Rosai-Dorfman Disease with Intracrnial Involment in a Young Female

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Professor, Consultant Pathologist, American Board Certified Pathologist, Fellow College of American Pathologists. Areas of interest Fine Needle Aspiration Cytology, Cytology, Cancer, Ethics, Islam, Humanity

Rosai-Dorfman Disease with Intracrnial Involment in a Young Female

Maryam Fatima, Ahmareen Khalid Sheikh and Ashok Kumar Tanwan

Department of Pathology, Pakistan Institute of Medical Science, Islamabad, Pakistan.

Abstract: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai Dorfman Disease (RDD) is an uncommon benign, idiopathic disease that mimics lymphoma clinically. Strong clinical suspicion is required to diagnose this disease. Laboratory investigations may be non-specific. RDD most commonly affects lymph nodes, but extranodal involvement of multiple organs has been reported in literature. CNS involvement by RDD is an uncommon finding and may mimic meningioma radiologically. Histopathology is the gold standard for the confirmation of diagnosis. Histological diagnosis is obtained after incisional biopsy or excision of the lesion. We report a case of a young girl who presented with massive bilateral cervical lymphadenopathy and headache. MRI brain showed extra-axial, lobulated right CP angle mass. Her CSF examination, Fine needle aspiration cytology of cervical lymph nodes and excision biopsy of one lymph node showed histological hallmark of RDD, which is lymphophagocytosis or emperipolesis displayed by the histiocytes. Controversy exists about the etiology and pathogenesis of RDD. It has a relapsing and remitting clinical course and therefore therapeutic management varies with the clinical presentation of the patient.

Key Words: Sinus histiocytosis, Emperipolesis, Lymphadenopathy.


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