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1.
Article in Chinese | WPRIM | ID: wpr-927864

ABSTRACT

Objective To investigate the clinical and magnetic resonance imaging(MRI) manifestations of Rosai-Dorfman disease(RDD) in central nervous system. Method The clinical and MRI data of 5 cases of RDD in central nervous system confirmed by pathology in the PLA General Hospital were analyzed retrospectively. Results The 5 cases included 4 males and 1 female,aged(39.8±21.7) years on average.Among them,4 cases were located in the intracranial area and 1 case in the thoracic spinal canal.The lesion showed isointense signal on T1 weighted image and iso,slight-hypo,and slight-hyperintense signals on T2 weighted image,and it presented intensively homogeneous enhancement in contrast-enhanced MRI.Two cases showed compressed brain area with edema around the left parietal and left frontotemporal dura,thickening and enhancement in the adjacent dura,and dural tail sign.Three cases presented bone destruction in adjacent diploe and thoracic vertebrae.One case showcased slight-hypo perfusion of the left parietal dura in arterial spin labeling. Conclusions RDD lesion usually appears as iso,slight hypo and slight hyper-intense signals on T2 weighted image and presents intensively homogeneous enhancement in contrast-enhanced MRI.The disease may involve the adjacent bone and the lesion shows slight hypo-perfusion on perfusion images.The MRI manifestations of RDD are characteristic,which are helpful for preoperative diagnosis and evaluation of RDD.


Subject(s)
Central Nervous System/pathology , Female , Head , Histiocytosis, Sinus/pathology , Humans , Magnetic Resonance Imaging/methods , Male , Retrospective Studies
3.
An. bras. dermatol ; 93(3): 432-434, May-June 2018. graf
Article in English | LILACS | ID: biblio-949869

ABSTRACT

Abstract: A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.


Subject(s)
Humans , Male , Aged , Histiocytosis, Sinus/pathology , Multiple Myeloma/pathology , Skin/pathology , Biopsy , Histiocytosis, Sinus/diagnosis , Multiple Myeloma/diagnosis
4.
Autops. Case Rep ; 6(4): 49-55, Oct.-Dec. 2016. ilus
Article in English | LILACS | ID: biblio-905100

ABSTRACT

Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.


Subject(s)
Humans , Female , Adult , Histiocytosis, Sinus/drug therapy , Histiocytosis, Sinus/pathology , Maxilla/pathology , Rare Diseases/diagnosis
5.
An. bras. dermatol ; 91(5): 634-637, Sept.-Oct. 2016. graf
Article in English | LILACS | ID: biblio-827744

ABSTRACT

Abstract: Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.


Subject(s)
Humans , Female , Adolescent , Skin Diseases/pathology , Histiocytosis, Sinus/pathology , Arm , Antigens, Differentiation, Myelomonocytic/metabolism , S100 Proteins/metabolism , Antigens, CD/metabolism , Diagnosis, Differential , Histiocytes/pathology , Leg
6.
An. bras. dermatol ; 89(1): 171-172, Jan-Feb/2014. graf
Article in English | LILACS | ID: lil-703529

ABSTRACT

Cutaneous Rosai-Dorfman disease is a rare, lymphoproliferative disease. It is benign and self-limited, only involves skin and subcutaneous tissue and typically occurs as histiocyte-rich inflammatory infiltrates, manifesting as erythematous to brown papules, plaques, or nodules, without predilection for site. The authors describe a case of cutaneous Rosai-Dorfman disease in a 72-year-old man who presented erythematous and scaled plaque on the right neck for three months without systemic symptoms. Owing to local involvement, the patient received a surgery to exsect the lesion completely and remained asymptomatic with no signs of recurrence at the 9-month follow up.


Subject(s)
Humans , Male , Aged , Skin Diseases/pathology , Histiocytosis, Sinus/pathology , Skin/pathology , Skin Diseases/surgery , Histiocytosis, Sinus/surgery , Neck/pathology
7.
An. bras. dermatol ; 88(2): 256-259, abr. 2013. graf
Article in English | LILACS | ID: lil-674176

ABSTRACT

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.


A doença de Rosai-Dorfman é uma desordem benigna, autolimitada, idiopática, decorrente da proliferação de histiócitos. Foi descrita pela primeira vez por Rosai e Dorfman em 1969. Tipicamente, a doença se caracteriza por extensa linfadenopatia cervical, febre, gamopatia monoclonal e leucocitose com neutrofilia. Manifestações extranodais são encontradas em 43% dos casos, sendo a pele o sítio extranodal mais comumente afetado. No trabalho, apresentamos um caso de doença de Rosai-Dorfman com apresentação cutânea exuberante na coxa direita e discreto comprometimento linfonodal limitado à cadeia regional.


