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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 is a newly recognized benign disease affecting mainly children and young adults and usually having a protracted clinical course that is relatively unaffected by therapy. This paper describes four additional patients who had orbital involvement initially and reviews the salient clinical and histopathologic features of this entity. The outstanding clinical feature is cervical lymphadenopathy. Associated findings include low-grade fever, anemia, leukocytosis, and elevated IgG levels. A small percentage of patients develop proptosis with palpable orbital tumors. Such patients may not have appreciable lymphadenopathy. Progressive proptosis may lead to exposure keratitis, corneal ulceration, endophthalmitis, and loss of the eye. Histopathologically, the lymph nodes and orbital mass show a proliferation of large histiocytes intermixed with a variable proportion of lymphocytes and plasma cells. Lymphocytes and occasionally other cells derived from the hematopoietic system are commonly seen within the cytoplasm of the histiocytes.
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PMID:Orbital involvement in 'sinus' histiocytosis. A report of four cases. 92 80

We describe two children with Rosai-Dorfman disease who presented with exophthalmos, leukocytosis, an elevated sedimentation rate and hypergammaglobulinemia. Both became blind as a result of this condition. One child had associated bilateral cervical lymphadenopathy. Investigation revealed involvement of the nasal fossae and retro-orbital spaces by tumoural masses histologically consistent with the diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML). Immunohistochemical studies suggest that these histiocytes are activated macrophages derived from a phenotype hybrid between "professional" phagocytic cells and immune accessory cells, expressing lysosomal antigens, S-100 protein but rarely, CDla. An underlying immune dysfunction may be central to the pathogenesis of this disease.
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PMID:Extranodal Rosai-Dorfman disease in childhood. 895 50

This is a case report of Rosai-Dorfman syndrome in a 36-year-old Caucasian male, involving the lacrimal gland, cervical lymph nodes, nasal and sinusal mucosa. It was successfully treated with appropriate immunosuppression. He had initially presented to the ENT surgeon with nasal and sinusal mucosal thickening and bleeding. Cervical lymph node biopsy produced a histological diagnosis compatible with Rosai-Dorfman disease. Later he developed an acute red proptotic eye. He had severe proptosis due to an enlarged lacrimal gland. He refused surgical excision of the tumour, which is suggested if there is an ocular adnexal involvement. Conservative treatment with systemic steroid resulted in the resolution of lacrimal gland swelling, nasal sinusal mucosal thickening and cervical lymphadenopathy. Previous studies have shown that patients with Rosai-Dorfman syndrome are often black males1 and require surgery.
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PMID:Rosai-Dorfman syndrome affecting the lacrimal gland. 1204 17

We report the clinical and histopathological features of 2 cases of orbital Rosai-Dorfman disease with lacrimal gland involvement but no lymphadenopathy or cutaneous involvement. The first case was a 7-year-old boy, who was referred to the ophthalmology department with a proptosis in the left eye. The second case involved a 57-year-old African man, who developed oedema of the right upper eyelid over a 4-month period. Ocular involvement in Rosai-Dorfman disease is rare (10%). The diagnosis is histological in combination with immunohistology and is often delicate, especially in extranodal localizations. Histopathologically, normal gland morphology was altered by fibrosis and inflammatory cells. To our knowledge, this is the first report on lacrimal involvement in incomplete, thus uncommon sinus histiocytosis with massive lymphadenopathy.
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PMID:Orbital sinus histiocytosis (Rosai-Dorfman disease): a lacrimal gland involvement. 1220 33

Rosai-Dorfman disease is a rare, non-neoplastic disease characterized by an unusual proliferation of histiocytic cells. It rarely manifests intracranially, and only 50 cases of intracranial lesions have been reported. We describe an unusual case of a huge, solid mass in the paranasal sinuses, orbits, cavernous sinuses, and suprasellar cisterns. A 29-year-old patient was admitted to our hospital with nasal obstruction and proptosis and visual loss in the right eye. A biopsy was done at another institution and the diagnosis was "pseudotumor of orbit". A right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy was used to excise the tumor with right orbital exenteration. The histopathological diagnosis was consistent with Rosai-Dorfman disease. The patient underwent postoperative chemotherapy. Involvement of the central nervous system in Rosai-Dorfman disease is rare, but the disease's ability to mimic other pathologies underlines its importance.
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PMID:Giant intracranial Rosai-Dorfman disease. 1517 15

