EN
TÜRKÇE
Poster Display - 226
Recurrent Bilateral Ovarian Teratoma with Wilms Tumor Component: A Rare Case Report and Literature Review
Sarra Aggoun, Guy Brisseau
Sidra Medicine, Qatar
Background: Ovarian teratomas are the most common germ cell tumors of the ovary, typically benign and unilateral. Bilateral and recurrent forms are rare, particularly in pediatric patients. The presence of Wilms tumor (nephroblastoma), a renal malignancy of childhood, within an ovarian teratoma is exceptionally rare and poses diagnostic and management challenges.
Case Presentation: We report a case of a 12-year-old female with a history of bilateral mature ovarian teratomas, initially treated at age 8 with laparoscopic surgery converted to open bilateral oocyte-sparing ovarian cystectomy. Five years later, she presented with recurrent pelvic pain. Imaging revealed bilateral ovarian masses, and she underwent surgical excision with bilateral enucleation and ovarian preservation.
Histopathology confirmed recurrent mature teratomas. Unexpectedly, microscopic examination revealed components consistent with Wilms tumor within the teratomatous tissue. Immunohistochemistry was positive for WT1 (nuclear, focal), PAX8 (diffuse), AE1/AE3, and CD56, and negative for markers including SALL-4, glypican-3, AFP, and others, confirming the diagnosis.
Two management approaches were discussed: the European protocol recommending 10 weeks of single-agent vincristine for Stage I Wilms tumor, and the North American approach favoring observation after complete resection with no adverse histology. The family chose the surveillance strategy. The patient is currently followed with abdominal ultrasound every three months and is also under adolescent gynecology care.
Conclusion: This rare case underscores the importance of considering Wilms tumor as a possible component within ovarian teratomas, particularly in recurrent or bilateral cases. Thorough histopathological and immunohistochemical analysis is essential to guide diagnosis and treatment. Close follow-up remains crucial due to the rarity and unpredictable behavior of this entity.