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)

We report the cytologic findings of a case of Rosai-Dorfman disease of the breast in a 52-year-old diabetic woman, initially sampled by fine-needle aspiration biopsy (FNA). The patient presented with a 2-week history of a 3 x 2 cm nodule in the mid-upper area of the left breast. A mammogram taken 6 months prior was negative. FNA smears demonstrated lymphocytes, plasma cells, and large pale cells, with enlarged irregular nuclei, admixed with fragments of fibrous tissue and calcific debris. Lymphophagocytosis (emperipolesis) was scarce. Our diagnosis was atypical lymphohistiocytic proliferation. Flow cytometry was negative, but in the face of a strong clinical suspicion of a lymphoid malignancy, excision was performed. The final diagnosis was Rosai-Dorfman disease (RDD). The differential diagnosis of FNA of breast inflammatory lesions with atypical cytology is discussed, with a review of the literature. The early recognition on FNA of the hallmarks of this rare disease should prevent unnecessary radical surgery. Diagn. Cytopathol. 1999;21:287-291.
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PMID:Fine-needle aspiration cytology of Rosai-Dorfman disease of the breast: A case report. 1049 25

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is primarily a nodal-based, idiopathic, benign proliferative disorder of histiocytes with 43% of these cases also involving extranodal sites. The breast is an unusual site of occurrence of RDD. We report two cases of this exceptional event. The first represents an intramammary nodal Rosai-Dorfman disease, while the second is an extranodal disease with sole involvement of the breast. In both, the possibility of malignancy was raised. Histological examination of the two breast lesions revealed sheets of characteristic large histiocytes displaying emperipolesis, a microscopic hallmark of this disease. Immunohistochemical and ultrastructural studies were also performed; the former showed cytoplasmic staining of histiocytes for S-100 protein, while histiocytes that engulfed lymphocytes and plasma cells were identified on electron microscopy.
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PMID:Rosai-Dorfman disease of the breast: a mimic of breast malignancy. 1074 Jul 98

A case of invasive bladder cancer accompanied with enlarged pelvic lymph nodes, which were interpreted as metastasis, is reported. The lymphadenopathy eventually regressed spontaneously while the bladder cancer increased in size. Total cystectomy and pelvic lymph node dissection were performed. Histopathological examination of the lymph nodes revealed dilation of the sinuses and marked increase in the number of histiocytes, which suggested sinus histiocytosis. Sinus histiocytosis is often associated with malignant tumor. It is considered as a sign of the host's immune activation with favorable prognostic significance. Nevertheless, such lymphadenopathy mimics a metastatic lymph node, making proper staging difficult.
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PMID:Lymphadenopathy of sinus histiocytosis mimicking metastatic bladder cancer: a case report. 1288 74

Lymphadenopathy is a common clinical finding and is frequently benign. Warning signs suggestive of a malignant etiology include lymph nodes >2 cm in size, supraclavicular location, and generalized lymphadenopathy associated with hepatosplenomegaly or systemic symptoms. A metastatic solid tumor is always in the differential diagnosis of localized lymphadenopathy, particularly in older individuals. In the case of more generalized lymphadenopathy, in addition to the more common lymphomas, benign etiologies as well as benign and atypical lymphoproliferative disorders need to be considered. Benign etiologies of lymphadenopathy can include infections, autoimmune disorders, drug hypersensitivity reactions, sarcoidosis, and amyloidosis. Rare but benign lymphoproliferative disorders include Kikuchi's disease, Rosai-Dorfman disease, and progressive transformation of germinal centers. Atypical lymphoproliferative disorders that bear close surveillance for evolution to a more aggressive malignancy include Castleman's disease, lymphomatoid granulomatosis, and lymphomatoid papulosis. Previously considered in this category but now classified as a true lymphoma is angioimmunoblastic lymphadenopathy with dysproteinemia. Physicians need to be aware of all of these disorders when evaluating suspicious lymphadenopathy, while also considering the more common lymphomas and leukemias.
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PMID:Clinical mimics of lymphoma. 1695 99

Sinus histiocytosis with massive lymphadenopathy (SHML) is a histiocytic disorder affecting children and adults. It usually presents as markedly enlarged lymph nodes that require surgical biopsy for confirmation. This lesion is usually self-limited but can present in areas that can cause significant morbidity or disfigurement. We report a case that required therapy due to the severe disfigurement but was resistant till treated with dexamethasone. This case illustrates that SHML may be resistant to prednisone but still be sensitive to dexamethasone.
Pediatr Blood Cancer 2005 Jan
PMID:Sinus histiocytosis with massive lymphadenopathy (SHML) prednisone resistant but dexamethasone sensitive. 1583 58