Subject(s)
Female , Humans , Middle Aged , Histiocytosis, Sinus/pathology , Skin Diseases/pathology , Skin/pathology , Biopsy , Thigh
8.
Article in English | IMSEAR | ID: sea-140198

ABSTRACT

Background: Oral squamous cell carcinoma is the most common neoplasm and comprises of approximately 80% of the cancers occurring in the oral cavity. The role of the host response to this neoplasm has been recognized, and for many years the regional lymph node in tumor-bearing hosts has been considered as an anatomic barrier to the systematic dissemination of tumor cells. Morphological evaluation of the regional nodes has aided in understanding the immune response. Aim: The current study was carried out to observe the morphological changes occurring in the regional lymph nodes and to evaluate whether these features could be helpful in assessing the immunological status of the patient, and thereby, the prognosis of the patient. Materials and Methods: The study was based on lymph nodes from 63 patients with oral squamous cell carcinoma, who underwent radical neck dissection or modified neck dissection. In the lymph node, four morphological patterns were observed that included lymphocyte predominance, germinal center predominance, mixed pattern (sinus Histiocytosis), and an unstimulated pattern. The cases were then divided into four groups according to the predominant immunoreactivity pattern based on the World Health Organization (WHO) standardized system for reporting human lymph node morphology. Results: Revealed that risk of metastases to cervical lymph nodes in patients with lymphocyte predominance was less (28.6%) when compared to the high risk of metastases with germinal center predominance (68%), and these results were statistically significant (P < 0.05). Patients with a mixed pattern showed less risk of metastases (45.4%), while those with an unstimulated pattern had increased risk of metastases (66.6%), but the results were not statistically significant. It was also found that in the positive nodes, germinal center hyperplasia (50.2%) was the predominant pattern. Conclusion: The present study revealed that patients with lymphocyte predominance had less risk of metastases and patients with germinal center predominance had a high risk of metastases to the lymph node.


Subject(s)
Capillaries/pathology , Carcinoma, Squamous Cell/immunology , Carcinoma, Squamous Cell/pathology , Carcinoma, Squamous Cell/secondary , Endothelial Cells/pathology , Endothelium, Vascular/pathology , Forecasting , Germinal Center/pathology , Histiocytosis, Sinus/pathology , Humans , Hyperplasia , Lymph Nodes/immunology , Lymph Nodes/pathology , Lymphatic Metastasis/immunology , Lymphatic Metastasis/pathology , Lymphocytes/pathology , Macrophages/pathology , Mouth Neoplasms/immunology , Mouth Neoplasms/pathology , Neck Dissection/methods , Prognosis , Risk Factors
9.
Hematology, Oncology and Stem Cell Therapy. 2011; 4 (2): 94-96
in English | IMEMR | ID: emr-129764

ABSTRACT

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign indolent disorder, characterized by enlarged lymph nodes filled with histiocytes. Extranodal involvement is uncommon. The disease rarely affects the nose and paranasal sinuses. We report a case that presented with a right nasal mass, extending into all the paranasal sinuses and right orbit without any accompanying lymphadenopathy. Because of the absence of lymphadenopathy it posed a diagnostic challenge until the pathology was confirmed on histopathological examination


Subject(s)
Humans , Female , Adult , Histiocytosis, Sinus/pathology , Histiocytosis, Sinus/drug therapy , Paranasal Sinus Neoplasms/diagnosis , Orbital Neoplasms/diagnosis
10.
MEAJO-Middle East African Journal of Ophthalmology. 2011; 18 (4): 323-325
in English | IMEMR | ID: emr-144110

ABSTRACT

Rosai-Dorfman disease [RDD] is characterized by histiocytic proliferation and massive cervical lymphadenopathy, although some patients have extra-nodal involvement. We report a case of extranodal RDD in a five-year-old child, initially misdiagnosed as orbital inflammatory disease and treated with oral steroids. A subsequent orbital biopsy three years later confirmed the diagnosis of Rosai Dorfman disease


Subject(s)
Humans , Male , Orbital Diseases/diagnosis , Histiocytosis, Sinus/pathology
11.
An. bras. dermatol ; 85(5): 687-690, set.-out. 2010. ilus, tab
Article in Portuguese | LILACS | ID: lil-567831

