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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, also known as Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites. However, clinical suspicion is difficult and there is also a lack of useful diagnostic markers for this disorder prior to histological confirmation. High elevation of serum ferritin is known to be a useful diagnostic marker for various hematologic diseases, including hemophagocytic lymphohistiocytosis and lymphoma. Here, we report a case of fever of unknown origin that presented along with highly elevated serum ferritin (5,780 ng/mL), and was finally diagnosed as Rosai-Dorfman disease by lymph node biopsy.
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PMID:A case of rosai-dorfman disease with highly elevated serum ferritin. 2238 84

Rosai-Dorfman disease (RDD) is a rare macrophage-related histiocytic disorder that occurs primarily in lymph nodes or extranodal sites. Primary splenic lymphoma is an even rarer form of malignant lymphoma exhibiting characteristic diagnosis, treatment and outcome that have been poorly documented. Documentation of a rare case of primary splenic diffuse large B-cell lymphoma concomitant with isolated thymus RDD is reported in a 42-year-old male patient who was admitted to our hospital for persistent fever and splenomegaly after diagnostic thymectomy. On pathological review of the patient's resected thymus tissue, notable characteristics of thymus RDD were observed. The patient's condition quickly deteriorated, and the spleen progressively enlarged over the course of steroid treatment in our facility. Subsequent diagnostic splenectomy results indicated that non-Hodgkin's lymphoma (nongerminal center diffuse large B-cell lymphoma) was present in splenic tissues. Results of the current case study suggest that early exclusion of possible lymphoma is necessary in RDD patients who exhibit limited or no response to steroid therapy.
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PMID:Primary splenic diffuse large B-cell lymphoma in a patient with thymus Rosai-Dorfman disease. 2262 62

A 14-year-old boy was referred to us with a history of progressive painless massive symmetrical cervical lymphadenopathy, provisionally diagnosed as lymphoma. Multiple biopsies were needed, and confirmed the diagnosis of Rosai Dorfman disease (RDD), which is a very rare benign condition. Sinus histiocytosis with massive lymphadenopathy, also known as RDD, manifests as bulky lymphadenopathy in children and young adults. Extranodal sites such as the skin, upper airways, gastrointestinal tract and central nervous system can be involved. There is a characteristic pattern of lymphoid proliferation with a thick fibrous capsule, distention of lymphoid sinuses, accumulation of plasma cells and proliferation of large, often atypical, histiocytes showing emperipolesis is characteristic. The disease is considered to be benign and usually self-limited. We emphasise the need for doing a repeat lymphnode biopsy even if the prior histopathology is suggestive of reactive lypmhnode hyperplasia in every case of persisting lymphadenopathy.
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PMID:Rosai Dorfman disease: appearances can be deceptive. 2300 Oct 99

Rosai-Dorfman disease is a self-limiting condition caused by histiocyte proliferation within the sinusoids of lymph nodes and in extranodal tissue. It is a rare disease, particularly in children, that progresses with extensive lymphadenopathy. This paper reports on the case of a 2-year-old child with progressive cervical lymphadenopathy associated with persistent fever and radiological findings suggestive of lymphoma. Histopathological and immunohistochemistry studies of a lymph node biopsy established the diagnosis of Rosai-Dorfman disease. Both lymphadenopathy and fever resolved spontaneously.
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PMID:Rosai-Dorfman disease with spontaneous resolution: case report of a child. 2304 24

Sinus histiocytosis with massive lymphadenopathy (also known as Rosai Dorfman syndrome) is a unique disease of unknown etiology with a childhood predilection. It is an uncommon benign condition, often confused with lymphoma. Patients usually present with massive, painless, bilateral cervical lymphadenopathy and clinical course is variable. This condition has been sporadically reported in Indian literature. Here we report a 26-year-old women presenting with a painless bilateral cervical lymphadenopathy. A FNAC from cervical lymph node was compatible with Rosai Dorfman syndrome and confirmed histopathologically.
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PMID:Rosai Dorfman syndrome - A rare clinical entity. 2312 Apr 28

Kikuchi-Fujimoto disease, Kimura disease, Rosai-Dorfman disease and IgG4 related lymphadenopathy may present with enlarging masses clinically mimicking lymphoma. A combination of clinical and histologic findings is necessary to diagnose these important rare entities, which may occasionally have aggressive clinical behavior. The recognition of these disorders is important in order to avoid misdiagnosis of malignancy, other systemic diseases such as systemic lupus, and to institute correct management and therapy, such as steroid treatment for IgG4 related lymphadenopathy. The underlying etiologies of these diseases are not completely clear at present, however, their recognition has become more common as diagnostic techniques improve. Their diagnosis and recognition may help to elucidate their underlying pathobiology.
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PMID:Reactive lymphadenopathies that mimic lymphoma: entities of unknown etiology. 2353 13

We report the case of a 56-year-old woman who had severe leg pain and whose radiographs initially suggested metastatic carcinoma, lymphoma, osteogenic sarcoma, or adamantinoma. Results of multiple biopsies confirmed a diagnosis of Rosai-Dorfman disease, which typically presents in children and young adults (mean age at onset, 20 years).
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PMID:Extranodal Rosai-Dorfman disease as solitary lesion of the tibia in a 56-year-old woman. 2407 67

A previously healthy 3-year-old girl developed rhinorrhea, painful right neck swelling, and fever. Contrast-enhanced CT of the neck revealed cervical lymphadenopathy, for which lymphoma was considered. FDG PET/CT images showed intense activity not only in the neck but also in the thorax, abdomen, and pelvis. Biopsy was performed, and the diagnosis of Rosai-Dorfman disease was made.
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PMID:Rosai-Dorfman disease mimics lymphoma on FDG PET/CT in a pediatric patient. 2415 60

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

We report a 48-year-old man who presents with a 20-month history of left parotid enlargement despite treatment with antibiotics and steroids. He presented with a non-painful palpable mass in his left parotid without facial weakness or otologic symptoms. Fine needle aspiration was inconclusive, and an attempted excisional biopsy was aborted due to involvement of the facial nerve and suspicion of lymphoma. Subsequently, he underwent a total parotidectomy with facial nerve preservation. The specimen yielded results that were consistent with Rosai-Dorfman disease. Of note in our patient is the lack of cervical lymphadenopathy, which classically presents with this condition. Head and neck extranodal involvement, while not unusual for Rosai-Dorfman, is rarely seen in the parotid. Previous reports of this disease found in the parotid indicate an underlying autoimmune process, which was not found in this patient.
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PMID:Rosai Dorfman Disease presenting as unilateral chronic parotitis. 2496 Jun 76


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