Sinus histiocytosis with massive lymphadenopathy: a case of simultaneous upper respiratory tract and CNS disease without lymphadenopathy.
نویسندگان
چکیده
The authors describe a 20-year-old man who initially developed an intradural mass in the upper cervical region and subsequently presented with nasal/paranasal sinus and posterior fossa masses, well demonstrated by CT and MR. Histopathology demonstrated dense fibrous tissue, aggregates of histiocytes with round to oval vesicular nuclei, and other features diagnostic of Rosai-Dorfman disease; a right nasal biopsy prior to surgery showed similar microscopic findings.
منابع مشابه
Rosai-dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence.
Sinus histiocytosis with massive lymphadenopathy (SHML) was first described in 1969 by Rosai and Dorfman [1]. It is a rare inflammatory disorder with key clinicopathological characteristics such as emperipolesis and positive immunostaining for S-100 protein [2] and CD68. Rosai–Dorfman disease (RDD) is primarily manifested in the lymph nodes, yet extranodal disease has been reported in as many a...
متن کاملRosai-Dorfman Disease: A Case Report and Literature Review
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 cervi...
متن کاملIntracranial multifocal Rosai-Dorfman disease
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is an uncommon benign histioproliferative disorder with systemic symptoms and lymphadenopathy. Mostly affecting lymph nodes, RDD typically presents as substantial painless bilateral cervical lymphadenopathy associated with fever, leukocytosis, anemia, polyclonal hypergammaglobulinemia, and increased ery...
متن کاملSinus histiocytosis with massive lymphadenopathy.
Sinus histiocytosis with massive lymphadenopathy (SHML) is a newly recognized, distinct, pseudolymphomatous benign entity with very characteristic microscopic features. Most patients are children or young adults with massive painless cervical adenopathy, although other node groups and extranodal sites often are involved. Sixteen patients with SHML involving the upper respiratory tract and/ or s...
متن کاملRosai-Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report.
A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome) in a 42-year-old man is described. The disease involved the respiratory tract and skeletal apparatus, led to considerable general impairment and was unusually responsive to glucocorticoids. Molecular and immunophenotype analysis seem to confirm the reactive nature of a disorder that shows profound T-cell immunod...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- AJNR. American journal of neuroradiology
دوره 14 1 شماره
صفحات -
تاریخ انتشار 1993