Case Of Rosai-Dorfman Disease Presenting Research Articles

Rosai-Dorfman Disease Presenting with Recurrent, Unilateral Submandibular Lymph Adenitis: a Case Report.

Rosai-Dorfman Disease (RDD) is a rare disease of unknown aetiology, initially described as a separate entity in 1969 by Rosai and Dorfman. We describe here a case of RDD presenting with acute, recurrent lymphadenitis and massive lymphadenopathy. The patient's good response to the antibiotics was a false pointer towards infective pathology. The repeated fine needle aspiration cytology was inconclusive or suggested nonspecific reactive hyperplasia. The excision biopsy confirmed the diagnosis of RDD and was supported by immune-histochemistry for S-100 antigen. No other lymph node group was involved clinically or radiologically. The patient was treated with long-term antibiotic and low-dose predenisolone. The residual lymph node mass was excised after 3months of first surgery. The patient is on regular follow-up for last 2years and no recurrence has been reported.

Rosai-Dorfman Disease

ABSTRACTRosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a rare benign disorder of unknown etiology, which present most commonly as bilateral massive lymphadenopathy of neck associated with fever. These cases are frequently misdiagnosed as lymphoma, and thus it is important to distinguish Rosai-Dorfman disease from other causes of neck swelling because of different treatment modalities.We report here a case of Rosai-Dorfman disease presenting with massive right cervical lymphadenopathy.How to cite this articleJoshi S. Rosai-Dorfman Disease. Int J Otorhinolaryngol Clin 2014;6(3):121-122.

Rosai–Dorfman disease presenting as a solitary mediastinal mass

Rosai-Dorfman disease (RDD) involving an extranodal site is a diagnostic challenge. Reported herein is the case of a 67-year-old man who presented with a solitary superior mediastinal mass. The lesion was clinically suspected of malignancy including lymphoma because of its high uptake during a (67)Ga-scintigram and (18)F-fluorodeoxyglucose-positron emission tomography. There was no evidence of spread of the disease. Histology of thoracoscopic biopsy specimens indicated granulomatous lesion with infiltration of lymphocytes, plasma cells, and histiocytes with lymphocytes engulfed in their cytoplasm. The lesion did not contain lymph node or thymic elements. On immunohistochemistry the histiocytes were positive for S-100 protein, CD68, and CD163 but were negative for CD1a. These findings suggested a diagnosis of RDD. Despite lack of intervention, the lesion remained almost the same size for 3 years. To the best of the authors' knowledge this is the first case of RDD presenting as a solitary mediastinal mass.

Extranodal Rosai-Dorfman Disease presenting as an isolated epibulbar mass

Rosai-Dorfman disease is a rare idiopathic disorder characterized by painless lymphadenopathy with cervical involvement in more than 80% cases. We report a case of Rosai-Dorfman disease presenting as an isolated epibulbar mass in a healthy young adult male. Epibulbar involvement in Rosai-Dorfman disease is a rare presentation as can be seen from a review of all literature. The presentation, differential diagnosis and treatment are discussed.