ABSTRACT

A doença de Rosai-Dorfman, também denominada histiocitose sinusal com linfadenopatia maciça, é histiocitose de células não Langerhans, idiopática e de curso benigno. Descrita em 1969, caracteriza-se por linfadenomegalia não dolorosa, sendo a cadeia cervical a mais envolvida, além de febre, perda de peso e sudorese. O envolvimento extranodal ocorre em 43 por cento dos casos, em que múltiplos sítios podem ser acometidos. Já foram descritos casos exclusivamente extranodais, inclusive formas limitadas à pele. Relata-se um caso de doença de Rosai-Dorfman extranodal cutânea pura, devido à raridade dessa apresentação clínica.


Rosai-Dorfman disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a non-Langerhans cell histiocytosis with a benign course and unknown etiology. It was described in 1969 as a painless cervical lymph node enlargement in association with fever, weight loss and sweating. Extranodal disease has been reported in 43 percent of cases, with involvement of multiple organs. Purely extranodal Rosai-Dorfman disease has been already reported, including forms restricted to the skin. This paper reports a case of purely cutaneous Rosai-Dorfman disease, which is of interest in view of the rarity of this condition.


Subject(s)
Adult , Female , Humans , Histiocytosis, Sinus/pathology , Biopsy , Histiocytosis, Sinus/surgery , Immunohistochemistry , Skin/pathology
13.
J. bras. pneumol ; 35(7): 717-720, jul. 2009. ilus
Article in English, Portuguese | LILACS | ID: lil-521401

ABSTRACT

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.


A doença de Rosai-Dorfman (DRD) consiste em histiocitose sinusal com linfadenopatia maciça. O envolvimento extranodal ocorre em até 43% dos casos. Entretanto, a DRD de partes moles de forma isolada é rara. A forma isolada de DRD mediastinal é muito rara, com apenas três relatos prévios. O envolvimento do mediastino posterior só foi descrito no contexto da DRD disseminada. Relatamos o caso de uma paciente de 49 anos de idade com história de dor e linfadenomegalia cervical há dois anos, com resolução espontânea. A TC revelou uma massa paravertebral à esquerda medindo 6 cm de diâmetro. A paciente foi submetida à ressecção cirúrgica da massa. O exame microscópico e estudo do imunofenótipo da peça cirúrgica definiram o diagnóstico de DRD. A paciente foi acompanhada por 12 meses, queixando-se de tosse discreta e dor torácica. O acompanhamento por imagem não detectou recorrência, e nenhuma linfadenomegalia cervical foi observada após a cirurgia.


Subject(s)
Female , Humans , Middle Aged , Histiocytosis, Sinus/pathology , Mediastinal Diseases/pathology
14.
An. bras. dermatol ; 84(3): 275-278, jul. 2009. ilus
Article in English, Portuguese | LILACS | ID: lil-521753

ABSTRACT

Doença de Rosai-Dorfman é uma doença benigna e autolimitada. Tem etiologia desconhecida e foi descrita pela primeira vez por Rosai e Dorfman em 1969. As manifestações clássicas incluem linfadenopatia cervical usualmente acompanhada por febre, leucocitose com neutrofilia e hipergamaglobulinemia policlonal. Apesar de a pele ser o principal sítio extranodal da doença, sua manifestação restrita à pele costuma ser bastante rara com poucos casos descritos na literatura. O trabalho tem como objetivo relatar um caso de Doença de Rosai-Dorfman cutânea com revisão da literatura onde são abordados os diferentes aspectos do diagnóstico e do tratamento dessa doença.


Rosai-Dorfman disease is a self-limited benign disease. Rosai and Dorfman first described it in 1969, and the etiology of the disease remains unknown. Main manifestations are cervical adenopathy associated with fever, leukocytosis with neutrophilia and polyclonal gamaglobulinemia. Although the skin is the most common site of extra nodal disease, Rosai-Dorfman disease restricted to the skin is very rare with only a few cases described in the literature. This paper reports a rare case of cutaneous Rosai-Dorfman with skin as the sole site, and reviews the controversies of diagnosis and treatment.


