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1.
Medicine (Baltimore) ; 99(2): e18757, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31914099

RESUMO

RATIONALE: Rosai-Dorfman disease (RDD) is a rare nonmalignant cell histiocytosis. Here, we report a rare, unusual clinical presentation of epibulbar and orbital tumor as a manifestation of RDD. We also review the literatures on clinical cases of orbital RDD. PATIENT CONCERNS: A 44-year-old Chinese male was admitted with a 3-month history of eye redness, subconjunctival mass, and diplopia. DIAGNOSIS: An initial diagnosis of epibulbar and orbital tumor was made according to the clinical symptoms, signs, and pre-operative contrast-enhanced computerized tomography results. INTERVENTIONS: The mass was completely resected and pathology confirmed the RDD diagnosis. The patient received steroids after surgery. OUTCOMES: The patient recovered well on 18-month follow-up. CONCLUSION: This appears to be the first report of RDD manifesting as epibulbar and orbital tumor. Pre-operative diagnosis of RDD remains challenging. When the lesion causes diplopia, surgical resection is the most effective treatment.


Assuntos
Histiocitose Sinusal/diagnóstico , Histiocitose Sinusal/patologia , Adulto , Diagnóstico Diferencial , Neoplasias Oculares/diagnóstico , Neoplasias Oculares/patologia , Histiocitose Sinusal/cirurgia , Humanos , Masculino , Tomografia Computadorizada por Raios X
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
9.
Ann Diagn Pathol ; 40: 166-173, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31108464

RESUMO

BACKGROUND: Cutaneous Rosai-Dorfman (CRD) disease is a rare entity that is characterized by histiocytic proliferation in the skin. The disease has been reported to exhibit different clinical profiles and occasionally confounding histologic features that may be challenging for a correct diagnosis. The purpose of this study was to assess the pathobiology and highlight the variance in clinical and histologic spectrum of the disease based on published literature. METHODS: A PUBMED search was performed to retrieve cases of cutaneous Rosai-Dorfman disease published in the literature. A PRISMA-guided review of the included articles was performed. Three interesting case reports from our institution are also described. RESULTS: A total of 263 patients, of which 220 with purely cutaneous disease were identified in 152 studies. The mean age at presentation was 45.2 years with a slight female preponderance, and East-Asian, Caucasian and African populations being largely affected. Majority of the patients presented with multiple lesions, predominantly on limbs and comprising of nodules, plaques and papules that were occasionally pigmented. The classis histologic findings included large foamy histiocytes, exhibiting emperipolesis and a specific immunophenotype (S100+, CD68+, CD1a-). Inconspicuous emperipolesis, fibrosis, increased vascularity, neutrophilic microabscesses and concurrent langerhans cell histiocytosis and lymphoma in few cases highlighted the importance of immunohistochemistry for a definitive diagnosis. The disease shows an indolent and benign course with excision and chemotherapy being most effective for extensive and refractory cases. CONCLUSIONS: This review of largest cohort of CRD patients provides an updated insight into the clinicopathologic features with possible diagnostic pitfalls and effective therapeutic options that should be useful in diagnosis, management and future research opportunities.


Assuntos
Histiocitose de Células de Langerhans/diagnóstico , Histiocitose Sinusal/diagnóstico , Dermatopatias/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Histiocitose de Células de Langerhans/patologia , Histiocitose Sinusal/patologia , Humanos , Imunofenotipagem , Masculino , Pessoa de Meia-Idade , Pele/patologia , Dermatopatias/patologia , Adulto Jovem
10.
Medicine (Baltimore) ; 98(18): e15548, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045846

RESUMO

RATIONALE: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis. Petroclival RDD is extremely rare. To the best of our knowledge, only 7 cases of petroclival RDD have been reported so far. Herein, we present the 8th case of intracranial RDD with the petroclival and parasellar involvement mimicking multiple meningiomas. PATIENT CONCERNS: A 57-year-old woman presented with a 1-year history of vision diminution and 1-month hearing loss in her right ear. DIAGNOSES: Contrast-enhanced Magnetic resonance imaging (MRI) of the brain demonstrated multiple well-defined, homogenous mass which closely related to the dura mater in the bilateral parasellar and petroclival regions range from the basement of anterior to posterior cranial fossa. The lesions were T1 isointense, T2 hypointense, and homogeneously enhanced. Initial diagnosis of multiple meningiomas was made according to MRI findings. Final diagnosis of RDD was confirmed by histopathological and immunohistochemical examinations after subtotal surgical resection. INTERVENTIONS: The patient received subtotal resection because multiple lesions were extensive. OUTCOMES: The vision diminution recovered well after the surgery but the hearing loss in her right ear was still persisted. LESSONS: Although rare, a standard RDD typically are dural-based, extra-axial, well-circumscribed masses mimicking meningioma, and presenting with characterized hypo to isointense on T1-weighted images, hypo to isointense on T2-weighted images, and obvious enhancement. Resection of the intracranial lesion is the most effective treatment. In case of subtotal resection, the application of adjunctive radiotherapy and/or steroid agents should be advised. Final diagnosis of RDD should be confirmed by histopathological and immunohistochemical examinations.


