Invasive squamous cell carcinoma of the vagina in the setting of true hermaphroditism.

Background: Primary vaginal cancer constitutes less than 2% of all malignancies of the female genital tract and is even rarer in true hermaphrodites. A unique case report of the occurrence of both of these developments in the same patient is presented.

Case: A 31-year-old 46,XX hermaphrodite presented with a history of vague abdominal symptoms and weight loss. Surgery was performed, and pathology revealed invasive squamous cell cancer arising in an epithelial-lined tissue consistent with vagina. The patient subsequently completed whole pelvic external-beam radiation therapy with cisplatin chemosensitization.

Conclusion: This is a case of primary vaginal cancer in a patient with true hermaphroditism, which is exceedingly rare. It is a reminder that malignant changes in residual mullerian tissue should be considered in these patients.