| Summary: | Abstract Introduction Mixed sex cord stromal tumor is defined as a tumor consisting of various combinations of sex cord stromal elements, and the tumor is extremely rare. Case presentation A 76-year-old Japanese male visited our hospital complaining of left scrotal swelling. Magnetic resonance imaging of the mass showed a multilocular cystic pattern with different degrees of intensities in each cyst. The solid component was observed in part showing hypointensity on the T2-weighted image. Although there was no apparent evidence of malignancy in cytology of punctured fluid of the cystic tumor, malignant potential was not ruled out completely. Therefore, transinguinal radical orchiectomy was performed, and the tumor was diagnosed as mixed sex cord stromal tumor consisting of adult-type granulosa cell tumor, Leydig cell tumor and Sertoli cell tumor components. The patient recovered without any postoperative event. In addition, neither apparent recurrence nor metastasis was observed at 7 years after surgery. Conclusion The tumor showed a multilocular cystic appearance with solid component, which was similar in appearance to previous reports of the same pathological features. Pathological findings of each component were compatible with those of mixed sex cord stromal tumor, and immunohistochemical analysis was useful for accurate diagnosis. The tumor was successfully resected, and no apparent recurrence was observed at 7 years after surgery.
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