Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present a case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) manifesting primarily as a pleural disease without involvement of the lung parenchyma. The patient is an 81-year-old man who presented with increasing shortness of breath. Radiographic studies revealed a large loculated left-sided pleural effusion and parietal pleural thickening. Video-assisted thoracoscopic biopsy of the pleura showed the characteristic S100-positive histiocytes exhibiting emperipolesis. In this rare case of pleural Rosai-Dorfman disease, the extensive involvement of the pleural lymphatics by the characteristic histiocytes appears to have been responsible for the pleural effusion.
 ...
PMID:Rosai-Dorfman disease of the pleura: a rare extranodal presentation. 1465 24

Rosai-Dorfman disease is a rare condition characterised by massive lymphadenopathy involving the orbit in 11% of cases. Immunohistochemical analysis remains key to the diagnosis with characteristic immunohistochemical S100 staining of histiocytes and lymphocyte phagocytosis. We report the case of a 43-year-old female with recurrence of bilateral orbital Rosai-Dorfman disease following long-term immunosuppressant therapy. Long-term follow-up is advised in conservatively managed patients and surgery may offer more definitive treatment.
 ...
PMID:Rosai-Dorfman disease recurrence with bilateral orbital masses following immunosuppressant therapy. 1576 18

Rosai-Dorfman disease (RDD) is an idiopathic, non-neoplastic, lymphoproliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. When RDD involves the central nervous system the lesion simulates a meningioma. Histological and immunohistochemical confirmation is essential for a definitive diagnosis. In this paper, ten cases of RDD confined to the central nervous system are reported. Another case with orbital RDD was excluded. Nine cases involved the cranial cavity alone; in one, the cervical extradural region was also involved. Treatment consisted of surgical excision or biopsy. Histology and immunohistochemistry revealed a mixed cell population of predominantly mature histiocytes with evidence of emperipolesis and strong positivity for S100 protein in all cases. No recurrence was observed during follow up ranging from three months to eight years.
 ...
PMID:Rosai-Dorfman disease of the central nervous system. 1609 62

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is an idiopathic condition typically associated with cervical lymphadenopathy, fever and hypergammaglobulinaemia. Extranodal involvement has been reported in diverse sites such as the skin, upper respiratory tract, orbit and the central nervous system. We document a case of intracranial RDD in a 40-year-old woman with rapid evolution over a period of three months. Clinically, the patient suffered from headache. The MRT showed a left parietal tumour with dural attachment. Histologically, the lesion consisted of pale-staining histiocytes with emperipolesis, neutrophilic granulocytes and scattered lymphocytes. Focally, the granulocytes dominated the histological picture. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein, CD68 and FXIIIa, but negative for CD1a. No Birbeck-granula were detectable in electron microscopic analysis. Granulocytes showed a positive Anti-HHV6b immunoreaction. The tumour was diagnosed asA'an intracranial manifestation of RDD primary to the CNS with an unusual preponderance of neutrophilic granulocytes and with only scattered lymphocytes. The postoperative clinical staging showed no other manifestations of the disease. On postoperative MRI the lesion had been completely resected. No further therapy has been applied and the patient has had an unremarkable clinical course for the last ten months.
 ...
PMID:A case of rapidly progressive Rosai-Dorfman disease restricted to the central nervous system. 1611 57

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.
 ...
PMID:Crystal deposition in a case of cutaneous Rosai-Dorfman disease. 1612 Oct 57

Sinus histocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare histiocytic disorder that typically presents with chronic, self-limiting, cervical lymphadenopathy. We present a case, a diagnostic dilemma for multiple consultation services, of an otherwise well 17-year-old boy without lymphadenopathy who, 8 months after excision of a T9 lytic vertebral lesion and epidural mass that caused cord compression, again presented with cord compression from progressive vertebral disease, recurrent epidural mass, and development of a paraspinal mass and tibial lesion. The excised vertebral and epidural lesions, 2 paraspinal biopsies, and tibial biopsy were interpreted as chronic inflammation until large histiocytes were noted, which were positive for CD68, S100 protein, fascin, and MAC387, and demonstrated characteristic emperipolesis (lymphophagocytosis) that was diagnostic of Rosai-Dorfman disease. This atypical clinical behavior and sites of involvement of multiple bones but not of lymph nodes is unusual and constitutes the aggressive end of the clinical spectrum and a rare cause for cord compression.
 ...
PMID:Extranodal Rosai-Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression. 1621 43

