title: A lethal intracranial Rosai-Dorfman disease of the brainstem diagnosed at autopsy.
abstract: Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder characterized by the accumulation of histiocytes in lymph nodes and various other organs. RDD seldom involves the central nervous system, and cases of purely intracranial RDD are particularly rare. We report a case of purely intracranial RDD involving the brainstem that was diagnosed at autopsy. A 68-year-old woman visited our hospital because of visual disturbances and loss of energy. Magnetic resonance imaging revealed an obscure mass in the brainstem. Despite exhaustive work-ups, the etiology of the intracranial mass remained unclear. The patient died of respiratory depression, and an autopsy was performed for pathological investigation. Macroscopically, a pink pale mass 2.5 cm in diameter was found in the brainstem, with no attachment to the dura. Histologically, it was composed of histiocytic cells with incorporation of small lymphocytes (emperipolesis). Immunohistochemical staining revealed that the cells were positive for CD68 and S100 and negative for CD1a, consistent with a diagnosis of RDD. Purely intracranial RDD is extremely rare and considered benign. To date, nine cases (including ours) have been reported. To our knowledge, this is the first case of intracranial RDD with autopsy. Although generally considered benign, RDD involving the brainstem might be lethal.
authors.affiliation: Department of Pathology, Jichi Medical University Hospital, Shimotsuke, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Imada
authors.affiliation: Department of Pathology, Jichi Medical University Hospital, Shimotsuke, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Sakatani
authors.affiliation: Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Shimotsuke, Japan.
authors.forename: Mikio
authors.initials: M
authors.lastname: Sawada
authors.affiliation: Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Shimotsuke, Japan.
authors.forename: Tohru
authors.initials: T
authors.lastname: Matsuura
authors.affiliation: Department of Pathology, Jichi Medical University Hospital, Shimotsuke, Japan.
authors.forename: Noriyoshi
authors.initials: N
authors.lastname: Fukushima
authors.affiliation: Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Shimotsuke, Japan.
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism*; D001344:Autopsy; D001933:Brain Stem / Q000473:pathology*; D003937:Diagnosis, Differential; D004388:Dura Mater / Q000473:pathology*; D005260:Female; D006644:Histiocytes / Q000473:pathology*; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic
keywords: Rosai-Dorfman disease; autopsy; central nervous system; extra-nodal; intraparenchymal