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 (RDD) is rare and characterized by histiocytic proliferation and massive cervical lymphadenopathy. About 40% of patients have extra-nodal involvement. Opthalmic involvement is seen in 10% of cases. A case of orbital Rosai Dorfman disease in a 58 years old woman is presented here, who was misdiagnosed as orbital inflammatory disease initially. The patient did not respond to a course of oral prednisolone. Then complete surgical excision of the mass was performed and the histopathological examination was consistent with a diagnosis of RDD.
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PMID:Orbital Rosai-Dorfman Disease in a fifty-eight years old woman. 2435 90

Rosai-Dorfman disease (RDD) is also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML). It is a rare, benign, self-limiting disease of phagocytic histiocytes affecting a young age group presenting with massive painless cervical lymphadenopathy. RDD has nodal and also extra-nodal involvement with episodes of exacerbation and remissions, whose aetiology remains poorly elucidated. It is highly variable in its clinical presentation and response to treatment. Its treatment is poorly defined but the prognosis is usually favourable. Here we are reporting a rare, unusual clinical presentation of cervical lymphadenopathy and nasal mass diagnosed as RDD with cyto-histopathological correlation. Only a few such cases have been reported in the literature. We also emphasise that clinicians and pathologists should always be aware of RDD in making a differential diagnosis of cervical lymphadenopathy.
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PMID:Rosai-Dorfman disease: A rare clinico-pathological presentation. 2461 Oct 75

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a reactive condition of unknown etiology, characterised by a proliferation of histiocytes exhibiting emperipolesis of lymphocytes and plasma cells. It usually presents as bilateral painless cervical lymphadenopathy. Extranodal RDD without nodal disease is seen in 23% of the cases. Intracranial RDD occurs in less than 5% of the total number of patients with extranodal disease. Isolated intracranial RDD in the absence of nodal disease is exceptional. We report a case of Rosai-Dorfman disease with isolated intracranial involvement. A 38-year-old man came with long-standing headache and the magnetic resonance imaging (MRI) scan showed features of meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive but CD1a and epithelial membrane antigen negative.
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PMID:Isolated intracranial rosai-dorfman disease involving the meninges: report of a rare case. 2564 May 69

Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, and subjacent muscle group, accompanied by obvious lymph node enlargement in the left part of the neck, supraclavicular fossa, and axillary fossa. The histopathology of the left cervical lymph node revealed diffuse effacement of the normal nodal architecture, with patchy chronic inflammatory cell infiltrates comprising lymphocytes and sheets of histiocytes. Some histiocytes contained lymphocytes within their pale cytoplasm. Many multinucleated giant cells were found; however, caseating granulomas were not seen. The skin and muscle biopsy specimen obtained from the back revealed infiltrating lymphocytes and histiocytes diffusely distributed in the dermis, subcutaneous tissue, and crevices of the muscle fibers. The phenomenon of emperipolesis and the presence of multinucleated giant cells were also seen. Immunohistochemical staining revealed that the histiocytes were positive for S-100 protein and CD68 but negative for CD1a. Immunophenotyping of the infiltrating lymphocytes indicated positive reactions to CD3, CD45RO, CD5, CD7, CD4, CD8 (partly), CD79a, CD20 (partly), and Ki-67 (<1%). The final diagnosis was Rosai-Dorfman disease. Owing to the extensive and deep involvement of the subcutaneous tissue and muscles, the patient did not undergo surgery to excise the massive skin nodule. The lesion showed no obvious change at the 12-month follow-up.
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PMID:Rosai-dorfman disease with massive cutaneous nodule on the shoulder and back. 2567 35

Rosai-Dorfman disease (RDD) is primarily a disease of lymph nodes caused by accumulation of excessive histiocytes. The extra-nodal sites are simultaneously involved in almost half the cases. However, presentation with isolated extra-nodal sites is uncommon and disease confined to skin and soft tissues is a rare entity. We report RDD in a 33 year old man who presented as a flank mass and was a diagnostic challenge for the surgical team. This case report strengthens the evidence that RDD should be added to the list of rare causes of skin and soft tissue swellings and informs how to manage such patients.
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PMID:Cutaneous Rosai-Dorfman Disease presenting as a flank swelling. 2578 Mar 63

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a non-neoplastic condition which typically presents as massive, bilateral cervical lymphadenopathy and can involve multiple extranodal organ systems such as skin, eyes, and upper respiratory tract in about 28% cases. Bone lesions in association with nodal disease are seen in less than 10% cases. Isolated bone involvement as the only manifestation of SHML is extremely rare, with less than 50 cases reported in the literature. We report a very uncommon case of Rosai-Dorfman disease with isolated multifocal osseous involvement as the only presenting feature, involving about 10 different sites with no lymphadenopathy or other organ system involvement.
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PMID:A rare case of extra nodal Rosai-Dorfman disease with isolated multifocal osseous manifestation. 2628 24

Multinucleated giant cells of osteoclastic type are known to occur in nodal and extranodal lesions of Langerhans cell histiocytosis (LCH). These giant cells are thought to be derived from phagocytic histiocytes, which correlate with the degree of necrosis in LCH lesions. Emperipolesis commonly seen in Rosai-Dorfman disease is a distinct phenomenon characterized by intact phagocytosed cells in an intracytoplasmic vacuole protected from proteolytic digestion. We present a case of emperipolesis of inflammatory cells especially of eosinophils by multinucleated giant cells of Langhans type in a lymph node involved by LCH--a finding that has not been described previously in the literature.
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PMID:Nodal Langerhans cell histiocytosis with prominent eosinophilic emperipolesis. 2638 11

We present a rare case of Rosai-Dorfman disease who was sent for whole-body 18F-FDG PET/CT to know the exact extent of involvement. PET/CT revealed extensive nodal and extranodal involvement from head to toe including osteolytic lesions in metacarpals, metatarsals, and phalanges. It is important to know the exact extent of the disease as this may alter the patient management.
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PMID:18F-FDG PET/CT of Widespread Rosai-Dorfman Disease. 2640 32

Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder that is characterized by persistent massive lymphadenopathy mimicking malignant tumors. Extranodal RDD is uncommon, and more severe fibrosis and fewer histiocytes in lesions make the diagnosis of RDD more difficult than that of nodal RDD. We herein described a 31-year-old male patient with isolated soft tissue RDD of the head and neck. The patient was referred to our hospital with a right neck mass. Computed tomography (CT) scans showed a diffuse enhanced tumor with an unclear border in the right side of the neck. 18F-fluorodeoxyglucose positron emission tomography (PET)/CT revealed high uptake in the corresponding lesion. Second wide local excisional biopsy led to a diagnosis of RDD, and immunohistochemistry was useful for diagnosing RDD. A systemic treatment with steroids improved his symptoms, including the neck mass. Physicians need to consider the diagnostic difficulty associated with extranodal soft tissue RDD as well as its rarity.
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PMID:Extranodal soft tissue Rosai-Dorfman disease of the head and neck and its diagnostic difficulty. 2652 42

Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence.
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PMID:Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule. 2658 39


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