Rosai-Dorfman disease (RDD) is a non-neoplastic proliferative histiocytic disorder that primarily affects lymph nodes (sinus histiocytosis with massive lymphadenopathy). Primary RDD of the central nervous system is most uncommon. We report on a 35-year-old man with isolated RDD of the meninges overlying the left cerebral hemisphere. Presenting signs and symptoms included severe progressive ipsilateral headaches of 4 months duration, as well as laboratory evidence of mild non-specific systemic inflammatory reaction. On magnetic resonance imaging, the lesion was seen as a contrast-enhancing, plaque-like thickening of the dura mater over the left convexity,without impinging on adjacent bone or cerebral parenchyma. Meningeal biopsy revealed a mixed mononuclear infiltrate dominated by CD68(+), S100(+), CD1a(-) non-Langerhans type histiocytes on a background of fibrosis. Bacteria, in particular mycobacteria, and fungi were excluded with special stains. Extensive clinical workup, encompassing computed tomography of thoracal and abdominal organs, bone marrow biopsy, and bronchoalveolar lavage failed to reveal any extracranial involvement. Laboratory tests for autoimmunity, including C- and P-antineutrophil cytoplasmic antibodies, antinuclear antibody, and serum rheumatoid factor, were negative. Methylprednisolone therapy induced complete remission of symptoms, with the neuroradiologic status remaining unchanged on follow-up after 2 months. We discuss the complex clinicopathologic differential diagnosis and therapeutic issues of this rare condition. While the correct diagnosis of central nervous system RDD is unlikely to be established without invasive procedures (biopsy), a conservative therapeutic approach may be considered a legitimate option.
 ...
PMID:Isolated Rosai-Dorfman disease of intracranial meninges. 1637

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and self-limiting benign disorder that most commonly involves the cervical lymph nodes. The authors describe two cases of SHML. Fine-needle aspiration of the lymphadenopathy was performed in both patients. Immunocytochemical and histologic features, as the evidence of emperipolesis and S100 protein positivity on immunostaining, were typical of SHML. Fine-needle aspiration cytology plays an important diagnostic role in SHML and may be conclusive in a typical clinical setting. The diagnosis of SHML should be considered in the differential diagnosis of massive, painless cervical lymphadenopathy. Long-term follow-up is necessary to observe the complete regression of the massive lymphadenopathy. However, specific therapy is available and should be limited to patients with compressive symptoms or extranodal disease.
 ...
PMID:Rosai-Dorfman disease: two case reports and diagnostic role of fine-needle aspiration cytology. 1646 85

A 47 year old Chinese man with diabetes mellitus and previously treated pulmonary tuberculosis presented with painless right testicular enlargement of 1 month's duration. He underwent an orchidectomy for presumed testicular neoplasm corroborated clinicoradiologically. Histological examination of the testicular mass revealed an inflammatory lesion comprising lymphocytes, plasma cells and sheets of pale staining histiocytes, some containing lymphocytes within their ample cytoplasm, suggestive of emperipolesis. S100 immunohistochemistry stained the histiocytes, while ultrastructural examination confirmed emperipolesis. A diagnosis of Rosai-Dorfman disease was made, an exceedingly rare testicular lesion. Clinicoradiological findings mimicked a neoplasm, while the inflammatory histological appearances with occasionally discerned multinucleated cells raised the possibility of xanthogranulomatous orchitis. Tuberculous orchitis was excluded through negative Ziehl-Neelsen stains and PCR for mycobacterium, while seminoma, which sometimes features a predominant inflammatory component obscuring neoplastic cells, was excluded by absent immunostaining for placental alkaline phosphatase and CD117.
 ...
PMID:Rosai-Dorfman disease of the testis: an unusual entity that mimics testicular malignancy. 1650 87

Rosai-Dorfman disease (RDD) is a rare non-neoplastic histiocytic proliferative disorder characterized by painless lymphadenopathy. Extranodal lesions frequently occur in the head and neck regions. We report the clinical and histological features of extranodal RDD in a 43-year-old man with a previously unreported combination of multiple gross anterior epibulbar nodules in the right eye, submucosal masses of nasal septum and trachea, and no lymphadenopathy during the 12-year follow-up. The patient underwent ophthalmological, otolaryngological and systemic evaluation; gallium 67 scintigraphy; bronchoscopy; ophthalmic ultrasound; head and neck CT scan; biopsies of epibulbar, nasal and tracheal tissues; and septoplasty. Histological specimens showed lymphocytophagocytosis and positive immunoperoxidase staining for S100 protein in foamy histiocytes; both features were typical for RDD. No response to topical or systemic steroids or to radiation therapy was recorded. Removal of nasal septum masses resolved nasal obstruction. The diagnosis of RDD requires histological and, in challenging cases, immunohistological specimens and is difficult--especially with pure extranodal localizations as in our case. RDD should be suspected in cases of subconjunctival mass and/or submucosal nasal and tracheal swellings not responding to systemic steroids.
 ...
PMID:Extranodal Rosai-Dorfman disease: involvement of eye, nose and trachea. 1672 Apr 53


<< Previous 1 2 3 4 5 6 7 8 Next >>