ROSAI-DORFMAN DESEASE WITH INFRA­TENTORIAL LOCALIZATION: A CASE REPORT

Abstract

SUMMARY Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive cervical lymphadenopathy, occurs mostly in children and young adults. RDD extremely rarely affects the infratentorial region, without involvement of other anatomical structures. We report a case of a 36-year old woman with isolated RDD in the cerebellum. Clinical features were presented with a neocerebellar syndrome. Brain MRI showed a lesion with varying density, located in the left cerebellar hemisphere. No extracranial lesions were detected, The patient was operated on, and recovery was full. Microscopically, the process presented with lymphoplasmatic and multinucleate histiocytic infiltration. The prognosis is good. Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive cervical lymphadenopathy is a very rare condiction (probably less than 1000 cases reported in the literature) of unknown etiology. It commonly presents as massive, painless, bilateral lymph node enlargement in the neck with fever. Most cases occur in the first or second decade of life and have a predilection for blacks. It was first described by Rosai and Dorfman in 1969. They reported 4 cases of a disorder diagnosed as „malignant reticuloendotheliosis”. The disease can involve lymph nodes (nodular form) or the respiratory system , nasal sinuses, eye-orbits, bones skin (extranodular form). Nodular cases have been reported, with extranodular localizations . Intracranial localization of the disease without involvement of other sites is a very rare. Clinical presentation in 90% of the cases is lymphadenopathy, but there are cases where it can be asymptomatic, painful, anemia (normo- or hypochromic microcytosis) with antibodies against erythrocytes (polyclonal hypergamaglobulinemia) was registred in 90% of the cases. Diagnosis is based on histological investigations. Lymphoplasmatic cells are differentiated, though not atypical in shape. The cytoplasm of some histiocytes is pale and eosinophylic, or yeasty. Electron microscopy reveals phylopods protruding from membranes of all histiocytes. The abovementioned facts prove that diagnosis based on clinical features alone is impossible.