Extranodal Rosai–Dorfman disease involving bilateral ovaries in a patient with a ventriculoperitoneal shunt.

Rosai–Dorfman disease (RDD) is a rare condition of unknown etiology, and female genital tract involvement in RDD is uncommon. We describe the first case of RDD with bilateral ovarian involvement in a patient implanted with a ventriculoperitoneal (VP) shunt. The patient was a 17-year-old Japanese woman who had undergone radiotherapy, surgery for extranodal RDD involving the brain, and VP shunt insertion at age 12. Bilateral pelvic masses were incidentally detected on a computed tomography scan. She underwent laparotomy for lesion extirpation. On abdominal washing cytology, histiocytes showing emperipolesis were identified. Bilateral salpingo-oophorectomy was performed instead of extirpation, as it was difficult to identify the lesion margins. At 24 months after surgery, the patient is well and has not developed local recurrence. Thus, RDD can recur because of implantation of lesion cells into the abdominal cavity through a VP shunt, as is observed in the case of cerebral neoplasms. [ABSTRACT FROM AUTHOR]