[Rosai-Dorfman disease with spinal and cranial tumors. A clinical case reported].

Revista medica del Instituto Mexicano del Seguro Social

PubMedID: 24758863

Molina-Carrión LE, Mendoza-Álvarez SA, Vera-Lastra OL, Caldera-Duarte A, Lara-Torres H, Hernández-González C. [Rosai-Dorfman disease with spinal and cranial tumors. A clinical case reported]. Rev Med Inst Mex Seguro Soc. 2014;52(2):218-23.
Rosai-Dorfman disease, known as well as sinus histiocytosis with massive lymphadenopathy, is a histiocytic proliferative disorder which may affect, with an extranodal presentation, the central nervous system, in 5 % of cases with exceptional reports of simultaneous development of spinal and cranial tumors. When it affects the central nervous system it appears more in men and it is shown as a mass in the cranial dura mater or in the spinal cord. The clinical symptoms of Rosai-Dorfman disease are fever, general malayse, weight loss, and nocturnal diaphoresis. Also, when Rosai-Dorfman disease affects the spinal cord, it has an impact on the thoracic spine, which causes paraparesis, quadriparesis, and sensory disorder. Histopathologically, the lymph nodes show emperipolesis. The diagnosis of Rosai-Dorfman disease is usually good, since 40 % of the patients present a spontaneous remission if they are treated with oral corticosteroids, even though the lesion can be managed with fractionated radiotherapy or with radical surgery. We report the case of a 34-year-old male who started with spinal injuries, and a year later showed intracranial lesions.