We report herein a case of isolated Rosai-Dorfman disease (Sinus Histiocytosis with Massive Lymphadenopathy) diagnosed upon routine microscopic examination of tonsils in a child. The patient is a 4-year-old girl who had a history of sleep apnea, but no other significant medical history or other findings on physical examination. In this report, we discuss the various approaches to handling of to...

Rosai-Dorfman Disease (RDD) is a rare non-neoplastic entity, also known as sinus histiocytosis with massive lymphadenopathy (SHML), characterized by a benign proliferation of histiocytes in lymph nodes. Localized forms of RDD involving the tracheobronchial tree are very rare. There is no consensus regarding the management of central airway forms and recurrence is frequent. We report the case of...

Ann Saudi Med 29(1) January-February 2009 www.saudiannals.net 55 Lampert and Lennert in 1961 were the first to describe what is now known as Rosai-Dorfman disease (RDD). Subsequently in 1969, Rosai and Dorfman described 4 cases of a disease they called sinus histocytosis with massive lymphadenopathy (better known as RDD).1 In 1972 they analyzed 30 additional cases, establishing RDD2 as a clinic...

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 pat...

As Rosai-Dorfman Disease presents generally nonspecific symptoms, differential diagnosis can be of great learning curve for physicians. Additionally, RDD does not usually threaten life and spontaneous remission is frequently observed. However, unusually in our case the patient passed away within 1 month of confirmed diagnosis.