title: Dysgerminoma developing from an ectopic ovary in a patient with WAGR syndrome: A case report.
abstract: WAGR syndrome is caused by an 11p13 deletion and includes Wilms' tumor, aniridia, genitourinary anomalies and mental retardation. We encountered a case of a dysgerminoma originating in an ectopic ovary in a woman with WAGR syndrome. Our patient was a 24-year-old nulliparous woman who was diagnosed with WAGR syndrome. The patient had undergone left nephrectomy for a Wilms' tumor and postoperative chemotherapy at the age of 7 months. She also had a history of glaucoma surgery in both eyes, and was followed up at the Department of Pediatrics for diabetes mellitus, hypertension, liver dysfunction and hyperuricemia. The patient was investigated for oliguria and had elevated levels of blood urea nitrogen (45 mg/dl) and creatinine (5.4 mg/dl); she was admitted to the hospital with acute renal failure and a computed tomography scan revealed a pelvic tumor with a long axis of 10 cm that was obstructing the right ureter. Following insertion of a ureteral stent, the tumor was removed. The tumor had developed in the retroperitoneal space independent of the ovaries. The right adnexa were normal. The tumor was histopathologically diagnosed as dysgerminoma. Follicles were found in part of the tumor; it was thus hypothesized that the tumor developed from an ectopic ovary. The patient was administered etoposide after surgery, and has been recurrence-free for 4 years since treatment.
authors.affiliation: Department of Obstetrics and Gynecology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Rie
authors.initials: R
authors.lastname: Miura
authors.affiliation: Department of Obstetrics and Gynecology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Yoshihito
authors.initials: Y
authors.lastname: Yokoyama
authors.affiliation: Department of Obstetrics and Gynecology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Tatsuhiko
authors.initials: T
authors.lastname: Shigeto
authors.affiliation: Department of Obstetrics and Gynecology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Futagami
authors.affiliation: Department of Obstetrics and Gynecology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Mizunuma
authors.affiliation: Department of Anatomic Pathology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Kurose
authors.affiliation: Department of Pediatrics, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Kazushi
authors.initials: K
authors.lastname: Tsuruga
authors.affiliation: Department of Pediatrics, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Sasaki
authors.affiliation: Department of Pediatrics, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Kiminori
authors.initials: K
authors.lastname: Terui
authors.affiliation: Department of Pediatrics, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori 036-8562, Japan.
authors.forename: Etsuro
authors.initials: E
authors.lastname: Ito
keywords: WAGR syndrome; dysgerminoma; ectopic ovary; etoposide; surgery