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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disease of the lymph nodes, still of unknown origin. We are reporting the case of a 16 year old boy with SHML which occurred in 1983. Investigations showed a massive lymphadenopathy of the mediastinal and abdominal nodes, causing displacement and compression of surrounding tissue. The patient further developed a blockage of the vena cava superior and inferior, leading to numerous collateral circulation routes in the upper and lower extremities. The etiology of the venous blockage is still disputed. It is possible that they are the result of compression of the major veins. Alternatively, the cause could lie in the disruption of the coagulation system. Finally and more likely, the problem could be the result of fibrosis developing through the healing process.
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PMID:[Sinus histiocytosis with massive lymphadenopathy with complete occlusion of the superior and inferior vena cava]. 912 Oct 77

We report the cytologic findings of a case of Rosai-Dorfman disease of the breast in a 52-year-old diabetic woman, initially sampled by fine-needle aspiration biopsy (FNA). The patient presented with a 2-week history of a 3 x 2 cm nodule in the mid-upper area of the left breast. A mammogram taken 6 months prior was negative. FNA smears demonstrated lymphocytes, plasma cells, and large pale cells, with enlarged irregular nuclei, admixed with fragments of fibrous tissue and calcific debris. Lymphophagocytosis (emperipolesis) was scarce. Our diagnosis was atypical lymphohistiocytic proliferation. Flow cytometry was negative, but in the face of a strong clinical suspicion of a lymphoid malignancy, excision was performed. The final diagnosis was Rosai-Dorfman disease (RDD). The differential diagnosis of FNA of breast inflammatory lesions with atypical cytology is discussed, with a review of the literature. The early recognition on FNA of the hallmarks of this rare disease should prevent unnecessary radical surgery. Diagn. Cytopathol. 1999;21:287-291.
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PMID:Fine-needle aspiration cytology of Rosai-Dorfman disease of the breast: A case report. 1049 25

Rosai-Dorfman disease is a rare disease characterized histologically by proliferation of histiocytes and has clinical features suggestive of a lymphomalike disease. Lymph nodes and extranodal sites might be involved, but renal involvement is rare. We present computed tomographic findings in three cases of renal involvement by Rosai-Dorfman disease. Two cases showed renal hilar masses and one case showed subcapsular hypodense infiltration. Renal involvement by Rosai-Dorfman disease has a characteristic appearance and should be included in the differential diagnosis of renal hilar masses or subcapsular hypodense infiltration.
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PMID:Renal involvement by Rosai-Dorfman disease: CT findings. 1184 83

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare disease characterised by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal involvement. Orbital involvement occurs in 11% of cases and onset of the disease is typically in the first two decades of life. A benign course is usual, but in some cases blindness or fatality may result. We present a case with late onset at age 78 years and lacrimal gland involvement, and review the literature on this disease.
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PMID:A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient. 1202 74

We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.
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PMID:PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. 1456 72

Sinus histocytosis with massive lymphadenopathy is a rare disease that has been described by Rosai and Dorfman. It is characterized by massive, cervical lymphadenopathy, with extranodal manifestations in about 40% of patients. It occurs as a distinct entity, never associated with other diseases, and in most cases the prognosis is good. Lymphadenopathy is also a frequent sign of patients with primary Sjogren's syndrome (SS), usually associated with disease activity or concurrent infection. However, excessive lymphadenopathy in SS patients is a sign of lymphoproliferative disorder development. In this report, we describe a patient with primary SS, and excessive lymphadenopathy and splenomegaly who developed Rosai-Dorfman disease, and we discuss the possible aetiopathophysiological mechanism linking these two entities.
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PMID:Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sjogren's syndrome. 1516 14

We present a unique case of a man presenting with an abnormal prostate and large renal mass who was ultimately found to have prostate cancer and renal Rosai-Dorfman disease. We discuss the method of diagnosis and treatment in this patient, as well as review the presentation, diagnosis, and treatment of this rare disease.
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PMID:Rosai-Dorfman disease presenting as extranodal renal mass. 1636 Apr 69

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy (SHML) is a histiocytic proliferative disorder that typically involves the cervical lymph nodes with or without extranodal involvement of the skin, soft tissues, respiratory tract, or virtually any other site of the body. We present a case report of SHML involving the cervical lymph nodes, parotid gland and breast. There is only one other Rosai-Dorfman registered case that involves both breast and parotid disease and no previously published case reports. The patient presented atypically with breast masses found on screening mammogram rather than massive cervical lymphadenopathy. We describe the subsequent radiographic and surgical pathologic evidence that led to the diagnosis of this rare disease.
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PMID:Rosai-Dorfman disease of the breast and parotid gland. 1866 7

Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes in the lymph nodes with or without extranodal involvement. RDD limited to the skin without nodal involvement, known as cutaneous Rosai-Dorfman disease, is very rare. We describe a 34-year-old female with RDD of the skin over the chest. A large nodule with satellite lesions was excised for histopathological examination. Microscopically, there were many large histiocytes (Rosai-Dorfman cells) exhibiting emperipolesis, among many plasma cells, lymphocytes and neutrophils throughout the dermis and the subcutaneous tissue. The histiocytes were immunohistochemically positive for S-100 protein but negative for CD 1a. Physical examination showed no lymphadenopathy or any extra-cutaneous lesions. Serological tests indicated a past infection with Epstein-Barr virus and cytomegalovirus. The diagnosis of cutaneous RDD may be difficult in the absence of associated lymphadenopathy or any specific features of the skin lesion. Hence, not only is histopathological examination required for definitive diagnosis but a high index of suspicion by the clinicians and pathologists is essential to help diagnose this very rare disease.
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PMID:Cutaneous Rosai-Dorfman disease. 1910 29

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease. A third of patients with this disease have extranodal involvement affecting the skin. Of these individuals, only around 3% will have purely cutaneous Rosai-Dorfman disease, which is limited to skin manifestations without systemic involvement. Cutaneous (localized) scleroderma or morphea, on the other hand, is a more common disease that most often affects women of all ages. Both conditions have unknown etiologies. Presented here is a case of a 60-year-old white woman with cutaneous Rosai-Dorfman disease and coexisting morphea. Representative biopsies from both areas were performed: one showing a dermal S-100+ histiocytic infiltrate with emperipolesis and the other showing a deep perivascular and interstitial plasma cell infiltrate with dermal sclerosis and loss of perieccrine fat. A laboratory and radiologic workup revealed no evidence of systemic involvement by either entity. The diagnosis of coexisting cutaneous Rosai-Dorfman disease and morphea was established. To our knowledge, this is the first report of these 2 entities found simultaneously in 1 patient.
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PMID:Cutaneous rosai-dorfman disease and morphea: coincidence or association? 1954 28


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