We report a case of pflügerome, a tumor now designated by the term "mixed germ cell sex cord and stroma tumor". This infrequent tumor consists in a proliferation of germ cells surrounded by Sertoli-granulosa-type cells and arises in patients with a feminine phenotype and a 46XY karyotype; it should be differentiated from gonadoblastomas that are seen in patients with a female phenotype and a 46XY or 46XX/46XY mosaic karyotype. Prognosis is good and recovery occurs following exeresis. On the basis of their studies on gonadal differentiation in human embryos, the authors propose the following terms: "tumors of the gonadal anlage with male differenciation" for gonadoblastomas, "tumors of the gonadal anlage with female differentiation" for pflügeromes.
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