Rosai-Dorfman disease is a rare condition of marrow hematopoietic stem-cell origin. Patients can show extranodal involvement as well as lymphatic involvement, but only about 5% of extranodal cases involve intracranial lesions. A 53-year-old male was admitted to our hospital with bilateral cervical lymphadenopathy. Intracranial tumors and bone lesions were also detected. Cervical lymph node biop...

Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology typically characterized by cervical lymphadenopathy. Extranodal involvement often manifests in the head and neck region. We present a 10-year-old male who presented to our hospital with left epiphora from an aggressive paranasal mass invading the left orbit with osseous destruction. The mass was surgically bi...

Rosai-Dorfman disease is a rare disorder which seems to involve not only the lymph nodes but most other organs of the body. The case history is presented of a woman who was thought to have atypical carcinoma of the lung but in whom a diagnosis of Rosai-Dorfman disease was eventually made following a range of investigations including CT scans, positron emission tomography (PET) and histological ...

UNLABELLED Rosai-Dorfman Syndrome (RDD) is a rare, benign, self-limiting disorder which is characterized by the non-malignant proliferation of distinctive histiocytic cell within lymphatic system. RDD has been described as a dynamic entity in the spectrum of histiocytosis with non-Langerhans cell histiocytosis at one end and Langerhans cell histiocytosis at the other. The exact etiology of this...

rosai-dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disorder that typically manifests as lymphadenopathy and systemic symptoms whose etiology remains poorly elucidated. the diagnosis is based on immunohistochemistry. its treatment is poorly defined but the prognosis is usually favorable. here we report a 14 year old boy who presented with massive bilateral cervic...

S inus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease [RDD]) is a benign, idiopathic, self-limiting lymphoproliferative disease, described by Rosai and Dorfman in 1969. Extranodal manifestations represent about 43% of cases. The disease is classically accompanied by fever, malaise, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. Rosai-Dorfm...

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been r...

Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy. Isolated extranodal involvement is uncommon, and isolated laryngeal involvement is extremely rare. We report an unusual case of Rosai-Dorfman disease with isolated laryngeal involvement that led to recurrent dysphonia and airway obstruction. We discuss the challenges we faced...

An Askin-Rosai tumour presenting as effusive constrictive pericarditis is reported. A 2-D echocardiogram was strongly suggestive of a primary pericardial tumour, and multiple pericardial and pleural aspiration failed to yield malignant cells. The final diagnosis was made at autopsy. Primary pericardial Askin-Rosai tumour has not to our knowledge been previously reported.

BACKGROUND A proportion of cases of Rosai-Dorfman disease exhibit some histological features consistent with IgG4-related disease (IgG4RD). Several investigators have discussed whether Rosai-Dorfman disease belongs to the spectrum of IgG4RD or is concurrent with it by coincidence. OBJECTIVE To elucidate the relationship between the two diseases, we report key features, including IgG4 and amyl...