Case presentation: Isolated Intracranial Rosai-Dorfman Disease.

Solapas principales

Zumaeta J.1, Palacios F.1, Anicama W.2, Burgos C.2

 

ABSTRACT

            Rosai-Dorfman disease is a histiocytic, proliferative, idiopathic and benign disease characterized by sinus histiocytosis and massive lymphadenopathy. The most common clinical presentation is painless bilateral cervical lymphadenopathy. Extranodal involvement occurs in 43% of cases and central nervous system involvement by 4%. Rosai-Dorfman disease with central nervous system involvement occurs more in men and typically manifests as a mass in the cranial dura, which may or may not be associated with nodal involvement. It is a little-known clinical-pathological entity, there is controversy about its etiopathogenesis, clinical course and therapeutic management. We present the case of a 51-year-old woman with a history of sinusitis, who began her clinical condition with holocranial headache predominantly right, associated with periods of disorientation and ideomotor apraxia. Cerebral Magnetic Resonance showed a right parieto-occipital extraaxial lesion, constrictor, with implantation in the cranial dura mater. Right parietal craniotomy was performed with subtotal resection plus duroplasty with galea. The pathological anatomy was reported as Rosai-Dorfman's disease of meninges. The evolution after surgery was favorable, remitting symptoms.The involvement of the Central Nervous System of Rosai-Dorfman's disease is rare; in our hospital it is the first case reported and there is no record of any other case in our country. The patient is of the least affected sex and with an age at the upper limit of the range of presentation. The symptoms coincide with the description in the literature simulating focal seizures. The images coincide with the most common presentation simulating meningioma of the convexity. In the surgery, a lesion with a fibrous and hard pseudocapsule was found, different from what is commonly found in meningioma. The definitive diagnosis was made with histopathology finding emperipolesis and positivity of histiocytes for proteins S-100 and CD 68, but negative for CD1a. After surgery, most show improvement and do not have recurrence as our patient. Rosai-Dorfman disease should be included in the differential diagnosis of lesions based on dural implantation. Its diagnosis is eminently histological. Although there is no specific therapy, surgical removal is the most effective treatment in most cases. Adjuvant therapies such as steroids and radiation can help control residual or recurrent disease. The prognosis of Rosai-Dorfman disease is good where there is no nodal or multiorgan involvement.

Key Words: Rosai-Dorfman disease,  central nervous system.

 


  1. Servicio de Vascular y Tumores, Departamento de Neurocirugía del Hospital Nacional Guillermo Almenara Irigoyen. Lima. Perú.
  1. Servicio de Patología Quirúrgica y Necropsias, Departamento de Anatomía Patológica del Hospital Nacional Guillermo Almenara Irigoyen. Lima. Perú. 

 

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