Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rosai-Dorfman disease is a rare, idiopathic, benign histiocytic proliferation usually seen in younger patients. Massive lymphadenopathy most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes. Nearly half of the patients will have extranodal involvement, 75% occurring in sites in the head and neck. Three cases of extranodal Rosai-Dorfman disease of the head and neck involving the nose, paranasal sinuses, and parotid gland are presented. The clinical presentation, histologic characteristics, radiographic findings, and treatment of the disease are discussed. Because of the scarcity of cases, the clinical and histopathologic features of this disease may be overlooked. Familiarity with its relatively frequent clinical manifestations in the head and neck, as well as with the diagnostic histopathology, should preclude confusion with other disease entities.
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PMID:Extranodal Rosai-Dorfman disease of the head and neck. 861 84

Rosai-Dorfman disease (RDD) rarely presents a diagnostic challenge. However, unusual sites of presentation can cause confusion. We present a case of RDD of the colon in a symptomatic 51-year-old woman. The gastrointestinal tract is the organ system least commonly affected by RDD, with only 11 cases reported. Most cases have been found incidentally or at autopsy. The widespread use of endoscopic procedures makes it more likely that this entity will be encountered. Most patients with gastrointestinal tract involvement have evidence of widespread extranodal disease and have a protracted clinical course.
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PMID:Rosai-Dorfman disease presenting in the gastrointestinal tract. 1256 57

Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman Disease, presents with bilateral painless cervical adenomegaly, fever, and several hematologic abnormalities. Skin is the extranodal site most frequently affected. We described four additional cases of SHML limited to the skin, emphasizing possible confusion with other dermatoses. Clinical, morphologic, and immunohistochemical aspects of four patients with cutaneous SHLM, three diagnosed in the ABC Medical Center in Mexico City and the other at the Hermanos Ameijeiras Hospital of Havana, Cuba, are reviewed. Three males and one female, 48, 35, 42, and 55 years of age, presented with chronic asymptomatic dermal nodules, papules, or plaques on trunk, extremities, and face. Skin biopsies showed dense infiltrates of foamy histiocytes, lymphocytes, and plasma cells; histiocytes presented with prominent emperipolesis and intense S100 and CD68 immunostain. The four cases reported here had histiocytic benign proliferative disorder corresponding with cutaneous SHML: Emperipolesis suggested the diagnosis. Immunohistochemistry demonstrated positivity for S100 protein in macrophages. Cutaneous lesions of SHML are easily recognized when they are found in the classical clinical picture, but as purely skin disease could be confused with other dermatoses.
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PMID:[Rosai-Dorfman disease limited to the skin. Four case reports]. 1266 3

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a systemic disease involving nodal and extranodal tissues. We report a 48-year-old female with recurrent nasal obstruction due to polypoidal masses involving the nasal sinuses, turbinates and septum bilaterally, and lumps in the right infra-orbital region and region of the right lacrimal sac. A 4 cm right upper neck mass was also noted, which was initially diagnosed as histiocytic lymphoma. Histopathology of the nasal and infraorbital lesions revealed fibro-inflammatory masses containing histiocytic cells with large vesicular nuclei and abundant foamy cytoplasm exhibiting emperipolesis and lymphophagocytosis, admixed with scattered plasma cells and lymphocytes. These histiocytes revealed immunohistochemical positivity for S-100 protein and CD68, but were negative for CDla. The findings supported a diagnosis of RDD. This report serves to remind pathologists and clinicians of the extranodal manifestations of RDD and its potential confusion with lymphomas.
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PMID:Multifocal nodal and extranodal Rosai-Dorfman disease initially diagnosed as histiocytic lymphoma. 1910 14

Rosai-Dorfman disease (RDD) is an uncommon histiocytic disease of unknown etiology. It typically presents as massive lymphadenopathy with a predilection for the cervical lymph nodes of children and young adults. However, extranodal involvement is not uncommon and may cause confusion with other neoplasms or reactive disease. We describe here a unique case of extranodal RDD manifesting as a pericardial mass in a 69-year-old man. The lesion was detected by computed tomography during a periodic examination of the chest. Subsequently positron emission tomography scan showed mild increase of flurodeoxyglucose uptake. Clinically, it was supposed to be a mesothelioma. Histological examination showed the typical features of RDD confirmed by the staining of S100 protein, which highlighted the emperipolesis of the characteristic histiocytes. To the best of our knowledge, pericardial RDD represents an extremely rare condition and should be included in the differential diagnosis of pericardial neoplasms.
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PMID:Rosai-Dorfman disease of the pericardium: a case report and review of literature. 2503 68