CD163, a hemoglobin scavenger receptor, is expressed in monocytes and macrophages. We tested the expression of the CD163 protein in 1,105 human malignancies and normal tissues using tissue microarrays and conventional paraffin-embedded tissue sections. Besides staining nonneoplastic monocytes and histiocytes (tissue macrophages), membranous/cytoplasmic staining for CD163 was primarily limited to neoplasms with monocytic/histiocytic differentiation. CD163 reactivity was not observed in normal tissues, lymphomas, carcinomas, and in a majority of mesenchymal neoplasms, including follicular dendritic cell tumors (0 of 4), although it stained admixed histiocytes. Staining for CD163 was seen in Rosai-Dorfman disease (5 of 6), histiocytic sarcoma (3 of 4), littoral cell angioma (6 of 6), and Langerhans cell histiocytosis (3 of 5). A subset of atypical fibrous histiocytomas (9 of 16), benign fibrous histiocytomas (6 of 9), and atypical fibroxanthomas (1 of 3) also showed CD163 staining. Our studies also confirm earlier work showing that CD163 is expressed in acute myeloid leukemia with monocytic differentiation (AML, FAB subtype M5) (2 of 6), as well as a majority of giant cell tenosynovial tumors (7 of 8). Its limited range of expression and tissue specificity indicate that CD163 may have significant diagnostic utility in separating specific tumors with monocytic and histiocytic derivation from other entities in their differential diagnosis.
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PMID:Expression of CD163 (hemoglobin scavenger receptor) in normal tissues, lymphomas, carcinomas, and sarcomas is largely restricted to the monocyte/macrophage lineage. 1583 85

Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease) is a nonneoplastic, usually self-limiting disease. Alhough it affects all age groups, it is more commonly seen in young males in their first or second decades. The disease primarily manifests as painless lymphadenopathy of cervical region, but other nodal groups and extranodal sites may also be affected. Seldom SHML may involve the salivary glands. We present the cytologic features and differential diagnoses of one such case.A 45-yr-old woman presented with an enlarged submandibular gland on the left side with ipsilateral cervical lymphadenopathy that had been persisting for 1 mo. The gland and the two enlarged nodes measured 2.5 x 2.5 cm(2) each and were firm in consistency. Clinically, tumor of the salivary glands was suspected. Fine-needle aspiration (FNA) smears showed moderate cellularity, with large histiocytes dispersed in the background of intense lymphoplasmacytic infiltrate. These histiocytes showed lymphophagocytosis. Isolated stromal fragments consisting of fibrocytes were seen separate from salivary acinar clusters on repeat aspirations. The diagnosis of SHML involving left submandibular gland and ipsilateral lymph nodes was returned. When analyzed in the context of clinical findings (laboratory data), the cytologic features of SHML involving salivary gland could be differentiated from those of malignancies and other benign lesions, especially Kuttner's tumor of the submandibular gland, which mimics neoplasm clinically.
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PMID:Salivary gland manifestations of sinus histiocytosis with massive lymphadenopathy: fine-needle aspiration cytology findings. A case report. 1607 53

Rosai-Dorfman disease (RDD) is a rare, sporadic histiocytic disorder characterized by painless but protracted lymphadenopathy. Its etiology remains unclear. The observation of congenital disease and reports of familial cases with seven pairs of siblings including three sets of identical twins suggests a genetic predisposition in some patients with this condition. We now report two brothers of consanguineous Palestinian parents, whose lymphadenopathy, lymph node histology, and polyclonal hypergammaglobulinemia indicated RDD. The presence of intrauterine fractures, short stature, and sensorineural hearing impairment suggested a rare familial form of the disorder. Moynihan et al. recently described a Pakistani family with a familial histiocytic disorder highly reminiscent of the brothers reported here, whose lymph node morphology was apparently consistent with RDD as well. The presence of sensorineural deafness, short stature, and joint contractures, however, suggested a separate, rare autosomal recessive syndrome referred to as Faisalabad histiocytosis, after the family's place of origin. We believe that the brothers described here represent a second family with Faisalabad histiocytosis, which mimics RDD histologically.
Pediatr Blood Cancer 2006 Oct 15
PMID:Faisalabad histiocytosis mimics Rosai-Dorfman disease: brothers with lymphadenopathy, intrauterine fractures, short stature, and sensorineural deafness. 1615 31

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare but well-defined histiocytic proliferative disorder of unknown etiology that usually presents with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia in an otherwise healthy child. Although many patients undergo spontaneous remission, a subset of patients with systemic disease has a more serious course. For those patients with a poor outcome, steroids and chemotherapeutic agents such as etoposide or 6-mercaptopurine plus low dose methotrexate have been used. We present a child with a massive cervical lymphadenopathy treated with 2-chlorodeoxyadenosine (2-CdA, cladribine) after other approaches failed.
Pediatr Blood Cancer 2006 Oct 15
PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine. 1630 14

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder of unknown cause. RDD most commonly involves the cervical lymph nodes, but extranodal involvement has been described. We report the case of a patient with extranodal RDD that presented as a retroperitoneal mass obstructing the left ureter. The patient underwent surgical resection of the mass, followed by a 5-month course of vinblastine. There was no evidence of progressive disease 22 months after surgery.
Int J Gynecol Cancer
PMID:Extranodal Rosai-Dorfman disease as a pelvic mass. 1651 11


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