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Soft tissue Rosai-Dorfman disease of the posterior mediastinum

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...

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Bibliographic Details
Published in:Jornal brasileiro de pneumologia 2009-07, Vol.35 (7), p.717-720
Main Authors: Costa, Antônio Luiz Penna, Silva, Natália Oliveira E, Motta, Marina Pamponet, Athanazio, Rodrigo Abensur, Athanazio, Daniel Abensur, Athanazio, Paulo Roberto Fontes
Format: Article
Language:eng ; por
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Summary: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.
ISSN:1806-3713
1806-3756
1806-3756
1806-3713
DOI:10.1590/S1806-37132009000700015