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A lethal intracranial Rosai-Dorfman disease of the brainstem diagnosed at autopsy.
Imada, Hiroki; Sakatani, Takashi; Sawada, Mikio; Matsuura, Tohru; Fukushima, Noriyoshi; Nakano, Imaharu.
Afiliação
  • Imada H; Department of Pathology, Jichi Medical University Hospital, Shimotsuke, Japan.
  • Sakatani T; Department of Pathology, Jichi Medical University Hospital, Shimotsuke, Japan.
  • Sawada M; Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Shimotsuke, Japan.
  • Matsuura T; Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Shimotsuke, Japan.
  • Fukushima N; Department of Pathology, Jichi Medical University Hospital, Shimotsuke, Japan.
  • Nakano I; Division of Neurology, Department of Internal Medicine, Jichi Medical University School of Medicine, Shimotsuke, Japan.
Pathol Int ; 65(10): 549-53, 2015 Oct.
Article em En | MEDLINE | ID: mdl-26184902
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Tronco Encefálico / Antígenos de Diferenciação Mielomonocítica / Histiocitose Sinusal / Dura-Máter / Histiócitos Tipo de estudo: Diagnostic_studies Limite: Aged / Female / Humans Idioma: En Revista: Pathol Int Assunto da revista: PATOLOGIA Ano de publicação: 2015 Tipo de documento: Article País de afiliação: Japão País de publicação: Austrália

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Tronco Encefálico / Antígenos de Diferenciação Mielomonocítica / Histiocitose Sinusal / Dura-Máter / Histiócitos Tipo de estudo: Diagnostic_studies Limite: Aged / Female / Humans Idioma: En Revista: Pathol Int Assunto da revista: PATOLOGIA Ano de publicação: 2015 Tipo de documento: Article País de afiliação: Japão País de publicação: Austrália