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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report on 21 cases of "primary" xanthoma of bone. Twenty of the patients were older than 20 years old. The male-female ratio was 2:1. The presenting symptom was pain in 13 patients and neurologic symptoms in 2; in 6 patients, the lesion was an incidental finding. All but one of the lesions in this series were solitary, and the flat bones (pelvis, rib, skull) were the most frequently involved sites. Radiographically, a well-defined, sometimes expansile lytic lesion, with either a small area of surrounding reactive bone or a distinct sclerotic margin, was seen. Microscopically, foam cells, giant cells, cholesterol clefts, and fibrosis were present in varying degrees. In none of these cases was there an identifiable underlying lesion. The differential diagnosis includes Erdheim-Chester disease (a multisystemic granulomatosis) and bone involvement in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). More important is the differential diagnosis with metastatic clear cell carcinoma. Xanthoma of bone is a benign lesion, and complete or even partial removal is effective. Xanthomas may represent a "burnt-out" benign condition such as fibrous dysplasia or histiocytosis X.
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PMID:Xanthoma of bone. 314 Jun 52

The term sinus histiocytosis with massive lymphadenopathy (SHML) was introduced by Rosai and Dorfman in 1969. Although SHML was initially described as low-pain cervical lymphadenopathy accompanied by fever, leukocytosis, an increased erythrocyte sedimentation rate, and hypergammaglobulinemia, extranodal involvement is observed in 25 to 40% of cases. This pathology is very rare and involvement of the nasal fossae and paranasal sinuses is exceptional. We present two atypical cases of extranodal involvement exclusively confined to the head and review the literature. The first case showed a dramatic involvement of facial skin and muscles, orbit, and paranasal sinuses. In the second case, the disease was limited to the nasal fossae. Both cases showed exclusive extranodal involvement limited to the head region. In the absence of lymphadenopathy, diagnosing Rosai-Dorfman disease is difficult because of the lack of the most typical manifestation of the disease.
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PMID:Rosai-Dorfman disease with extranodal manifestation in the head. 1572 44

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.
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PMID:Soft tissue Rosai-Dorfman disease of the posterior mediastinum. 1966 12

Rosai-Dorfman disease is a rare, benign, idiopathic histio-proliferative disorder. Only 5% of cases involve the central nervous system. We describe a 10-year-old girl with pain in her lower limbs and back. Spinal magnetic resonance imaging revealed an intradural extramedullary lesion at T9-T10. We decided on surgical treatment. An anatomic/pathologic examination revealed histiocytic-like cells and extensive fibrosis. Immunohistochemistry revealed positivity for CD68 protein and negativity for CD1a protein. Craniospinal magnetic resonance imaging demonstrated an extra-axial lesion in the right frontal region, a small nodule in the left middle cerebellar peduncle, and another small lesion in the right ventral pons. We performed a complete removal of the frontal lesion. The histologic examination produced results compatible with Rosai-Dorfman disease. Most lesions in intracranial Rosai-Dorfman disease mimic meningioma. The definitive diagnosis relies on pathologic and immunohistochemical characteristics. Surgical removal is generally regarded as the treatment of choice. Disease progression after surgical resection is uncommon. Surgical treatment is not recommended until clear disease progression is detected, or focal disease causes neurologic compression. This disease must be included in the differential diagnosis of lesions that mimic meningioma.
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PMID:Multiple involvement of the central nervous system in Rosai-Dorfman disease. 2219 95

We report a case of extranodal Rosai-Dorfman Disease (RDD) of the scophoid in a 52-year old female. The patient presented with pain, swelling, and tenderness on deep palpation of the left wrist. Clinicoradiological diagnosis was osteomyelitis or tenosynovitis and curettage was performed on the lytic lesion over scaphoid to procure tissue. Diagnosis was made by histomorphology supported by immunostaining. The patient was managed conservatively with resolution of the lesion.
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PMID:Extranodal Rosai-Dorfman disease in a carpal bone. 2291 28