Rosai-Dorfman disease is a rare benign idiopathic histioproliferative disorder usually manifesting as massive painless adenopathy. Extranodal involvement of the Central Nervous System (CNS) mimicking a skull base meningioma is rare. A 42-year-old male presented with painless, progressive left visual loss of 4 months duration. Clinically, he had a left ptosis, proptosis and ophthalmoplegia. Magnetic Resonance Imaging (MRI) of the brain with gadolinium revealed a destructive lesion of the left orbital apex, middle cranial fossa and cavernous sinus. He was treated with corticosteroids and underwent debulking. Pathology showed inflammatory infiltrate in the absence of an infectious agent, emperipolesis and a positive S100 stain was consistent with Rosai-Dorfman disease. As there was no improvement following steroids and debulking, he underwent radiation therapy with significant improvement of his symptoms. Although a rare entity, Rosai-Dorfman disease should be considered in the differential of a skull base lesion.
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PMID:Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. 1902 Aug 7

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare histiocytic proliferative disorder of unknown aetiology. The classic presentation of Rosai-Dorfman disease is massive, bilateral, painless cervical lymphadenopathy. Extranodal disease is also common, often with a particular predilection for the head and neck regions. We report a rare case of Rosai-Dorfman disease with nodal and multiple extranodal manifestations in a 13-year-old girl. The patient had cervical and mediastinal lymphadenopathy, multiple soft tissue swellings of the scalp, bilateral proptosis and goitre due to thyroid gland involvement. She responded to steroids, with remission of the swellings and symptoms. This case is being reported for its rarity, multiple extranodal manifestations and thyroid gland involvement. Thyroid gland involvement in Rosai-Dorfman disease has rarely been reported in the literature.
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PMID:Rosai-Dorfman disease. 2110 7

Rosai-Dorfman disease (RDD) is a rare lymphoproliferative disorder with nodal and extranodal involvements. Here we report a case of RDD in a 15-year-old female who presented with epigastric pain, fatigue, Raynaud phenomenon in fingers, submandibular lymphadenopathy, proptosis, hepatosplenomegaly, and round shape painless patches on the extensor surfaces. Histological examination of the submandibular lymph nodes and skin biopsy demonstrated evidences of RDD. Patient was treated with prednisone and thereafter, with azathioprine. After one year, prednisone was discontinued and all of the symptoms and signs, except proptosis, were resolved. This report highlights the extranodal manifestations of RDD. The presentation, differential diagnosis, and treatment are discussed.
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PMID:Rosai-Dorfman Disease with nodal and extranodal involvements: A case report. 2297 96

A case of a 9-year-old Black African male with right-sided proptosis is presented. The clinical interpretation was that of orbital rhabdomyosarcoma and an incisional biopsy was performed. This revealed the typical features of Rosai-Dorfman disease, featuring S100 positive non-Langerhans histiocytes with emperipolesis. There were also numerous plasma cells in the biopsy, in the more fibrotic areas. More than 40% of these plasma cells were positive for IgG4. This case represents the first case in the orbit to make the association of IgG4 plasma cell positivity with Rosai-Dorfman disease. Potential associations are discussed, and the relevance of this finding to ophthalmic pathology interpretation and differential diagnosis are highighted.
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PMID:A case of orbital Rosai-Dorfman disease with IgG4 positive plasma cells. 2387 15

Extranodal Rosai-Dorfman disease involving the orbit has been reported severally with extensive discourse on management strategies. However, rhabdomyoblastic differentiation coexisting with the disease is yet unreported. We present the clinical and histopathological features of a 12-year-old boy presenting with both. A 12-year-old boy was referred to our Ophthalmic Plastic Surgical Service with a 5-year history of bilateral, progressively enlarging eyelid, orbital masses causing proptosis, and severe cosmetic blemish. He had suffered vision loss to the right eye following trauma 2 years prior to the onset of the disease. Computed tomography of the orbits revealed preseptal and intraorbital components of well-defined multi-lobulated masses in both orbits. No significant nodal involvement was noted. He underwent excision biopsy of the tumor in both eyes performed 1 month apart. A histopathologic diagnosis of the Rosai-Dorfman disease with focal areas of rhabdomyoblastic differentiation was made. He was treated postoperatively with a course of oral steroids with close observation for the rhabdomyoblastic differentiation. Rosai-Dorfman disease is an uncommon disease and has been reported to coexist with other pathologies. We highlight focal myoblastic differentiation within the excised tissues which may not necessarily warrant a diagnosis of rhabdomyosarcoma. Long-term follow-up of this patient is required to establish the safety of observation.
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PMID:Rhabdomyoblastic differentiation in rosai dorfman disease of the orbit in a 12-year-old male. 3056 Aug 34


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