de Olho , Tomografia Computadorizada Espiral/métodos , Neoplasias Oculares/diagnóstico por imagem , Neoplasias Oculares/cirurgia , Imuno-Histoquímica
18.
Ann Neurol ; 84(1): 147-152, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-30014527

RESUMO

Rosai-Dorfman disease (RDD) is an uncommon histiocytic proliferative disorder that can present in nodal, extranodal, or, extremely rarely, in central nervous system (CNS)-restricted form. RDD is characterized histologically as a non-Langerhans cell histiocytosis composed of atypical CD68+ /S-100+ /CD1a- macrophages demonstrating prominent emperipolesis and effacement of the surrounding tissue. Previously thought to represent a reactive process, recent studies have raised the possibility that RDD and other histiocytic lesions, including Erdheim-Chester and Langerhans cell histiocytosis, are clonal processes linked to somatic mutations in the mitogen-activated protein (MAP) kinase pathway. Herein, we present a fatal case of RDD isolated to the CNS and used a next-generation targeted gene panel and Sanger sequencing to uncover a pathogenic deletion in the ß3-αC loop of the kinase domain in exon 12 of BRAF. This mutation, previously described in melanoma and Langerhans cell histiocytosis, represents the first BRAF mutation of this kind identified in RDD. These findings support the idea that RDD is a neoplastic condition and raise the possibility that inhibitors of the MAP kinase pathway may be effective in RDD. Ann Neurol 2018;83:147-152.


Assuntos
Sistema Nervoso Central/patologia , Histiocitose Sinusal/genética , Histiocitose Sinusal/patologia , Mutação/genética , Proteínas Proto-Oncogênicas B-raf/genética , Antígenos CD/metabolismo , Sistema Nervoso Central/metabolismo , Feminino , Testes Genéticos , Proteína Glial Fibrilar Ácida/metabolismo , Histiocitose Sinusal/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Modelos Moleculares , Proteínas S100/metabolismo
20.
Gulf J Oncolog ; 1(26): 11-17, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29607816

RESUMO

AIM: To study the pattern of presentation and management of nodal and extranodal primary RDD. METHODOLOGY: From 2010 to 2015, eleven patients diagnosed with Rosai-Dorfman Disease (RDD) presented at our institute. Nine of them were analyzed as they received treatment at our center. We had five neck primaries, three intracranial and one cutaneous disease. Hematological, radiological, histopathological and immunohistochemistry evaluation along with the treatment data was collected and analyzed. RESULTS: Nine cases treated at our institute had a median follow up of 41 months. Four of the five neck node primaries were treated with corticosteroids. Of the four, two had complete and two partial responses locally. Remaining one patient had partial response to surgery and corticosteroids. Three patients with CNS presentation treated with radiotherapy to a total dose of 20-40 Gy, had complete response locally. However, two patients had simultaneous neck nodes during presentation. Partial response was seen with corticosteroids alone in one and with radiation to the neck in the other patient, of which one succumbed to myocardial infarction within 3 months post treatment. Two of them received re-irradiation to the recurrent lesions with good results. CONCLUSION: Due to its rarity there is no particular guidelines regarding the management of patients with RDD. We conclude that low dose radiation either in combination with surgery or corticosteroids in nodal or extranodal lesions has shown a better local control.


Assuntos
Histiocitose Sinusal/patologia , Histiocitose Sinusal/terapia , Imagem Multimodal/métodos , Centros de Atenção Terciária , Adolescente , Adulto , Idoso , Terapia Combinada , Feminino , Histiocitose Sinusal/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
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