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Persistent mü llerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism. Bilateral fallopian tubes and a uterus occur with an otherwise normal male phenotype. Testicular neoplasm in PMDS has been reported, but malignant degeneration of the remnant female genitalia has only once been reported. We present imaging, surgical specimen, and surgical pathology of the first documented case of uterine adenosarcoma of a mü llerian remnant in a 14-year-old boy with PMDS presenting with gross hematuria and increasing abdominal protuberance. The child died of metastatic disease after surgical resection.
Persistent mü llerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism. Bilateral fallopian tubes and a uterus occur with an otherwise normal male phenotype. Testicular neoplasm in PMDS has been reported, but malignant degeneration of the remnant female genitalia has only once been been reported. We present imaging, surgical specimen, and surgical pathology of the first documented case of uterine adenosarcoma of a mü llerian remnant in a 14-year-old boy with PMDS presenting with gross hematuria and increasing abdominal protuberance. The child died of metastatic disease after surgical resection.