Subject(s)
Adult , Humans , Male , Histiocytosis, Sinus , Histiocytosis, Sinus/pathology , Histiocytosis, Sinus/surgery , Skin Diseases/pathology , Skin Diseases/surgery
15.
APMC-Annals of Punjab Medical College. 2008; 2 (1): 65-68
in English | IMEMR | ID: emr-108395
16.
Indian J Pathol Microbiol ; 2006 Oct; 49(4): 509-15
Article in English | IMSEAR | ID: sea-74786

ABSTRACT

Sinus histiocytosis with massive lymphadenopathy (SHML) also called as Rosai Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology. Histological features currently define it. Persistent painless lymphadenopathy due to expansion of sinuses infiltrated with benign histiocytes and plasma cells and emperipolesis are the characteristic features of SHML. Our study includes seven cases (5 nodal and 2 extranodal) of SHML over a 5-year period whose slides and blocks were reviewed. IHC was performed on the main lesion, from a panel of S100, CD68, LCA, CD20, CD3, CD30, CD43, bcl2, cytokeratin and epithelial membrane antigen. In our series we have work up available in 7 cases out of which a detailed follow-up is available in 5 patients. Out of these 5 patients, 4 have a stable disease, while one developed histiocytic sarcoma after a gap of four years.


Subject(s)
Adolescent , Adult , Aged , Female , Histiocytes/pathology , Histiocytosis, Sinus/pathology , Humans , Lymph Nodes/pathology , Lymphatic Diseases/pathology , Male , Middle Aged , Phagocytosis
17.
Article in English | IMSEAR | ID: sea-74344

ABSTRACT

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Both dendritic cell and monocyte-macrophage lineage have been considered as the origin of the histiocytes that are classical of the disorder. We analyzed seven cases of Rosai-Dorfman disease to determine the immunophenotypic profile of these cells. Immunohistochemical analysis was undertaken by using: (a) fascin as a novel mature dendritic cell marker; (b) S-100 protein; (c) CD68 (both KP1 and PGM1) as macrophage related markers; (d) CD79a and (e) CD3. The Rosai-Dorfman histiocytes exhibited strong cytoplasmic staining for fascin in all the cases and demonstratedformation of a meshwork of fine dendritic processes emanating from the cell cytoplasm. S-100 protein was uniformly expressed in all the cases. Expression of both KP1 and PGM1 epitopes of CD68 was found to be weak and variable. The phagocytosed lymphocytes were composed of an admixture of both B and T-lymphocytes. The characteristic expression pattern of the histiocytes for fascin along with co-expression of S-100 protein and a consistently weaker and variable expression of macrophage-lineage markers point more towards a dendritic cell ontogeny of these cells. Further, abundant fascin expression and presence of dendritic processes indicate a differentiated or mature dendritic cell phenotype for these cells.


Subject(s)
Adolescent , Adult , Carrier Proteins/metabolism , Cell Differentiation , Cell Lineage , Child , Child, Preschool , Dendritic Cells/cytology , Female , Histiocytosis, Sinus/pathology , Humans , Immunohistochemistry , Immunophenotyping , Lymphatic Diseases/pathology , Male , Microfilament Proteins/metabolism , S100 Proteins/metabolism
18.
Neurol India ; 2005 Mar; 53(1): 110-1
Article in English | IMSEAR | ID: sea-120761

ABSTRACT

A 40-year-old male presented with a single generalized tonic-clonic seizure. MRI revealed an enhancing, dural-based, left lateral sphenoid wing lesion suggestive of a meningioma. At microsurgical excision, the lesion was firm and relatively avascular. The histopathological report revealed S-100 positive histiocytic proliferation with lymphophagocytosis (emperipolesis) characteristic of the Rosai-Dorfman disease. The case and its management are discussed.


Subject(s)
Adult , Diagnosis, Differential , Histiocytosis, Sinus/pathology , Humans , Magnetic Resonance Imaging , Male , Meningeal Neoplasms/pathology , Meningioma/pathology , Sphenoid Bone
19.
J Indian Med Assoc ; 2003 Jan; 101(1): 28-30
Article in English | IMSEAR | ID: sea-96601

ABSTRACT

A two and half years old male child of sinus histiocytosis with massive lymphadenopathy, paraplegia and spinal cord involvement was treated with surgery and radiotherapy for the spinal cord compression and later with chemotherapy for his nodal disease in the neck. There was a significant improvement in his neurologic status as well as in his nodal status reiterating the role of combination therapy in this disease.


Subject(s)
Child, Preschool , Histiocytosis, Sinus/pathology , Humans , Magnetic Resonance Imaging , Male , Spinal Cord Compression/pathology
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