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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 an uncommon pathologic condition of unknown ethiology with an idiopathic proliferation of the hystiocytes. It is generally presented with massive bilateral hypertrophy of the cervical lymph nodes. But other lymph nodes may also be involved. Approximately, 30% of these patients have extra nodal mass or lesion with different signs or symptoms depending on localization. We present two male siblings with Rosai-Dorfman disease who have classical cervical lymphadenopathy associated with progressive neurosensorial hearing loss and dural-based intracranial lesions.
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PMID:Rosai-Dorfman disease associated with neurosensorial hearing loss in two siblings. 1523

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. It is typically characterized by painless cervical lymphadenopathy, fever and weight loss. Although extranodal involvement has been reported in diverse sites, intracranial presentation, particularly in the absence of nodal disease is uncommon. To the best of our knowledge, 48 patients with intracranial masses have been reported previously. A 31-year-old man was admitted to our clinic with a 4-month history of progressive headache. His medical history was unremarkable except for occasional fever. There were not any neurological deficit and weight loss. No lymphadenopathy (particularly bilateral cervical) and extranodal involvement in diverse sites were revealed by physical and radiological examinations. Routine hematological and biochemical studies were normal except for mild leukocytosis and elevated erythrocyte sedimentation rate. The patient underwent magnetic resonance imaging (MRI) testing that revealed an enhancing mass in the left temporal lobe. Preoperative diagnosis was meningioma. The patient underwent a left frontotemporal craniotomy with complete resection of the mass. Histopathology was compatible with RDD. Extranodal RDD is rarely found intracranially. Prognosis is benign especially in the absence of nodal disease. It is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis.
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PMID:Isolated intracranial Rosai-Dorfman disease mimicking meningioma. 1558 Oct 22

Rosai-Dorfman disease (RDD) is an idiopathic proliferation of histiocytes that affects the lymph nodes. Central nervous system involvement in the absence of nodal disease is extremely rare. On neuroimaging studies, intracranial RDD appears as solitary or multiple well-circumscribed, dura-based lesions. The authors report on two cases of RDD with locally aggressive features including dural sinus invasion, which to their knowledge has never before been described. A 60-year-old woman presented with progressive dizziness and vertigo that had lasted for 1 week. Cranial computerized tomography and magnetic resonance imaging revealed an extraaxial homogeneous lobulated enhancing mass involving the right occipital lobe and the right cerebellar hemisphere. Invasion of the right transverse sinus was identified on a cerebral digital subtraction angiogram. A 59-year-old man with no prior medical illness experienced progressive weakness of both upper extremities and a partial complex seizure. Magnetic resonance imaging of his brain revealed a well-circumscribed enhancing mass in the left frontal lobe with extension to the right frontal lobe and invasion of the superior sagittal sinus. Both patients underwent resection of their brain masses. Pathological studies identified the disease as RDD in both patients.
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PMID:Rosai-Dorfman disease with dural sinus invasion. Report of two cases. 1579 95

Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a clinically benign, frequently chronic, painless lymphadenopathy. It can also involve extranodal sites. We describe a 37-year-old man with a recent diagnosis of systemic lupus erythematosus and antiphospholipid antibody syndrome who had lacrimal gland and orbital involvement and nodal and extranodal sites with RDD.
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PMID:Rosai-Dorfman disease in a patient with systemic lupus erythematosus. 1586 35

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), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement. This uncommon entity should also be included in the list of differential diagnoses of diffuse and massive lymphadenopathy, which includes includes infectious/granulomatous conditions, lymphomatous and metastatic disease.
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PMID:Rosai-Dorfman disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy. 1611 Jan 9

Rosai-Dorfman Disease (RDD) is an idiopathic reactive proliferation of distinctive histiocytes that have abundant cytoplasm and commonly exhibit intracytoplasmic ingestion of inflammatory cells (emperipolesis). The histiocytes are immunopositive for S100 protein and are typically associated with an infiltrate of lymphocytes, plasma cells, and neutrophils. The classic clinical presentation is massive enlargement of (usually) cervical lymph nodes with a histologic appearance that mimics exaggerated sinus histiocytosis. RDD can also involve extra-nodal sites and skin involvement is common either as part of disseminated disease or as a result of primary disease. We report an exceptional case of cutaneous RDD with crystal deposition in a young male presenting with skin nodules. Skin biopsy showed classic features of cutaneous RDD with the additional feature of conspicuous rhomboidal and needle-shaped crystals within the cytoplasm of many lesional plasma cells, histiocytes, and also in an extra-cellular location. The plasma cells were polyclonal by light chain immunostaining. Crystal deposition has not been reported to date in RDD and is likely a result of the reactive plasma cell proliferation.
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PMID:Crystal deposition in a case of cutaneous Rosai-Dorfman disease. 1612 Oct 57

Rosai-Dorfman disease in soft tissue without nodal disease has been recognized as a distinct clinicopathologic entity. It may represent a diagnostic challenge and the natural history and optimal treatment has not been well clarified. We investigated a patient in whom Rosai-Dorfman disease was confined to the subcutis of the abdominal wall and recurred after incomplete excision. Complete resolution was achieved by wide surgical excision with negative margins. Pathologic examination confirmed the diagnosis of extranodal Rosai-Dorfman disease. The patient is disease-free after 1 year of follow-up. Despite the possibility that spontaneous remission may occur, our results suggest that when anatomically feasible, complete excision can be a treatment option for persistence or recurrence of exclusively extranodal disease. Larger case series and longer follow-up are needed to assess the long-term efficacy in these patients.
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PMID:Subcutaneous Rosai-Dorfman disease: is surgical excision justified? 1626 85

Rosai-Dorfman disease is a rare, benign histiocytic proliferative disorder which commonly affects the lymph nodes. Extranodal involvement along with concomitant nodal disease occurs in about 43% of cases. Isolated extranodal Rosai-Dorfman disease is relatively uncommon. We report this rare case of isolated extranodal Rosai-Dorfman disease and its ultrasound findings.
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PMID:A rare case of extranodal Rosai-Dorfman disease. 1698 Jun 64

Rosai-Dorfman disease (RDD) is a rare, non-neoplastic histiocytosis most commonly characterized by painless, massive cervical lymphadenopathy. Over half of all patients with lymph node involvement also demonstrate extranodal disease, with most affected individuals exhibiting lesions within the region of the head and neck. Oral manifestations of RDD are extremely rare, and almost always identified in association with nodal or other extranodal disease. However, rare examples of extranodal RDD, including oral RDD, without clinical or radiographic evidence of concomitant lymph node involvement, have also been described. We report a rare case of solitary, extranodal RDD arising within the mandible in a pregnant woman. To our knowledge, this case represents only the fourth documented example of solitary oral RDD. A review of the clinical and histopathologic features associated with RDD is also presented.
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PMID:Rosai-Dorfman disease of the mandible. 1699 19


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