Infantile Rosai-Dorfman Disease With Isolated Brain Lesions Disseminated to the Parenchyma and Intraventricular Ependyma, Alteration of Leukocytes as a Promotion Factor in Immune Defense, and New Proposals: A Case Report and Literature Review

Abstract

The patient is a one-year-old girl referred to the hospital for an enlarged head after a 1.5-month history of two falls, followed by polydipsia, polyuria, and slow movement and growth. Three subsequent magnetic resonance imaging (MRI) examinations of the brain revealed nodular lesions disseminated in the brain parenchyma and intraventricular ependyma, resulting in obstructive hydrocephalus. Thoracic and abdominopelvic sonography showed no additional lesions. The preliminary diagnosis was a primary or metastatic neoplasm or infection. A biopsy of a lesion in the right frontal lobe was taken. The histological examination revealed features of Rosai-Dorfman disease (RDD), consisting of limited perivascular lymphoplasma cell infiltration with intervening sheets of proliferated histiocytes, with some of the histiocytes showing endocytosis of a single intact lymphocyte (emperipolesis).