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)

Two cases of Rosai-Dorfman disease with polypoid nasal infiltration mimicking nasal tuberculosis and malignant lymphoma are reported. This rare benign disease was first described by Rosai and Dorfman in 1969 and is characterized by histiocytic proliferation. It is seldom considered in the differential diagnosis of granulomatous diseases due to its rarity and histological similarity to other diseases. Extranodal manifestations of this disease are uncommon. Although no specific treatment can guarantee a sustained remission of this disease, surgery for loco-regional lesions can result in long-term symptomatic control and restoration of function. Both patients underwent endoscopic resection of the nasal polypoid lesions and have subsequently been free of recurrence. Loco-regional infiltration of the nasal cavity by Rosai-Dorfman disease is effectively managed by endoscopic resection.
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PMID:Nasal manifestation of extranodal Rosai-Dorfman disease--diagnosis and management. 1043 44

A 47 year old Chinese man with diabetes mellitus and previously treated pulmonary tuberculosis presented with painless right testicular enlargement of 1 month's duration. He underwent an orchidectomy for presumed testicular neoplasm corroborated clinicoradiologically. Histological examination of the testicular mass revealed an inflammatory lesion comprising lymphocytes, plasma cells and sheets of pale staining histiocytes, some containing lymphocytes within their ample cytoplasm, suggestive of emperipolesis. S100 immunohistochemistry stained the histiocytes, while ultrastructural examination confirmed emperipolesis. A diagnosis of Rosai-Dorfman disease was made, an exceedingly rare testicular lesion. Clinicoradiological findings mimicked a neoplasm, while the inflammatory histological appearances with occasionally discerned multinucleated cells raised the possibility of xanthogranulomatous orchitis. Tuberculous orchitis was excluded through negative Ziehl-Neelsen stains and PCR for mycobacterium, while seminoma, which sometimes features a predominant inflammatory component obscuring neoplastic cells, was excluded by absent immunostaining for placental alkaline phosphatase and CD117.
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PMID:Rosai-Dorfman disease of the testis: an unusual entity that mimics testicular malignancy. 1650 87

The use of positron emission tomography (PET) scans is rapidly evolving in the diagnosis, treatment, and surveillance of lymphoma. However, significant challenges exist in differentiating lymphomatous from benign lesions. Herein we describe the clinical and radiographic uptake patterns seen in common clinical mimics of lymphoma, including infections such as human immunodeficiency virus and tuberculosis, inflammatory disorders such as sarcoidosis and connective tissue disorders, and other benign lymphoproliferative disorders such as Kikuchi's disease and Rosai-Dorfman disease. The clinical utility of PET scans has encompassed varying fields, not just oncology. Future challenges with the use of improved radiopharmaceuticals in accurately defining and differentiating lesions that would affect clinical treatment would be of paramount importance.
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PMID:Positron emission tomography findings in clinical mimics of lymphoma. 2171 19

Meningioma is the most common mass involving the dura, making it number one in the differential diagnosis for any dural-based mass; however, a variety of other neoplastic and nonneoplastic lesions also involve the dura. Knowledge of the dural anatomy can provide clues to the various processes that may involve this location. The neoplastic processes include both benign and malignant lesions such as hemangiopericytoma, lymphoma, solitary fibrous tumor, melanocytic lesions, Epstein-Barr virus-associated smooth muscle tumors, Rosai-Dorfman disease, and metastatic lesions. The nonneoplastic processes include infectious and inflammatory entities such as tuberculosis and sarcoid, which may mimic mass lesions. In some cases, neoplasms such as gliosarcoma may arise peripherally from the brain parenchyma, appearing dural-based and even inciting a dural tail. Many of these share similar computed tomographic, magnetic resonance imaging, and angiographic characteristics with meningiomas, such as a dural tail, increased vascularity, avid enhancement, and similar signal characteristics; however, knowledge of the patient's age, gender, and underlying conditions and certain imaging characteristics may provide valuable clues to recognizing these lesions. For example, in the population with human immunodeficiency virus infection, Epstein-Barr virus-associated smooth muscle tumors should be included in the differential diagnosis for dural-based lesions. The surgical course and prognosis for these lesions vary, and knowledge of the variety of lesions that involve the dura, their imaging appearances, and their clinical features assists in narrowing the radiologic differential diagnosis and optimizing patient treatment.
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PMID:From the radiologic pathology archives: mass lesions of the dura: beyond meningioma-radiologic-pathologic correlation. 2461 80