A 49-year-old woman with history of breast cancer presented with pain at the level of the left anterior proximal tibia. An x-ray of the tibia demonstrated a lytic cortical lesion that prompted a whole-body 99mTc-MDP bone scan. The bone scan revealed intense bone remodeling at the level of the tibial lytic lesion and in the cervical spine. CT demonstrated an expansile lesion eroding the vertebral bodies of C6 and C7 with a large soft tissue component. A biopsy of the cervical spine mass demonstrated features diagnostic of Rosai-Dorfman disease without evidence of neoplastic cells.
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PMID:Primary Rosai-Dorfman disease of the bone in a patient with history of breast cancer: appearance on 99mTc-MDP scintigraphy, CT, and X-ray. 2529 Feb 90

Rosai-Dorfman disease (RDD) is a rare, benign histiocyte disorder originally described by Rosai and Dorfman in 1969 as sinus histiocytosis with massive lymphadenopathy. It most typically presents as massive, painless cervical lymphadenopathy, and it is most often found in adolescents and young adults. Extranodal involvement is a common feature of RDD and may occur in more than 40% of patients. Less commonly, the disease can be seen in the bone. There are scattered case reports discussing the use of radiotherapy in the treatment of RDD. Here, we present a case of extranodal RDD of the femur in a 49-year-old African American female. The patient underwent three surgical procedures prior to undergoing radiotherapy. Pathology was consistent with extranodal RDD. The cells stained positive for CD68 and S-100. Pathognomonic features such as emperipolesis and replacement of the bone marrow by diffuse infiltrating of histiocytes and intervening bands of plasma cells proliferation were noted. Prior to radiotherapy, the patient required a walker to assist with ambulation and was utilizing a wheelchair while in the clinic. The decision was made to proceed with 30 Gy of external beam radiotherapy in 15 fractions. After five fractions, the patient's pain resolved entirely. She no longer required pain medication and was ambulating without assistance. She experienced no adverse events from the radiation. Extranodal RDD is a rare disorder, and evidence for treatment is derived from scattered case reports. Previous reports have indicated a dose response to radiotherapy in the 20-30 Gy range for RDD; however, our patient developed complete resolution of her symptoms after 10 Gy. While the optimal dose regimen has not yet been established, symptomatic patients appear to benefit from external beam radiotherapy for extranodal RDD.
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PMID:Extranodal rosai-dorfman disease of the bone treated with surgery and radiotherapy. 2556 48

Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.
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PMID:Rosai-Dorfman disease affecting the maxilla. 2821 May 74

Rosai-Dorfman disease (RDD) is a rare condition similar to lymphomas, presenting with cervical lymphadenopathy in young adults. Extra-nodal involvement is relatively common but involvement of the central nervous system (CNS) is rare. Cranial RDD presents with symptoms of raised intracranial pressure, focal or generalised seizures, while spinal RDD presents with pain, peripheral neurological deficits and radiculopathy. In contrast to other similar neoplastic or degenerative conditions affecting the CNS, RDD is a benign, non-infective, granulomatous disorder. Radiologically cranio-spinal RDD often mimics commoner dural-based lesions like meningioma, with only subtle radiological differentiating findings on Magnetic Resonance Imaging (MRI). The histopathology of RDD is diagnostic. Surgical excision is preferred modality of treatment. However, adjuvant therapies like steroids and radiation may help controlling residual or recurrent disease. There are multiple sporadic reports and short case publications in the literature, often focusing on a particular aspect of RDD. In this study, authors aim to present five cases of craniospinal RDD, and comprehensive review of literature and highlight neurological complications of systemic RDD.
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PMID:Cranio-spinal Rosai Dorfman disease: case series and literature review. 2853 72

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease (RDD), was first described in 1969 as a reactive condition of unknown etiology that is characterized by a proliferation of histiocytes exhibiting emperipolesis of both lymphocytes and plasma cells. It usually presents with painless cervical lymphadenopathy either with or without extranodal manifestations. Intracranial involvement of this disease is extremely rare. Intracranial RDD occurs in <5% of all patients with extranodal disease. Here, we report a case of RDD with isolated intracranial involvement. A 67-year-old male presented with a long-standing headache, retro-ocular pain, and progressive visual loss of the left eye. Magnetic resonance imaging showed features of optic nerve meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive; however, a CD1a and epithelial membrane antigen were negative.
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PMID:A Rare Case of Isolated Intracranial Rosai-Dorfman Disease Mimicking Optic Nerve Meningioma: A Case Report and Literature Review. 3028 62


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