Rosai-Dorfman disease with paravertebral and epidural thoracic spine involvement: A case report and literature review

A Rare Case Report of Rosai-Dorfman Disease Manifesting as a Mediastinal Mass

“…MRIs demonstrated an ill-defined mass with isointensity compared with the spinal cord on T1WI. 9 A previously reported mediastinal RDD showed similar imaging features as our case of isointensity to hypointensity on T2WI, which could be attributed to the hypercellularity of the histiocytes within the lesion and could suggest the diagnosis of RDD. 9,12 Our case had an additional peripheral hyperintensity on T2WI, which corresponded to edematous change and a relatively hypocellular area in the pathologic specimen.…”

“…9 A previously reported mediastinal RDD showed similar imaging features as our case of isointensity to hypointensity on T2WI, which could be attributed to the hypercellularity of the histiocytes within the lesion and could suggest the diagnosis of RDD. 9,12 Our case had an additional peripheral hyperintensity on T2WI, which corresponded to edematous change and a relatively hypocellular area in the pathologic specimen. A previous report has suggested that the high signal on T2WI was probably due to an acute stage inflammation of RDD 13 ; however, our results did not confirm this finding.…”

“…In the largest study of RDD, 182 (43%) of the 423 patients evaluated had extranodal disease, whereas only 13 (3%) presented the typical morphologic profile of soft tissue RDD without detectable lymphadenopathy. 1 Isolated mediastinal RDD is exceedingly rare, [3][4][5][6][7][8][9][10][11] and only several case reports discussed the radiologic appearance. [4][5][6][8][9][10][11] The differential diagnosis of mediastinal RDD includes thymic epithelial tumors, germ cell tumor, lymphoma, inflammatory pseudotumor, and Castleman disease.…”

“…1 Isolated mediastinal RDD is exceedingly rare, [3][4][5][6][7][8][9][10][11] and only several case reports discussed the radiologic appearance. [4][5][6][8][9][10][11] The differential diagnosis of mediastinal RDD includes thymic epithelial tumors, germ cell tumor, lymphoma, inflammatory pseudotumor, and Castleman disease. Mediastinal RDDs have been visualized as solitary masses with or without invasion of the surrounding tissue 3,[6][7][8][9][10][11] and demonstrated homogeneous 9,10 or heterogenous enhancement 6,11 on enhanced CT. Our case showed the extension along the anterior chest wall, which may likely be malignant lesions or inflammation and unlikely to be lowgrade thymomas.…”

Experience of diagnosing and managing a patient with spinal Rosai-Dorfman-Destombes disease in a tuberculosis hyperendemic region

“…Spinal cord involvement in Rosai-Dorfman-Destombes (RDD) is a rare clinical entity that mimics lymphoma and tuberculosis. The diagnosis is challenging and needs a high index of suspicion 7…”