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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 may involve extra-nodal tissue sites. Skeletal involvement is uncommon and has always been associated with disease in other nonosseous locations. The present case is the first to document sinus histiocytosis with massive lymphadenopathy arising primarily within bone and unaccompanied by disease elsewhere.
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PMID:Osseous pseudotumor. The sole manifestation of sinus histiocytosis with massive lymphadenopathy. 403 85

Sinus histiocytosis with massive lymphadenopathy (SHML) is generally regarded as a benign disorder in spite of its propensity to form large masses and to disseminate to both nodal and extranodal sites. Although in most patients the extent of SHML does not appear to determine disease outcome, recent reports have documented that infiltrates of SHML can cause death. To gain perspective on the role of SHML in patient deaths in this population, the authors analyzed the 14 known fatalities in an SHML registry comprising 215 patients. The average age at death was 33 years. Autopsy was performed on eight patients. SHML infiltrates were clearly the cause of death in only two patients (previously reported). In another four cases persistent SHML was prominent at death. The other deaths were the result of complications of defined immunologic abnormalities (five), or of unusual infections (three). The authors have not seen an example of cytologic malignant transformation of SHML.
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PMID:Sinus histiocytosis with massive lymphadenopathy. An analysis of 14 deaths occurring in a patient registry. 647 18

Rosai-Dorfman disease is rare. Two patients, one with nodal and another with extra-nodal disease, are presented. The clinical and pathological features of the two cases are typical of the disease. Because the disease can present with a diverse picture, it can be easily misdiagnosed. It is therefore suggested that physicians and paediatricians should exercise more vigilance in order to detect more cases, particularly in the early stages, for better understanding of the natural history of this disease about which little is known at this stage.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in Liberia. 789 9

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a benign, self-limited pseudolymphomatous entity with characteristic histologic features. The disease may be atypically present in the absence of detectable nodal involvement. This is a case of nasal septum involvement of Rosai-Dorfman disease without clinically apparent lymphadenopathy.
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PMID:Rosai-Dorfman disease involving the nasal septum. 855 83

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare entity characterized by the proliferation of cells belonging to the macrophage-histiocyte family, but whose exact origin is unknown. A case of SHML occurring in a 62-yr-old female is described in which the diagnosis is initially suggested by fine-needle aspiration (FNA) of a cervical node. The smears show a background of lymphocytes and plasma cells and large histiocytes with well-preserved lymphocytes in their cytoplasm (emperipolesis or lymphophagocytosis). Both nodal and extranodal (nasal mass) involvement are confirmed by surgical biopsy. The immunohistochemistry suggests that SHML cells are functionally activated macrophages. Eight months after diagnosis, the patient is clinically well, with partial improvement of lymphadenopathy.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): diagnosis with fine-needle aspiration in a case with nodal and nasal involvement. 909 55

Cutaneous lesions of Rosai-Dorfman disease (RDD) are usually associated with nodal or other extranodal localization. We describe a female patient with RDD clinically limited to the skin. The patient presented with asymptomatic red-brown papules and nodules on the legs, arms, back, and nose. Histologically, the lesions consisted of a proliferation of large histiocytes occasionally showing emperipolesis. Histiocytes were also observed within dilated lymphatic vessels. Immunohistochemical study showed that histiocytes expressed S-100 protein and both macrophage and monocyte markers. All lesions resolved completely with Roentgen therapy. No recurrence has been observed over a 3-year follow-up period.
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PMID:Purely cutaneous Rosai--Dorfman disease. 873 85

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a nonneoplastic lymphoproliferative disorder that usually presents with massive painless cervical lymphadenopathy accompanied by fever and weight loss. Extranodal involvement occurs in over 40% of patients; however, isolated extranodal disease without lymph node involvement is unusual. Extranodal involvement of the nervous system is unusual and isolated central nervous system disease in the absence of nodal disease is distinctly rare. The author describes the case of a 35-year-old man who presented with paraplegia and an enhancing intramedullary lesion on magnetic resonance imaging that was suggestive of a primary spinal cord tumor. The lesion was completely resected and histologically proved to be consistent with an isolated extranodal case of Rosai-Dorfman disease. Although a number of cases of this entity involving the CNS have been published, the author believes this case represents the first report of intramedullary spinal cord involvement as the initial and only sign of disease.
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PMID:Isolated extranodal sinus histiocytosis presenting as an intramedullary spinal cord tumor with paraplegia. Case report. 881 78

Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.
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PMID:Isolated intracranial Rosai-Dorfman disease: case report and literature review. 970 47

Sinus histiocytosis with massive lymphadenopathy (SHML) (Rosai-Dorfman disease) is a rare self-limited histiocytic disorder of unknown origin, usually present with cervical lymphadenopathy, fever, elevated ESR and haematologic abnormalities. Extranodal lesions may occur as initial manifestations of the disease. In most cases skin lesions are associated with nodal or extranodal localization. A 54-year-old male is reported who, for a period of ten months, presented numerous slowly enlarging, rounded papular lesions on the face, neck, trunk as well as severe cervical lymphadenopathy without general and haematological disturbances. Thereafter a fungating mass developed in the nasal cavity. Histological, histochemical and immunohistochemical tests of skin, mucosal and lymph-node lesions were consistent with a diagnosis of SHML. The immunohistochemical characterization of the cytophagocytic S-100+ macrophages revealed that they were highly positive for the adhesion molecules Vitronectin receptor av/b3, which is used by tissue macrophages for removal of effete cells through phagocytosis.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): case report with nodal and diffuse muco-cutaneous involvement. 987 Jun 77

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is primarily a nodal-based, idiopathic, benign proliferative disorder of histiocytes with 43% of these cases also involving extranodal sites. The breast is an unusual site of occurrence of RDD. We report two cases of this exceptional event. The first represents an intramammary nodal Rosai-Dorfman disease, while the second is an extranodal disease with sole involvement of the breast. In both, the possibility of malignancy was raised. Histological examination of the two breast lesions revealed sheets of characteristic large histiocytes displaying emperipolesis, a microscopic hallmark of this disease. Immunohistochemical and ultrastructural studies were also performed; the former showed cytoplasmic staining of histiocytes for S-100 protein, while histiocytes that engulfed lymphocytes and plasma cells were identified on electron microscopy.
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PMID:Rosai-Dorfman disease of the breast: a mimic of breast malignancy. 1074 Jul 98


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