Gynecology & Obstetrics
Open Access

Abstract

Metachronous Multifocal Osseous Rosai-Dorfman Disease in a Pregnant Woman: Report of an Unusual Case and Brief Review of Pertinent Literature

Marwah M Abdulkader, Samir S Amr, Mohammad M Yousef, Hesham A Musleh, Amani A Joudeh, Mohammad H Nahhas and Mousa A Al-Abbadi

Rosai-Dorfman disease (RDD) is a rare histiocytosis that most commonly involves the cervical lymph nodes. Extranodal involvement occurs in 30-40% of cases and most often in the head and neck region. Prognosis is generally excellent. We present an unusual case of metachronous Rosai-Dorfman disease arising in multifocal bone sites in a 25-year-old pregnant female. She presented with right shoulder pain, frontal headache associated with fever and sweating. Computed Tomography scan and Magnetic Resonance Imaging studies demonstrated well-circumscribed lesions in the proximal humerus followed by a similar lesion in the frontal bone of the skull. Microscopic examination revealed variable numbers of pale-staining histiocytes with emperipolesis and background lymphoplasmacytic infiltrate and fibrosis. Immunohistochemical studies revealed positive staining of these histiocytes for S100 and CD68, but they were negative for CD1a. These findings were characteristic of RDD. Our patient was treated by curettage of both lesions. She is well and alive but with recurrence in both locations.