Sinus histiocytosis with massive lymphadenopathy (SHML), Rosai-Dorfman Disease, is a rare histiocytic syndrome first described by Rosai and Dorfman, most frequently seen in children and young adults.The disease is more common in males and in individuals of African descent but rare in Asians. It is mainly characterized by painless bilateral cervical lymph node enlargement and is often associated with fever and leucocytosis. This case is being reported for its rarity in presentation in an elderly female with both generalized nodal as well as extranodal manifestations. Without the awareness about RDD, the diagnosis of RDD is unexpected especially in South East Asian Countries where certain lymphadenopathies such as tuberculosis, metastatic malignancies and lymphomas are common.
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PMID:A Rare Case of Multiorgan Involvement of Rosai Dorfman Disease in an Elderly Female. 2698 75

Patients with cirrhosis of the liver usually present with a small shrunken liver and a large spleen. The presence of an unusually huge liver should prompt the treating doctor to look for another cause, as this may be treatable and improve the patient's outcome. In South Africa tuberculosis and lymphoma in the presence of HIV infection should be excluded. Sinus histiocytosis with massive lymphadenopathy and cirrhosis is a rare combination and has not been reported before in the literature. This case is intended to make clinicians aware of this rare combination of diseases.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and cirrhosis of the liver: A case report and literature review. 2713 63

Sinus histiocytosis with massive lymphadenopathy also known as Rosai-Dorfman disease is a rare benign disease that typically manifests as lymphadenopathy with or without systemic manifestations whose etiology remains poorly understood. Most common clinical presentation is painless bilateral cervical lymphadenopathy. However, it also can present in various extranodal sites and can easily be missed because of its rarity if not considered in the differential diagnosis. It commonly occurs in children and young adults with a slightly male predominance. Clinically patients may be mistaken for lymphoma and other infectious disorders like tuberculosis especially in developing countries like in Pakistan where tuberculosis is more prevalent. Here we report a case of a 38-years-old gentleman with bilateral cervical lymphadenopathy and worsening ascites. His symptoms initially mimicking tuberculosis and lymphoma, was finally diagnosed as having Rosai-Dorfman's Disease on intra abdominal lymph node biopsy.
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PMID:Rosai-Dorfman's disease, an uncommon cause of common clinical presentation. 3143 83

Although MRI has a spectrum of findings which help in the diagnosis of tuberculosis (TB) spine, a broad spectrum of spine pathologies resemble Pott's spine on MRI and are often missed due to inadequate clinical details. As a result, patients are often subject to unnecessary biopsy. A blinded radiologist may misdiagnose such mimic cases as TB. Our aim is to enable the reader to learn the main criteria that differentiate spine TB from other spine etiologies that mimic TB. A retrospective search was done and authors collected only MRI spine reports that showed a differential diagnosis or diagnosis of TB spine from the computer-based data records of the institution over a four-year period. This revealed 306 cases of TB spine out of which 78 cases with an alternate diagnosis that resembled TB spine were included. We describe a single institute review of 78 such cases that resemble and mimic Pott's spine on MRI. The cases being: (n = 15) pyogenic spondylitis, (n = 1) brucellar spondylodiscitis, (n = 12) rheumatoid arthritis, (n = 12) metastases, (n = 8) lymphoma, (n = 5) post-trauma fractures, (n = 10) degenerative disc disease, (n = 2) Baastrup's disease, (n = 9) osteoporotic fracture, (n = 3) spinal neuropathic arthritis, and (n = 1) case of Rosai-Dorfman disease. The clinical and radiological findings of all these cases were correlated with lab findings and histopathology wherever necessary. Appropriate recognition of these entities that resemble and mimic TB spine on MRI is important for optimal patient care. This paper exposes radiologists to a variety of spine pathologies for which biopsy is not indicated, and highlights key imaging findings of these entities to facilitate greater diagnostic accuracy in clinical practice.
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PMID:An Institutional Review of Tuberculosis Spine Mimics on MR Imaging: Cases of Mistaken Identity. 3185 25

A 36-year-old female presented with pain and progressive decrease in the range of movements in the left elbow. There was contact with an open case of pulmonary tuberculosis. There were no constitutional symptoms except fever. X-ray showed periarticular osteopenia with destruction of the radial neck and head. MRI scan findings were also consistent with tuberculosis of the elbow. Histopathology examination confirmed the diagnosis of Rosai-Dorfman disease. The patient was treated with glucocorticoid and methotrexate and was asymptomatic after 18 months.
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PMID:Primary Rosai-Dorfman Disease of the Proximal Radius Masquerading as Tuberculosis of Elbow. 3248 8