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 (SHML)-Rosai-Dorfman disease-is a rare but well-defined benign histiocytic proliferative disorder. We report an unusual patient, cured of non-Hodgkin's lymphoma, who presented 12 years later with renotesticular SHML associated with IgA monoclonal gammopathy, but without any evidence of relapsed lymphoma. The genitourinary manifestations of this disorder with massive lymphadenopathy and its rare association with malignant lymphoma are reviewed.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a patient with isolated renotesticular involvement after cure of non-Hodgkin's lymphoma. 903 15

The entity known as sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RD disease), is an uncommon benign proliferation of hematopoietic and fibrous tissue that often presents in the head and neck region. Its initial manifestations most often include a roughly symmetric, painless, bilateral cervical adenopathy, although extranodal disease may develop in a minority of patients. The key histologic feature of SHML is the presence of various numbers of large, pale histiocytic cells that contain within their cellular borders apparently engulfed lymphocytes ("emperipolesis"); these distinctive large, pale cells - RD cells - are S-100 protein-positive by immunostaining and so differ from ordinary histiocytes. Despite its sometimes impressive clinical presentation, SHML is a benign and self-limited disease, whose treatment is aimed largely at controlling its local manifestations (most often by surgical therapy). The microscopic differential diagnosis, particularly in extranodal disease, is at times challenging and can include Langerhans' cell histiocytosis, Hodgkin's disease, non-Hodgkin's lymphoma, metastatic carcinoma, and metastatic malignant melanoma.
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PMID:Review of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of head and neck. 1057 39

We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.
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PMID:PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. 1456 72

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

Primary histiocytic sarcoma (HS) of the central nervous system (CNS) is a rare haematopoietic neoplasm. The inconsistent terminology and diagnostic criteria currently used for CNS HS have complicated the appreciation of the clinical aspects of the disease. The main differential diagnoses are non-Hodgkin's lymphoma, reactive histiocytic proliferation, dendritic cell neoplasm, undifferentiated carcinoma, inflammatory pseudotumour, Rosai-Dorfman disease and abscess. The true diagnosis of CNS HS requires an extensive immunophenotypic workup using specific histiocytic markers, such as CD163, with the exclusion of markers of other cell lineages. This clinicopathological case report describes an improved approach towards the differential diagnosis of CNS HS.
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PMID:Isolated III cranial nerve palsies may point to primary histiocytic sarcoma. 2512 71

The association between Rosai-Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.
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PMID:Isolated central nervous system Rosai-Dorfman disease and breast cancer: an unusual presentation. 3029 95