A case of extra nodal Rosai – Dorfman Disease of Lumbo Sacral Spine

Background - Rosai – Dorfman disease, or sinus histiocytosis with massive lymphadennopathy was first described as clinical distinct clinicopathlogical entity in 1969, in 43% of cases, extranodal sites are involved. The disease is most common in children and young adult. It has morphological features of greatly exaggerated reactive process. The etiology of the disease is uncertain, possible causes include, abnormal immune response and infections. Case history- A 20 years young man, born of consanguineous marriage, presented to our outpatient neurosurgical clinic with a history of rabidly progressing backache over the course of 6 months, he had nocturnal low grade fever and lower limbs pain for the same duration. Peripheral blood picture showed, Neutrophils 71%, lympocytes 18%, monocytes 9%and essinophils 2%, ESR 50 mm/hr. biochemical profile of renal and liver functions was normal. CT chest revealed no abnormality. MRI lumbo sacral spine was done and revealed, Straight lumber spine, there is an extra dural mass lesion noted occupying the posterior aspect of the dural sac, extending from the level of L1 lower border down to the upper border of L5. The patient underwent partial laminectomy L3 L5 and excision of the tumor. MRI in our case revealed that , diffuse enhancing lobulated masses without bony destruction this findings may be a diagnostic challenges for radiologist, although the diagnosis of Rosai dorfman disease depend on histopathological features and immno stain.