Signet-ring stromal tumor of the ovary is a rare disease and only four cases had been reported in the literature. The primary significance is to differentiate this benign disease from the malignant signet-ring adenocarcinoma. We report a case of signet-ring stromal tumor of the ovary in a 76-year-old woman who had suffered from low abdominal pain for one month. She underwent left oophorectomy, and the diagnosis was made based on immunohistochemical and electron microscopic characteristics. She has been well and free from recurrence for one year.
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