Assuntos
Encefalopatias/diagnóstico , Histiocitose Sinusal/diagnóstico , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Encefalopatias/patologia , Diagnóstico Diferencial , Feminino , Histiocitose Sinusal/patologia , Humanos , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Base do Crânio/diagnóstico por imagem , Base do Crânio/patologia
11.
BMC Pulm Med ; 19(1): 83, 2019 May 03.
Artigo em Inglês | MEDLINE | ID: mdl-31053123

RESUMO

BACKGROUND: Rosai-Dorfman disease (RDD) is a rare histiocytosis which involves principally lymph nodes. Thyroid involvement in RDD is a very rare situation, and lung involvement is even rarer. CASE PRESENTATION: We report the case of a 46-year-old woman presenting a painless mass in the right side of the neck and subacute dyspnoea. Computerised tomography (CT) scans of the neck and thorax showed a large thyroid mass causing tracheal stenosis and multiple cystic lesions in both lungs. Subtotal thyroidectomy with a tracheal segment resection and histological analysis confirmed the diagnosis of nodal and extranodal (thyroid, tracheal and probably lung) Rosai-Dorfman disease (RDD) with the presence of increased numbers of IgG4-bearing plasma cells. Clinical, functional and radiological follow up 4 years after surgery without medical treatment did not show any disease progression. CONCLUSIONS: This case report indicates a benign course of nodal RDD with thyroid and tracheal infiltration following surgical resection, association of typical histological signs of RDD (emperipolesis) with IgG4-related disease features, and that lung cysts might be a manifestation of RDD.


Assuntos
Histiocitose Sinusal/patologia , Histiocitose Sinusal/cirurgia , Linfonodos/patologia , Glândula Tireoide/patologia , Cistos/patologia , Diagnóstico Diferencial , Feminino , Humanos , Imunoglobulina G/sangue , Imuno-Histoquímica , Pulmão/patologia , Pessoa de Meia-Idade , Plasmócitos/patologia , Glândula Tireoide/crescimento & desenvolvimento , Tireoidectomia , Tomografia Computadorizada por Raios X , Estenose Traqueal/etiologia
14.
Indian J Pathol Microbiol ; 62(2): 319-322, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30971567

RESUMO

Emperipolesis is the hallmark finding for Rosai-Dorfman disease. Till now many studies in literatures have shown emperipolesis as a finding in other benign as well as malignant conditions. Very few cases of malignant lymphoma have this phenomenon. Herewith, we put forward a rare case of lymphoma with clinical presentation showing involvement of spleen, liver, lymph nodes as well as lleo-cecal region. Light microscopy revealed large to medium sized lymphoid cells with intervening plenty of histiocytes showing evidence of emperipolesis that mimics Rosai Dorfmann disease. Due to atypical clinical presentations we thought of lymphoma as a differential diagnosis. Further immunohistochemistry was performed using histiocytic as well as lymphoid markers. To our surprise, it turns out to be Non Hodgkin Lymphoma with extensive emperipolesis which is extremely rare in thorough literature search. This case is presented due to its unique clinical as well as histological presentations.


Assuntos
Emperipolese , Linfoma não Hodgkin/diagnóstico , Biomarcadores , Colectomia , Diagnóstico Diferencial , Histiócitos/patologia , Histiocitose Sinusal/diagnóstico , Histiocitose Sinusal/patologia , Humanos , Imuno-Histoquímica , Fígado/patologia , Fígado/cirurgia , Linfonodos/patologia , Linfoma não Hodgkin/complicações , Masculino , Pessoa de Meia-Idade , Baço/anormalidades , Baço/patologia , Esplenectomia
17.
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
20.
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
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