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)

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare disease that typically occurs in lymph nodes. While many body sites have been reported to be involved in extranodal manifestations of the disease, the cardiovascular system has been largely absent in this literature. We report two cases of Rosai-Dorfman disease that involves the heart. Case 1 was a 40-year-old man with chronic myelomonocytic leukemia who was incidentally noted to have Rosai-Dorfman disease on autopsy after succumbing to respiratory failure in the setting of adult respiratory distress syndrome. Case 2 was a 57-year-old woman known to have Rosai-Dorfman disease involving mediastinal lymph nodes and found to have a right atrial mass on workup for atypical chest pain. Both cases showed a similar histologic picture of large, multinucleated histiocytes with immunoreactivity to S100, emperipolesis, and marked plamacytosis. This study expands our knowledge of organs involved in extranodal Rosai-Dorfman disease.
Cardiovasc Pathol
PMID:Extranodal Rosai-Dorfman disease involving the heart: report of two cases. 1981 34

Rosai-Dorfman disease is rare and typically presents with cervical lymphadenopathy. The disease is generally indolent and self-limited, but it carries a poor or fatal prognosis when it is advanced or when it involves and compresses vital structures. We herein report a rare case of Rosai-Dorfman disease affecting only the mediastinal-hilar region in a 66-year-old woman.
Gen Thorac Cardiovasc Surg 2011 May
PMID:Rosai-Dorfman disease mimicking mediastinal lymphoma. 2154 40

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a nonmalignant disease of unknown etiology. It may present as bilateral, massive, painless cervical lymphadenopathy; however, one third of the cases have extranodal involvement. Extranodal sites include soft tissue, skin, upper respiratory and gastrointestinal tracts, and central nervous system, with head and neck being the most common. Here we report a rare case of intracardiac RDD in a 46-year-old, African-American female who presented with worsening shortness of breath and fatigue for 7 weeks. Comprehensive physical examination was within normal limits; the patient had no lymphadenopathy. Echocardiogram revealed a large mass in the left atrium extending into the mitral valve. The mass was resected under the pretext of clinical diagnosis of myxoma, and the patient underwent a radical extended interatrial septectomy with bovine pericardial patch reconstruction. The 8-cm resected mass consisted of areas of fibrosis alternating with sheets of histiocytes enmeshed in a mixed inflammatory infiltrate. Histiocytes contained intact lymphocytes, plasma cells, neutrophils, and erythrocytes suggesting emperipolesis. The diagnosis of RDD was confirmed by immunohistochemistry. This case highlights the extranodal manifestation of RDD in the heart which could be easily mistaken for a myxoma on clinical and radiologic evaluation. RDD must be considered in the differential diagnosis of any patient who presents with a cardiac mass.
Cardiovasc Pathol
PMID:Extranodal Rosai-Dorfman disease arising in the heart: clinical course and review of literature. 2879 81

Rosai-Dorfman disease or sinus histiocytosis is a nonmalignant disease chiefly presenting as a painless cervical lymphadenopathy, although extranodal involvement is also frequent. Involvement of the tracheobronchial tree is extremely rare, and there are no clear guidelines on management. Rosai-Dorfman disease usually presents as a tracheal tumor, although it may also present with extraluminal compression. Only 12 cases have been reported in the English language literature. Histopathology is required to confirm the diagnosis. We describe what we think is India's first reported case of histologically proven tracheal Rosai-Dorfman disease in a 40-year-old otherwise healthy lady.
Asian Cardiovasc Thorac Ann 2019 Feb
PMID:Rosai-Dorfman disease of the trachea: an extremely rare benign tumor. 3050 36